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					AD_HTT_33_40___MAR26_04         10/5/04       9:09 AM     Page 33




     How to Treat              Pull-out section                                                              now online @ www.australiandoctor.com.au

                                                                                                                                                                                            INSIDE
                                                                                                                                                                                            Juvenile arthritis

                                                                                                                                                                                            Knee pain

                                                                                                                                                                                            Widespread pain

                                                                                                                                                                                            Foot pain

                                                                                                                                                                                            Inflammatory
                                                                                                                                                                                            mimickers of
                                                                                                                                                                                            juvenile arthritis




                                                                                                                                                                                                  The authors




                                                                               Limb
                                                                               & joint pain
                                                                                                                                                                                            Dr Jane E Munro is
                                                                                                                                                                                            paediatric rheumatology
                                                                                                                                                                                            fellow, Monash Medical
                                                                                                                                                                                            Centre and Royal
                                                                                                                                                                                            Children’s Hospital,
                                                                                                                                                                                            Melbourne.




                                                                                  IN CHILDREN
                                                                                                                                                                                            Dr Susan E Piper is head
       Juvenile arthritis                                                                                                                                                                   of the paediatric
                                                                                                                                                                                            rheumatology unit,
      MANY conditions may present with            and by common usage as juvenile                                                                                                           Monash Medical Centre,
                                                                                                            Tips for history-taking in the child with suspected
      limb pain in children. “Is this juvenile    arthritis.                                                                                                                                and honorary senior
                                                                                                                              juvenile arthritis
      arthritis?” is the most common ques-           The term juvenile rheumatoid arthri-      ■   Check for other symptoms such as fever (including pattern), rash (colour, loca-          lecturer at Monash
      tion considered by the GP when assess-      tis is felt by many to imply that chil-          tion, migration and precipitants) or weakness.                                           University, Melbourne.
      ing a child with limb pain. The answer      dren with arthritis have a disease simi-     ■   Ask about the presence of early morning stiffness.
      is not easy in many cases, and the dif-     lar to adult rheumatoid arthritis and        ■   Check if there have been any antecedent infections or trauma to the area.
      ferential diagnosis includes many cond-     will be positive for rheumatoid factor,      ■   Assess impact on function (school, sport, sleep, appetite and activity compared
      itions.                                     but this is true only for very few chil-         with peers).
         Juvenile arthritis:                      dren with arthritis. Thus the name has       ■   Checking the family history, a history of slipped upper femoral epiphysis or HLA
      ■ Is an inflammatory arthritis;             lost favour with many paediatric                 B27-associated diseases, may be helpful.
      ■ Presents in young people under 16;        rheumatologists.
      ■ Affects one or more joints;                  Similarly, the word ‘chronic’ in juve-           Tips for examining the child with suspected juvenile arthritis
      ■ Lasts more than six weeks;                nile chronic arthritis has unfortunate       ■   Perform a thorough examination of all joints (including asymptomatic ones and
      ■ Is classified on the basis of disease     connotations for many patients. It is            ‘hidden’ joints such as hips, neck and temporomandibular joints).
        pattern over the first six months.        not meant to imply that the disease is       ■   Look for complications of the disease, such as joint-related problems (eg, leg
                                                  permanent but that the duration is               length discrepancy, muscle wasting or joint contractures) or systemic effects
      Diagnosis                                   longer than that seen in acute arthritis         (growth failure, anaemia).
      The diagnosis is made by excluding          (six weeks).                                 ■   Look for signs of conditions that mimic juvenile arthritis (eg, malignancy, SLE,
      other conditions. There is no diagnos-         So this term is also out of favour,           inflammatory bowel disease, juvenile dermatomyositis).
      tic test that confirms or excludes a        hence the compromise name of juve-
      diagnosis of juvenile arthritis.            nile idiopathic arthritis. However, most
                                                  patients and parents have trouble with      Differential diagnosis                        Non-inflammatory mimickers of juvenile
      Nomenclature                                the term idiopathic, so the terminol-       There are many conditions to con-             arthritis
      The nomenclature can be confusing.          ogy in common use is simply juvenile        sider in the differential diagnosis of        Non-inflammatory joint and limb
      The same disease is known as juvenile       arthritis.                                  juvenile arthritis. Although there are        conditions account for about 10%
      rheumatoid arthritis in the US, juve-          All these names are used in the med-     many rarer conditions in children             of children presenting to the GP.
      nile chronic arthritis in Europe, juve-     ical literature, particularly the less      that can present with features sug-           While many are minor or related to
      nile idiopathic arthritis by the Interna-   recent literature, and they refer to the    gestive of juvenile arthritis, we focus       trauma, there are some particular
      tional League Against Rheumatism,           same general condition.                     on the more common ones here.                                           cont’d page 35


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      from page 33
      conditions or patterns that                                                                                                X-ray showing          Child with suspected juvenile arthritis — pearls
                                                                                                                                 fragmentation                            and pitfalls
      are important to remember.
                                                                                                                                and collapse of    ■   There is no diagnostic test that confirms or excludes a diag-
                                                                                                                                    the femoral        nosis of juvenile arthritis. Suspect the ‘mimickers’ (see
      Irritable hip (transient                                                                                                 capital epiphysis
      synovitis)                                                                                                                                       page 37) and investigate or refer appropriately.
                                                                                                                              typical of Perthes
      This condition is the most                                                                                                                   ■   There is often a history of intercurrent viral infections, recent
                                                                                                                                        disease.
      common cause of limp in                                                                                                                          immunisations or minor trauma in children presenting with
      the preschool child. How-                                                                                                                        suspected juvenile arthritis; these are usually coincidental,
      ever, it is a diagnosis of                                                                                                                       not causal, and should not be relied upon to exclude more
      exclusion. The peak age of                                                                                                                       serious aetiologies.
      occurrence is 3-8 and there                                                                                                                  ■   Beware the isolated hip presentation, as this is rarely juvenile
      is often a history of a viral                                                                                                                    arthritis: look carefully, on repeated occasions, for a more
      URTI in the preceding 1-2                                                                                                                        serious cause, such as infection, orthopaedic causes of
      weeks.                                                                                                                                           mechanical hip pain, or inflammatory bowel disease.
         The child may refuse to                                                                                                                   ■   Early aggressive therapy has been shown to improve longer-
      walk but is otherwise gen-                                                                                                                       term outcome. A brief trial of NSAIDs (a few weeks) may be
      erally well and usually                                                                                                                          appropriate, but timely introduction of other therapies is often
      afebrile. Clinically other                                                                                                                       required.
      joints are normal but the                                                                                                                    ■   A multidisciplinary approach is optimal.
      hip joint has some reduced                                                                                                                   ■   Remember to get the eyes screened for iridocyclitis.
      range of motion (particu-                                                                                                                    ■   Get help early rather than late to avoid complications of both
      larly internal rotation).                                                                                                                        the disease (such as systemic and local growth effects) and
      Investigations usually reveal     by activity-related pain. It        Case study: a child with           unable to stand and the knee            the medications.
      normal       inflammatory         is related to trauma but            a limp                             is swollen.                                    When to refer the child with suspected
      markers (ESR, C-reactive          more often to repeated              A 10-year-old previously well         A week ago you saw her                                 juvenile arthritis
      protein, WCC), the X-ray          minor trauma rather than            boy presents to you with a         with an acute URTI, and three       ■   Immediately if the swelling is associated with any signs of
      is normal and ultrasound          one major episode. A vari-          two-week history of increas-       weeks ago she attended for              chronicity, such as leg length discrepancy, muscle wasting or
      may show a hip effusion.          ety of bones may be                 ing pain in his left groin. His    her preschool vaccinations.             joint contractures, or investigations reveal anaemia or a
         Management is rest (hard       affected, including the             mother thinks he may have          There is no history of rash,            marked inflammatory response.
      to enforce in a energetic         medial femoral condyle,             been limping for longer and        diarrhoea or abdominal pain,        ■   Persistent joint(s) swelling or pain needs investigation, eg, if a
      three-year-old!), NSAIDs          capitellum and talus.               recalls the physical education     and her mother has not been             post-viral arthritis persists beyond several weeks.
      and analgesia, usually at            X-rays may show a wedge-         teacher commenting on his          aware of a fever. She will          ■   If a mimicker is also suspected (particularly a malignancy).
      home. Occasionally hospi-         shaped area of separating           limp about a month ago.            stand, but limps when walk-         ■   If the pattern of illness is suggestive of systemic arthritis.
      talisation is required, and       bone on the joint surface, but         He is systemically well,        ing. Her mother comments
      joint aspiration to exclude       MRI will reveal more cases          although he had a URTI,            that she has been limping in
      infection and relieve pres-       showing the cartilage damage        along with his siblings, about     the morning recently before               Tips for history-taking in the child with a limp
      sure may be needed. Tran-         typical of this condition. The      six weeks ago. At that time he     kindergarten.                       ■   Pain-related information: it is essential to clarify if the limp is
      sient synovitis may recur         cartilage will be separated         also fell while rollerblading         Her temperature is 37.6˚C            associated with pain, particularly where and when the pain
      with a later viral infection      from the bone and the separa-       but seemed to recover              and she appears pale but has            occurs and the duration of the complaint(s).
      or increased activity.            tion may occur without any          quickly.                           no other abnormality on gen-        ■   Ask about other signs and symptoms, such as rash or
                                        evidence of bone damage. The           He has no other pains,          eral examination, although              weakness.
      Perthes Disease                   lesion can separate totally and     reports no joint swelling(s)       she is crying and totally unco-     ■   Check for fever.
      Perthes disease (also known       present as a loose body.            and does not have early            operative. You find she has a       ■   Check for any antecedent infections.
      as Legg-Calve-Perthes dis-           Bilateral disease occurs in      morning stiffness. He finds        swollen right knee with no          ■   Ask about a history of trauma to the area.
      ease) is avascular necrosis       at least 20% of cases and the       the pain gets worse when           hyperextension (unlike the          ■   Assess impact on function (school, sport, sleep, activity and
      of the capital epiphysis of       family history may be helpful.      standing or walking for long       normal left knee) and                   appetite compared with peers).
      the femoral head, occurring                                           periods and he stopped             restricted flexion, which you       ■   The family history, such as of slipped upper femoral
      in 2-12-year-olds, with a         Slipped upper femoral epiphysis     doing PE or playing football       assess as being due to the              epiphysis or HLA B27-associated diseases, may be helpful.
      peak in 4-8-year-olds.            This occurs predominantly in        this week because of the           degree of swelling.
         It is bilateral in about 20%   adolescents aged 10-15 and          pain, but has been attending          You order an X-ray of the                   Tips for examining the child with a limp
      of cases and has a                may present with acute, acute       school.                            knee to exclude injury or           ■   A thorough examination of the spine, sacroiliac joints, hips,
      male:female ratio of 4:1.         on chronic, or chronic pain in         There is no history of GI       other bony pathology and an             knees and ankles is essential.
      Children may present with         the hip, thigh or referred to       symptoms, weight loss or eye       FBC and ESR, which show             ■   Assess the gait.
      hip or groin pain (which may      the knee.                           complaints. Clinically he is       haemoglobin 110g/L (normal-         ■   Look for abductor weakness by Trendelenberg’s test.
      be referred to the buttock or        The onset is often insidious     afebrile and looks well, but is    range 115-130), WCC 11.5 ×          ■   Measure leg length: a difference of >0.5cm is abnormal.
      knee) and a limp. Occasion-       and it is important not to          limping with an antalgic gait.     109/L (4-11), platelets 470 ×       ■   Examine the neurological system, in particular looking for
      ally there is associated syn-     diagnose torn muscles or            He is overweight, with height      10 9/L (200-450), ESR                   muscle weakness.
      ovitis and therefore some         other equivalents in children       >75th centile and weight           20mm/h (0-10) and C-reactive        ■   Observe the shoes for patterns of wear.
      inflammatory features such        presenting in this manner. In       >90th centile.                     protein 12µg/mL (0-6).
      as early morning stiffness.       children presenting or re-pre-         He has pain on movement            When you review her late                  The child with a limp — pearls and pitfalls
         Clinically there is reduced    senting with hip, groin, thigh      of the left hip, with              the same afternoon she is           ■   Infection in the joint or bone is the first diagnosis that needs to
      internal rotation and             or knee pain, look hard for         decreased internal rotation        much more cooperative. She              be excluded: normal inflammatory markers do not exclude
      abduction (due to adductor        pathology.                          in particular. He flexes his       is afebrile. On joint examina-          infection (or prove it if raised). If there are any abnormalities on
      spasm). Inflammatory                 The condition is bilateral in    leg into external rotation.        tion the right knee is a little         the FBC or concern regarding joint sepsis, the joint must be
      markers are normal and X-         about 30% of cases; hence,          There is no leg length dis-        less swollen but you are not            aspirated (under general anesthesia in children under nine).
      ray may reveal changes in         many surgeons will operate          crepancy. He has a positive        sure whether there is swelling      ■   Viral arthritis can present acutely.
      the femoral head (with clas-      on both hips to treat or pre-       Trendelenberg test on the          of the left knee and the right      ■   Beware the isolated hip presentation, as it is rarely juvenile
      sic changes seen on bone          vent problems on the asymp-         left side (ie, pelvis drops on     wrist. On checking her leg              idiopathic arthritis: look carefully, on repeated occasions, for
      scan or MRI findings).            tomatic side. There may be a        the right side when standing       lengths you discover that her           a more serious cause, such as infection, orthopaedic causes
      Management includes rest          family history.                     on the left leg).                  right leg is 0.5cm longer than          of mechanical hip pain or inflammatory bowel disease.
      initially, possibly in hospi-        Clinically there is limitation      Both anteroposterior and        the left.                           ■   There is often a history of intercurrent viral infections or minor
      tal, with or without trac-        of internal rotation and possi-     frog-leg X-ray views are              You refer her to the local           trauma in children presenting with musculoskeletal com-
      tion, to relieve muscle           bly a positive Trendelenberg’s      essential and show an epiph-       paediatric rheumatologist               plaints; these are usually coincidental and should not be
      spasm, followed by gradual        test (abductor weakness).           ysis that has slipped down         who agrees to see her the               relied upon to exclude more serious aetiologies.
      mobilisation.                     Immediate cessation of weight-      and posteriorly. Blood tests       next day because it seems           ■   Children are not little adults: the causes of musculoskeletal
         Similar avascular necro-       bearing is strongly advised if      showed normal FBC, ESR             unlikely, as the day has                pains are often vastly different in the paediatric population;
      sis can develop in other          the condition is suspected.         and C-reactive protein.            evolved, that she has bacte-            strains and sprains are very uncommon causes of hip pain
      bones, such as in Scheuer-           Confirm the diagnosis               You diagnose a slipped          rial septic arthritis. During           and limp.
      mann’s disease, affecting         radiologically with X-ray           upper femoral epiphysis (see       the phone discussion the            ■   Hip pain may be referred to the knee or the thigh.
      the ring epiphysis of the         (anteroposterior and frog-leg       below) and call the local          rheumatologist comments             ■   Muscle weakness should be looked for and may be subtle.
      vertebral bodies, Kohler’s        lateral views) and/or bone          orthopaedic surgeon. The           that the leg length discrep-        ■   Don’t miss the mimickers — remember muscular weakness
      disease of the navicular          scan or MRI to detect early         patient is admitted to hospi-      ancy, the loss of hyperex-              or musculoskeletal malignancy as causes of a limp; acute
      bone, or Freiberg’s disease       slips. It is important not to       tal and has both his hips oper-    tension and the mild throm-             myositis may present with a painful gait and occurs most
      of the head of the second         miss the diagnosis of slipped       ated on the next day.              bocytosis might indicate                commonly in boys as a result of viral or post-viral infection.
      metatarsal bone (see Case         upper femoral epiphysis, as                                            that this is of longer dura-                      When to refer the child with a limp
      study: a child with foot          early surgery (fixation across      Case study: a swollen joint        tion than the history sug-          ■   Refer suspected slipped upper femoral epiphysis (urgent
      pain, page 37).                   the physis) can prevent fur-        A five-year-old girl presents      gests, and that the diagnosis           referral), osteochondritis dissecans or Perthes disease to an
                                        ther slippage, impairment of        with a history given by her        might be juvenile arthritis.            orthopaedic surgeon.
      Osteochondritis dissecans         vascular supply to the              mother of acute-onset right        However, it will be impor-          ■   Urgent referral is needed if bone or joint infection is a possibility.
      Osteochondritis dissecans is      femoral head and, ultimately,       knee pain that started the pre-    tant to consider other possi-       ■   Persistent musculoskeletal symptoms need investigation.
      a condition characterised         longer-term disability.             vious day. This morning she is     bilities.

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      how to treat - limb and joint pain in children


         Knee pain
      Patellofemoral                                                                              ray may show an enlarged frag-                          Tips for history-taking in the child with knee pain
      dysfunction                                                                                 mented tibial tuberosity on lateral          ■   Pain related information: clarify the nature of the pain, including where and
      THIS is a common cause of knee                                                              views.                                           when the pain occurs, ie, does it sound mechanical (hurts with movement,
      pain in adolescents and has several                                                            Usually symptoms settle with                  worse after exercise) or inflammatory (better with movement, early morning
      other names (chondromalacia patel-                                                          fusion of the tibial tuberosity epi-             stiffness, features of joint inflammation). Assess the duration of the
      lae, anterior knee pain, lateral pres-                                                      physes. Management consists of rest,             complaint(s).
      sure syndrome or maltracking of the                                                         physiotherapy for stretching, and            ■   Other symptoms such as fever, rash or weakness.
      patella).                                                                                   reassurance that the symptoms will           ■   Any antecedent infections.
         It is caused by a tracking abnor-                                                        improve with time.                           ■   History of trauma to the area.
      mality of the patella. Presentation is                                                                                                   ■   Assess impact on function (school, sport, sleep, activity and appetite
      with pain after exercise, particularly                                                      Benign nocturnal limb pains                      compared with peers).
      activity involving flexion of the knee                                                      (growing pains)
      and contraction of the quadriceps                                                           This is a common cause of limb                              Tips for examining the child with knee pain
      muscle. It may be exacerbated by                                                            pain in children aged 3-8 and                ■   A thorough examination of the spine, sacroiliac joints, hips, knees and
      walking up or down stairs.                                                                  occurs very late in the evening or               ankles is essential.
         Clinically there may be no abnor-                                                        may wake the child from sleep. The           ■   Look for any signs of joint inflammation.
      mality, but sometimes the pain is                                                           pain is poorly localised, often              ■   Measure leg lengths and perform Trendelenberg’s test.
      induced when the quadriceps is con-                                                         occurs along the shins and may               ■   Assess the quadriceps muscles for wasting.
      tracted, and there may be patellar                                                          occur after an active day. The child         ■   Palpate over the tibial tuberosity (see under Osgood-Schlatter disease).
      crepitus or a small knee effusion.                                                          is otherwise very well and active            ■   Assess the gait.
         Treatment involves a program for                                                         between episodes and there is no             ■   Look for inset hips and flat feet.
      strengthening the quadriceps mus-                                                           history of inflammatory symptoms             ■   Examine the neurological system and particularly look for muscle weakness.
      cles, with or without taping of the                                                         such as joint swelling or early              ■   Observe the shoes for patterns of wear.
      patella. Most children, particularly                                                        morning stiffness.
      after their diagnosis, should be able                                                          Although it is a clinical diagno-                         Child with knee pain — pearls and pitfalls
      to participate in sports again. The                                                         sis, if there are focal or systemic          ■   Beware of referred pain (eg, from the hip) as a cause of knee or thigh pain.
      condition is self-limiting and tends                                                        symptoms or signs, or the child is               Beware of monoarthritis — care must be taken to exclude more sinister
                                                     Knee changes typical of juvenile                                                          ■
      to settle with time over several               arthritis with muscle wasting above          unwell between episodes or the                   causes such as malignancy or infection (including more indolent but serious
      years.                                         and below the knee, giving the               onset has been acute, further investi-           infections such as TB).
                                                     appearance of bony enlargement,              gation is warranted (X-ray, FBC and          ■   A simple plan to increase adherence with quadriceps strengthening exer-
      Osgood-Schlatter’s condition                   minor enlargement of the femoral             ESR) to exclude other diagnoses such             cises is to encourage the child to pick a favourite television program they
      This is a common cause of knee pain,           epiphysis, valgus deformity secondary        as an osteoid osteoma (a benign bone             watch and, during the advertising breaks to extend one leg in front of them
      often bilateral, particularly in athletic      to medial femoral epiphyseal                 tumour), other bone pathology or                 (contracting the quadriceps) for the duration of that break, then to alternate
      adolescent boys, and is probably due           overgrowth and effusion of the knees.        leukaemia.                                       sides. Bike riding keeps the child physically fit and active as well as target-
      to repetitive strain injury (traction                                                          Management includes reassur-                  ing the quadriceps.
      apophysitis) at the insertion of the                                                        ance, as the condition is self-limit-
      patellar tendon into the tibial tuber-                                                      ing, and although intrusive (upon                               When to refer the child with knee pain
      cle. The pain increases with exercise                                                       the whole family) it is not serious.         ■   When there is joint swelling that does not improve over several weeks.
      and is relieved by icing and rest.                                                          Simple analgesia (possibly prophy-           ■   Urgent referral is required if bone or joint infection, neurological or oncologi-
         This is essentially a clinical diag-                                                     lactically if parents are able to pre-           cal causes are suspected.
      nosis (tender to palpation over the                                                         dict the evenings it occurs) and             ■   The pain does not improve with simple therapies such as paracetamol or
      tibial tuberosity, with no signs of                                                         local therapies such as heat or mas-             ibuprofen or physical therapies.
      joint inflammation), although an X-                                                         sage may be helpful.



         Widespread pain
      Benign joint                                                                                                                                            prolonged and patients need              The pain is worse at the end
      hypermobility syndrome                                   Tips for history-taking in the child with widespread pains                                     to avoid overuse injuries.            of the day, particularly behind
      HYPERMOBILITY is a                     ■    Evaluate the nature of the pain including when, where and for how long it occurs.                                                                 the knees and after running
      common cause of joint and/or           ■    Attempt to clarify both physical and psychosocial causes of the child’s presentation.                       Pain syndrome                         and gymnastics (which she
      limb pain in the older child or        ■    Assess pain behaviour, both in the child and the family (what is their response to the pain, how            The nomenclature of this con-         does three times a week). She
      adolescent. Normal joint range              do others respond to their pain?)                                                                           dition is confusing; other            has tried hot packs and parac-
      of motion varies with age and          ■    Assess the impact of the pain on the patient’s life and their family’s lives, as well as what their         names used include somato-            etamol, with no effect.
      racial background (with                     belief systems and expectations are (and whether these are helpful or realistic). Many children             form disorder, juvenile                  Clinically she is thin and
      younger children having much                with widespread pains withdraw from schooling, sporting and social activities.                              fibromyalgia and pain amplifi-        tall, at Tanner stage 4 of
      larger ranges of movement).                                                                                                                             cation syndrome.                      puberty. She has no signs of
         Generally children of Cau-                                Tips for examining the child with widespread pains                                            The aims of treatment              arthritis but her joints are
      casian background are more             ■    Examine the area(s) where there is pain, looking for a cause and associated signs of sympathetic            should be to eradicate the            hypermobile. She is able to
      flexible than children of                   nervous system overactivity (complex regional pain syndrome) or secondary deconditioning.                   pain, reduce both the patient’s       extend her elbows more than
      Mediterranean descent, while           ■    Assess for hypermobility of the joints as follows (score of 3/5 or more for diagnosis in a Cau-             and family’s pain behaviour           15˚, bring her thumb back to
      children of Asian descent are               casian child) — passive extension of metacarpophalangeal joints past 90˚; passive apposition of             and restore full function. This       her forearm and extend her
      more flexible again. Most chil-             the thumb to the forearm — hyperextension of knee past 10˚ and elbows past 15˚; passive appo-               can be done by both pharma-           knees more than 10˚ (she says
      dren satisfy the criteria for               sition of soles of feet with knees approximated; laying hands on floor while bending to touch toes          cological and non-pharmaco-           she has always been ‘bendy’).
      hypermobility until about age               with knees straight.                                                                                        logical methods, so a multidis-          There is no muscle tender-
      seven.                                                                                                                                                  ciplinary team approach is            ness on palpation, she is not
         Pain is usually exacerbated                                  When to refer the child with widespread pains                                           important, involving at a min-        dysmorphic, there is no evi-
      by activities, it may be diffuse,      ■    When there is evidence of complex regional pain syndrome not responsive to simple therapy or                imum the mental health prac-          dence of excessive scarring and
      and patellofemoral knee pain                with significant associated disability.                                                                     titioner and physiotherapist.         her skin is not lax. Her patellar
      (see below) may coexist. Look-         ■    When the pains have a significant impact on the function of a child.                                           Education, physical activity       tracking is normal and she has
      ing for any features of colla-                                                                                                                          and cognitive behavioural ther-       a negative apprehension test
      gen disorders is important                                    Child with widespread pains — pearls and pitfalls                                         apy techniques form the               (there is no pain on quadriceps
      (such as skin laxity, scarring         ■    Address the theories of mind and body linking how a person feels early in the management                    mainstay of management                contraction while you hold the
      or recurrent easy bruising).                phase to encourage the patient and family to engage in multidisciplinary therapies. If the mental           approaches to the child or ado-       patella).
      Some patients may develop                   health practitioners are mentioned at the end of extensive investigations, patients and their fami-         lescent with widespread pains.           You diagnose benign joint
      recurrent dislocation or sub-               lies often feel ‘dumped’ by their treating doctor and/or that they have been labelled as crazy or                                                 hypermobility syndrome.
      luxation of patellae or shoul-              malingerers.                                                                                                Case study: a 14-year-old             You explain the diagnosis to
      ders.                                                                                                                                                   with widespread pains                 the patient and recommend
         Clinically the patients may         volar aspect of their ipsilat-        ■ Place their hands flat on the                                            A 14-year-old adolescent girl         muscle-strengthening exer-
      be able to do all or some of           eral forearm.                           floor with feet adjacent and                                             presents with a two-month             cises. You suggest avoiding
      the following movements              ■ Hyperextend their elbow                 without bending their                                                    history of increasing pain in         sports such as gymnastics
      (three out of five are required        more than 15˚ or hyperex-               knees.                                                                   her wrists, fingers and knees.        and ballet because of the risk
      for diagnosis in a Caucasian           tend their knee more than                Management involves                                                     She has a history of trouble-         of soft tissue injuries, as she
      child):                                10˚.                                  muscle strengthening around                                                some constipation as a child          may have difficulty stabilis-
      ■ Passively dorsiflex their fifth    ■ Oppose the soles of the               the joints, simple analgesia                                               and recurrent abdominal pain          ing her joints, and suggest
        metacarpophalangeal joint            feet while sitting with legs          (often ineffective) and occa-                                              at age 7-10. There is no his-         sports that do not involve
        to more than 90˚.                    extended and knees                    sionally orthoses for the                                                  tory of early morning stiffness,      joint use at the extremes of
      ■ Oppose their thumb to the            touching.                             shoes. The course is often                                                 joint swelling, fever or rash.        range of movement.

    36   | Australian Doctor | 26 March 2004                                                            www.australiandoctor.com.au
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       Foot pain
      Stress fractures of the tarsal                    When to refer the child with         localised pain, especially on weight-       pain, the left side worse than the        the calcaneus inferiorly. The rest of
      bones (March fractures)                                 foot/heel pain                 bearing, on the second metatarsal           right. The pain is on the sole of his     his foot examination is unremarkable
      THESE injuries are caused by repeti-                                                   head, which may be associated with          foot under his heel and has become        and there is no enthesitis. His general
                                                    ■   When the pain is not improving
      tive activities such as running or                                                     swelling in this region. It is caused by    so bad he cannot finish playing a         joint examination is normal.
                                                        or resolving with simple therapies
      hiking, are often not visible on early                                                 repetitive trauma causing avascular         game of football. He is a keen foot-         X-ray is unremarkable and you
                                                        such as paracetamol or physical
      X-rays but may be seen on bone scan.                                                   necrosis.                                   baller and usually plays four times a     do not order any other tests. You
                                                        modalities.
      Treatment is symptomatic and the                                                         X-rays show flattening and squar-         week (for school and the local club)      diagnose an impact injury caused
      condition is usually self-limiting.                                                    ing of the second metatarsal head.          as well as at lunchtimes.                 by repeated impact on the calca-
                                                                                             Management is symptomatic, as the              He has tried over-the-counter arch     neus while wearing football shoes
      Sever’s disease                                                                        condition is self-limiting, and includes    supports, with no improvement. He         on hard ground. Poor landing tech-
      This is a traction apophysitis of the                                                  simple orthotic cushioning supports         has also had a short course of ibupro-    nique often contributes to the prob-
      calcaneus at the attachment of the                                                     and rest.                                   fen (again OTC) with no response.         lem. You suggest changing
      Achilles tendon. It occurs most             orthotic cushioning support for the                                                    He is otherwise well and there are no     footwear, especially for football (to
      commonly in 10-13-year-olds. It is          heel.                                      Case study: a child with                    other symptoms.                           a pair of boots with ‘wings’ and
      a self-limiting condition that                                                         foot pain                                      Clinical examination reveals a well-   shock-absorbing insole). He pro-
      responds to calf stretching exer-           Freiberg’s disease                         A nine-year-old boy presents with a         looking boy of normal growth para-        gressively improves over the next
      cises, with or without simple               This condition is characterised by         three-month history of bilateral foot       meters. He is tender to palpation over    four weeks.



       Inflammatory mimickers of juvenile arthritis
      Infection                                                                                                                                                                           Kawasaki disease
                                                                                               Hands showing typical changes of severe polyarticular juvenile arthritis
      BOTH viral and bacterial infection must always be considered.                            — swelling of the wrist extending over the carpus, swelling of the                         Kawasaki disease is also
      Viral arthritis is very common in children and the list of causative                       proximal and distal interphalangeal joints with relative sparing of                      more common in boys and
      agents is extremely long, making attempts to define the causative                            the metacarpophalangeal joints, and widening of the phalanges                          has a peak incidence in the
      agent usually fruitless.                                                                        secondary to tenosynovitis.                                                         6-11-month age group,
         The arthritis may be dramatic, with acute onset of marked                                                                                                                        although a wider age spread,
      joint swelling and pain over a few hours. Multiple joint involve-                                                                                                                   including 25% over five years
      ment is usual but monoarthritis does occur. Typically a rash                                                                                                                        of age, has been reported
      develops 7-10 days after a viral infection and lasts for a few days.                                                                                                                from Australia. It is charac-
         Apart from rubella infection, when the virus can sometimes be                                                                                                                    terised by:
      cultured from the joint, it is an immune-complex-mediated con-                                                                                                                      ■ Fever (39-40˚C) for at least

      dition and will usually resolve within 10 days. Arbovirus-medi-                                                                                                                       five days, unresponsive to
      ated disease, eg, Ross River fever, Barmah Forest fever and Q                                                                                                                         antibiotics;
      fever may last longer.                                                                                                                                                              ■ Conjunctivitis;

         While the acute-phase reactants may be markedly elevated, the                                                                                                                    ■ Lymphadenopathy;

      neutrophil count is about normal and the lymphocyte count                                                                                                                           ■ Polymorphous rash;

      may be either elevated or depressed (but rarely normal).                                                                                                                            ■ Dry, swollen red and

         However, if there is any question the infection might be bac-                                                                                                                      cracked lips with a ‘straw-
      terial, the joint should be aspirated, under general anaesthetic if                                                                                                                   berry’ tongue;
      the child is under nine, and with sedation as a minimum if a few                                                                                                                    ■ Erythema and swelling of

      years older. Culture should include media for meningococcus or                                                                                                                        palms and soles.
      gonococcus, both of which may present with only articular                                                                                                                              Early treatment with
      symptoms, and tuberculosis in ethnic groups at risk.                      Leukaemia is the                   showing areas of T2-enhanced        and should be undertaken by        aspirin, gamma-globulin and
                                                                                                                   hyperintensity in the muscle        practitioners experienced in       steroids has been associated
      Malignancy                                                                great mimicker in                  bellies. There is no association    the care of children with SLE.     with a reduced rate of coro-
      Oncological conditions can present with limb pain in children             paediatrics, and                   with malignancy in children.        GP involvement, especially for     nary artery aneurysm forma-
      and mimic many other conditions. Both primary or secondary                the doctor should                     Viral myositis is a more         screening for drug toxicity,       tion and death.
      malignancy can present with limb pain and at least initially the                                             common short-lived condition        flares of disease and manage-
      pain may not be severe. It is important to consider malignancy:           not necessarily be                 that occurs mostly in boys.         ment of intercurrent illnesses,    Perniosis (chilblains)
      ■ If the child appears more unwell than the examination would             reassured by the                   The child may present with          is invaluable.                     This condition is surprisingly
        suggest;                                                                                                   refusal to walk and marked                                             common. Typically it occurs
      ■ If there are other features, eg, night pain, lymphadenopathy
                                                                                first FBC being                    muscle weakness that devel-         Vasculitis                         in an adolescent girl who
        or hepatosplenomegaly;                                                  normal. Diligence                  oped overnight.                     Both Henoch-Schonlein pur-         dresses fashionably but poorly
      ■ If the child has pinpoint signs of tenderness, especially if this       and a high index                      Creatine phosphokinase           pura in older children and         for the weather, has recently
        is not over the joint line;                                                                                levels may be extremely high        Kawasaki disease in younger        had a growth spurt and is in
      ■ If the child fails to respond to simple analgesics or NSAIDs.
                                                                                of suspicion are                   but troponin levels are normal      children may present with          the ‘gangly’ phase, has little
         Leukaemia is the great mimicker in paediatrics, and the doctor         the order of the                   and the lymphocyte changes          limb pain.                         subcutaneous insulation and
      should not necessarily be reassured by the first FBC being                day.                               are typical of a viral infection.                                      is within 12 months of
      normal. Diligence and a high index of suspicion are the order of                                             Resolution is rapid, with serial    Henoch-Schonlein purpura           menarche.
      the day.                                                                                                     halving (or more) of the crea-      Henoch-Schonlein purpura is            There is almost always a
                                                                                                                   tine phosphokinase level fre-       more common in boys aged           history of itchiness around the
      Crohn’s disease                                                                                              quently seen from day to day        3-15 and is characterised by       time of onset. Skin thickening
      Inflammatory bowel disease may present with joint pain and/or                                                without any specific treat-         purpura, arthralgia or arthri-     and redness overlie the small
      inflammation. The bowel symptoms may be minimal or absent                                                    ment.                               tis, abdominal pain, GI            joints of the hands, resembling
      and the child may have features of arthritis only on history and                                                In contrast, juvenile derm-      bleeding and renal involve-        florid arthritis, but careful
      examination.                                                                                                 atomyositis will persist and        ment.                              examination will confirm that
         Pointers suggesting IBD, after a careful history for diarrhoea                                            progress for decades in about         In most children the con-        it is skin not synovial thicken-
      and abdominal pain, include elevated acute-phase reactants in                                                33% of cases. As a minimum,         dition resolves sponta-            ing that is causing the swelling
      excess of those expected for the degree of joint disease, or per-                                            children with myositis persist-     neously over four weeks and        and limiting function.
      sistent iron-deficiency anaemia, again in excess of that expected                                            ing for more than two weeks         requires only supportive               Most cases develop in early
      for the joint-disease activity. The presence of anal skin tags is sug-                                       should be referred for further      treatment. At least a third        winter, vary in severity
      gestive of IBD but the diagnosis will only be confirmed by                                                   invesigation.                       will have at least one recur-      through winter, resolve
      endoscopy.                                                                                                                                       rence.                             through summer and recur
         Controlling the bowel disease will resolve the joint problems in                                          Systemic lupus                        Some have a longer or            next winter. This pattern may
      almost all cases. Recurrence will usually herald a flare of the IBD.                                         erythematosus                       more severe course with            continue for 2-3 years but
                                                                                                                   SLE may present in children         progressive renal disease,         then improves or resolves.
      Juvenile dermatomyositis                                                                                     over age five but is more           and benefit from immuno-               Treatment is aimed at
      Juvenile dermatomyositis is an autoimmune inflammatory dis-                                                  common in adolescence, start-       suppressive therapy and            maintaining both core and
      ease of muscle, which develops in middle childhood.                                                          ing with arthralgia or arthritis    control of hypertension. A         peripheral temperature,
         It is almost always associated with the rash over the dorsum of                                           but usually progressing             small percentage will              using topical agents such as
      the metacarpophalangeal joints and upper eyelids, which may                                                  rapidly to multi-system dis-        progress to renal failure          heat packs and liniments, eg,
      also be present over the elbows, knees, rest of the face, shawl                                              ease. Most patients will have       despite treatment and it is        Dencorub, Bosisto’s euca-
      area, nailfolds and periungual regions.                                                                      multiple autoantibody pat-          recommended that all chil-         lyptus products or Deep
         Inflamed muscles are frequently tender, with pain increased by                                            terns. An isolated positive         dren with a past history of        Heat, or vasodilators like
      activity and progressive weakness, especially proximally.                                                    antinuclear antibody in a child     this disease have an annual        Rectogesic. Oral vasodilators
         Investigations reveal elevated creatine kinase and/or transam-                                            rarely suggests SLE.                check of their blood pres-         are usually ineffective and
      inase levels, but the ESR may be normal. MRI may be helpful                                                    Management is complex             sure for at least 10 years.        poorly tolerated.

                                                                                                   www.australiandoctor.com.au                                                     26 March 2004 | Australian Doctor |   37
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      how to treat - limb and joint pain in children


         Diagnosing and treating juvenile arthritis
      THE diagnosis of juvenile           ■ The only preparations that
                                                                                                                                   Table 1: Features of the different subtypes of juvenile arthritis
      arthritis requires the pres-          can be used in young chil-
      ence of joint inflammation            dren, but which are not                                                   Subtype                                       Features
      for six weeks in at least one         approved by the TGA for                                                   Systemic onset
      joint after excluding other           use in children, are:                                                     (sometimes known as Still’s disease)
      causes. Having considered a            — piroxicam 5mg or                                                       Polyarthritis                                 Five or more joints affected
      range of differential diag-               10mg (depending on                                                    Seropositive polyarthritis                    Only group with positive rheumatoid factor
      noses, juvenile arthritis may             body weight) once                                                     Oligoarthritis (also known as                 Four or fewer joints affected
      be the diagnosis in the pres-             daily. The soluble                                                    pauciarticular arthritis)
      ence of joint synovitis,                  preparation is suitable                                               Extended oligoarthritis                       Maximum of four joints at the six-month classification
      detected by the presence of               for young children;                                                                                                 point, but patients go on to develop more widespread
      two of the following:                  — rofecoxib suspension                                                                                                 joint disease
      ■ Joint line tenderness (careful          0.5mg/kg/day.                                                         Enthesitis-related arthritis (the enthesis
        examination is essential);        ■ Older children can use:
                                                                                                                      is a bone-tendon/ligament junction, eg,
      ■ Joint effusion or synovial           — naproxen 15-20mg/                                                      Achilles attachment to the calcaneus)
        thickening;                             kg/day divided into                                                   Psoriatic arthritis                           Either psoriasis (frequently only minor) affecting patient
      ■ Pain at the end of range of             two doses;                                                                                                          or first-degree relative; psoriasis may not develop in the
        movement (when the                   — diclofenac 25mg twice                                                                                                patient for 10 years. May have dactylitis (swelling of the
        inflamed synovium is                    a day;                                                                                                              whole digit, most commonly the toe)
        stretched or compressed).            — ibuprofen 40mg/kg/day
         The subtype classification             divided into four daily       trician with experience in the      response to therapy delayed               School visits by hospital           quences can be severe and
      is made six months from                   doses.                        management of paediatric            for 2-3 months.                         allied health staff or by             include blindness, cataract,
      onset because the disease can       ■ OTC ibuprofen (Nurofen)           rheumatology patients is               Gradual weaning is not ini-          Family and Children’s Ser-            glaucoma and band ker-
      change rapidly in its distrib-        will not provide anti-            advisable, if not essential.        tiated until there has been             vices officers of the state           atopathy, and ophthalmolo-
      ution over the early months.          inflammatory doses but            Paediatric rheumatologists          drug-related remission, with            arthritis foundation, either          gists with extensive paedi-
         The International League           may be useful for analgesia       are increasingly available in       no clinical or laboratory evi-          with particular staff or to           atric experience with young
      Against Rheumatism classi-            and fever, but not in com-        capital cities.                     dence of disease activity, for          school in-service days, are           children do a better job with
      fication was developed for            bination with other                  First-line therapy is            at least 12 months. Thus, once          available and many families           a fractious two-year-old.
      research purposes to ensure           NSAIDs;                           once-weekly methotrexate            started, treatment will con-            comment these visits made
      studies performed in differ-        ■ Aspirin has a very limited        10mg/m 2 of body surface            tinue for at least two years.           all the difference to their           Treatment outcomes
      ent centres around the world          role in Still’s Disease but       area, which has been shown                                                  child’s school experience.            Most children with appro-
      were being applied to com-            should only be used after         to have the best response           Physical therapies                                                            priately treated juvenile
      parable patients. The crite-          consultation with a paedi-        and the lowest toxicity rates       Physiotherapy has a vital               Ophthalmological review               arthritis attend school, play
      ria are strict and not always         atric     rheumatologist,         of all DMARDs. This can             role, with the experienced              Regular ophthalmology                 sport and participate fully
      easily applied to clinical            because of the risk of Reye’s     be given orally or by subcu-        physiotherapist working                 assessments for the presence          and normally in all activities.
      practice.                             syndrome.                         taneous injection.                  with the patient and family             and treatment of chronic iri-         However, this is not a
         Nonetheless the groups                                                  Other drugs used, in a           to address issues of joint              docyclitis are mandatory.             benign condition. A mini-
      identified are useful and,          Joint aspiration and injection      variety of circumstances,           position and range of move-             This asymptomatic non-                mum of 40% of children
      when the criteria are more          with corticosteroid,                include hydroxychloroquine,         ment, joint function, joint             granulomatous inflamma-               will have continuing joint
      loosely applied, allow for          and disease-modifying               sulfasalazine, leflunomide in       protection and muscle                   tion of the anterior chamber          inflammation 10 years after
      their use clinically. However,      antirheumatic drugs                 older teenagers, and,               strength.                               of the eye can occur in any           onset. At least 25% will
      a group of patients will con-       For younger children, and/or        recently, etanercept (a ‘bio-          The occupational therapist           child with arthritis but is           enter adulthood with some
      tinue to have features of sev-      particularly in monoarticu-         logical agent’), but patients       can assist patients with activ-         most commonly associated              disability. Ten per cent will
      eral groups and can only be         lar disease, some rheumatol-        requiring these therapies           ities of daily living adapted           with the presence of oligo-           have severe disability and
      fully diagnosed with the pas-       ogists prefer joint aspiration      need to be under the super-         to age-appropriate activities           articular disease of less than        this number is progressive
      sage of time.                       and steroid injection. This         vision of a paediatric              and levels of independence.             two years’ duration in a girl         with time even if the disease
         Seven subtypes are recog-        should be performed under a         rheumatologist or paediatric        Such assistance is essential            under four years of age who           process is controlled.
      nised, depending on the pat-        general anaesthetic and by          rheumatology unit. Appro-           to allow the child with                 is antinuclear-antibody posi-            Relatively minor sequelae
      tern of disease at six months       experienced practitioners.          priate and careful monitor-         severe arthritis to develop             tive.                                 such as a leg length differ-
      from onset of symptoms                If the response is inade-         ing for toxicity is manda-          normally to be able to func-               The frequency of assess-           ence may result in progres-
      (table 1 shows the features         quate, and particularly if          tory.                               tion as an adult.                       ment (from four-monthly to            sive secondary problems as
      of the different subtypes).         acute-phase reactants are ele-         All DMARDs require reg-             Liaison with the school can          annually) depends on the risk         the decades pass, such as
                                          vated, disease-modifying            ular blood tests for toxicity       make a world of difference.             category and should continue          back pain, hip or foot
      Management of                       antirheumatic           drugs       monitoring and this can be          Most teachers are only too              for seven years from diagno-          pathology. It behoves us to
      juvenile arthritis                  (DMARDs) should be con-             an issue. EMLA (‘angel’)            willing to help but, under-             sis. It is essential that all chil-   ensure minimal long-term
      Medications                         sidered. If DMARDs are              cream can be very helpful.          standably, do not have an               dren are assessed: despite this       problems by aggressive early
      Initial management is with          being considered, referral to          Using DMARDs is a long-          understanding of the disease or         condition being asympto-              treatment and a multidisci-
      NSAIDs, as follows:                 a rheumatologist or paedia-         term treatment, with the            what assistance can be useful.          matic, the ocular conse-              plinary team approach.


                                                                                        Characteristics of joint disease in children
                                                                             Polyarticular                                              Pauciarticular (oligoarthritis)
                                 Systemic                       Rh factor negative         Rh factor positive             Type I                 Extended                     Type II                  Psoriatic
                                                                                                                          oligoarthritis         oligoarthritis
         Usual age of            Most < 5                       Under 10                      Over 10                     1-5                    Cumulative total          Enthesitis-                 Increased incidence
         onset (years)                                                                                                                           of 5+ joints after        related                     with increased age
                                                                                                                                                 first 6 months            8-14
         Sex ratio               F=M                            F>M                           F >>> M                     F >> M                 F>M                       F <<< M                     F>M
         % of all juvenile       15-20                          25                            5-10                        25                     5-10                      20                          5-10
         chronic arthirits
         Clinical features       Fever (39-40°C ),              Symmetrical                 Nodules                       Chronic                  Asymmetrical            Acute iritis                Dactyltis,
                                 rash,                                                      and vasculitis,               iridocyclitis                                    20%,                        psoriasis (?minor)
                                 lymphadenopathy,                                           rapidly                       50%,                                             asymmetrical
                                 hepatosplenomegaly,                                        progressive                   asymmetrical
                                 serositis (pericarditis),                                  arthritis,
                                 arthritis/arthralgia                                       symmetrical
         Laboratory              Marked inflammatory            Mild to moderate abnormalities as                                                  Usually normal                                      Variable
         abnormalities           changes                            measures of disease activity
         Autoantibodies          Usually negative               ANA (25%)                   Rh factor (100%)              ANA (35%)                                                                    ANA (25%)
                                                                                            ANA (75%)
         HLA associations                                                                     HLA DR4                                                                      HLA B27 (75%)
         Outcome                 Systemic illness,              Arthritis destructive         Resembles severe            Major morbidity                                  Major morbidity relates     Subject to
                                 self-limiting                  in 10%,                       adult RA,                   relates to eye                                   to hip problems             relapse and
                                 arthritis                      younger child —               arthritis very              problems and                                     and progression to          remission recurrent
                                 destructive in 25%,            greater deformity             destructive in > 50%,       growth                                           sacroiliitis (50%)          throughout life
                                 early neck                                                   disease persists            disturbance                                      and anklosing
                                 involvement                                                  into adult life                                                              spondylitis


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      how to treat - limb and joint pain in children

         Conclusion
         LIMB pain is a common
                                               GP’s contribution
         presenting feature in chil-
                                                                                 was slightly anaemic, had                                                                                     It is quite common in that I
         dren. The causes are                                                                                         Typical
                                                                                 raised ESR at 41mm/h (this is                                                                               see or hear about several
         diverse and vary from                                                                                        vasculitic rash
                                                                                 moderately unusual in juve-          in a patient                                                           cases each year. However,
         benign sprains and strains
                                                                                 nile dermatomyositis) and            with SLE.                                                              Cefaclor is in common use
         to bone and muscle dis-
                                                                                 low-titre positive antinuclear                                                                              and I am unable to comment
         eases, infections, malig-
                                                                                 antibody.                                                                                                   on the frequency as a per-
         nancy and a large number
                                                                                    I arranged an urgent con-                                                                                centage of patients treated
         of inflammatory disorders,
                                                                                 sultation with a paediatric                                                                                 with Cefaclor.
         as well as mechanical and             DR RENATA CHAPMAN                 rheumatologist. He made a
         circulatory problems.
                                                     Chatswood, NSW              clinical diagnosis of dermato-                                                                              Do Australian children still
            While many can be man-
                                                                                 myositis based on the presence                                                                              contract rheumatic fever?
         aged by the family physi-
                                             Case study                          of a typical scaly, erythema-                                                                                 Rheumatic fever is very rare
         cian, others benefit from
                                             LUKE was always a sickly            tous eruption over the proxi-                                                                               in southern Australia but
         early referral, with associ-
                                             child. Born by normal vaginal       mal interphalangeal and                                                                                     common in northern Aus-
         ated improvement in long-
                                             delivery at 41 weeks with           metacarpophalangeal joints                                                                                  tralia, particularly among
         term outcome. The difficulty
                                             some meconium staining and          (Gottron’s sign), proximal                                                                                  Aboriginal communities.
         is knowing who to refer
                                             Apgar scores of 5 and 9, he         muscle weakness confirmed
         because both serious and
                                             was brought to my surgery           by the way Luke was rising                                                                                  With early presentation of a
         minor problems can look
                                             aged only 10 days with              from the floor (positive            a curious itchy rash over his       General questions for               swollen red joint, what
         similar early on. Experience
                                             watery diarrhoea.                   Gowers’ sign) and positive          legs and arms last year, which      the author                          would be the very first non-
         can be helpful and advice is
                                                From then on I saw him           nail-fold capillaroscopy (evi-      made me very nervous, but a         During my university days we        invasive test you would
         always available.
                                             regularly every 2-3 months          dence of cutaneous vasculitis).     dermatologist and rheumatol-        were taught that salicylates in     advise in diagnosing septic
                                             with URTIs, including one              The same day Luke was            ogist diagnosed it as erythema      full therapeutic doses are the      arthritis and osteomyelitis?
                                             episode of bronchiolitis that       admitted to hospital and fur-       multiforme and not a flare of       drugs of choice in the treat-          Clinically the presence of
         Key points
                                             required hospitalisation, fre-      ther investigations were per-       dermatomyositis.                    ment of rheumatic diseases.         fever in an unwell child, and
         ■   Juvenile idiopathic arthri-
                                             quent viral gastroenteritis and     formed, including MRI,                 Each time I see Luke’s name      Are they now out of favour?         an FBC with raised white cell
             tis is a diagnosis of exclu-
                                             atopic dermatitis.                  muscle biopsy, bone densito-        in my appointment book, I           What are the recommended            count, particularly neu-
             sion. It is relatively
                                                Nevertheless he was well         metry (pre-steroid treatment),      just hope that his visit has        doses?                              trophilia, and raised C-reac-
             common and is not a
                                             enough to receive his immuni-       physiotherapy assessment of         nothing to do with the juve-           Yes. The development of          tive protein level is diagnostic.
             benign disease. Com-
                                             sation and grew consistently,       the muscle strength, and            nile dermatomyositis.               NSAIDs has led to the with-         However septic arthritis and
             pared with previous eras,
                                             albeit along the lowest per-        blood levels of muscle                                                  drawal of salicylates from gen-     osteomyelitis can and do occur
             the treatments and out-
             comes have improved;
                                             centiles, to reach age four.        enzymes, aldolase, AST, ALT,        Questions for the author            eral management schedules.          in the absence of all these fea-
                                                One Friday afternoon his         creatine phosphokinase and          You have mentioned that juve-       Salicylates are poorly tolerated,   tures.
             earlier and more aggres-
                                             mother brought him from             factor VIII antigen.                nile dermatomyositis is not         with many side effects, and the        A high index of suspicion,
             sive therapeutic
                                             preschool with a rash on his           The results confirmed the        related to malignancy. Are          concerns about Reye’s syn-          blood cultures and joint aspi-
             approaches form the
                                             knees, elbows and face, as          diagnosis of juvenile der-          there any other comorbidities I     drome in children has meant         ration for culture in joint dis-
             mainstay of the manage-
                                             well as swollen fingers and         matomyositis. Luke was              should be on the look-out for?      that very few doctors or par-       ease, or blood cultures and
             ment plan.
                                             toes. She told me that three        started on IV methylypred-             There are no other disease       ents will accept treatment with     bone scan or MRI for
         ■   If there are any concerns
                                             weeks earlier he had a viral        nisone 30mg/kg daily for            associations but comorbidities      salicylates for any length of       osteomyelitis, are the best pre-
             regarding joint sepsis,
                                             illness, which she had              three days. He was discharged       related to steroid use, or to the   time.                               dictors. It is far better to over-
             urgent investigation and
                                             treated symptomatically at          home on oral prednisolone           calcinosis that develops in the        However, Kawasaki disease        investigate and treat until the
             referral is warranted.
                                             home.                               1mg/kg, to return for a fur-        later stages of the disease, are    remains an indication for sal-      diagnosis is clear than under-
         ■   Children are not little
                                                Luke looked generally tired      ther five IV pulses of methyl-      frequent causes of sequelae.        icylates for a short period and     investigate and to miss the
             adults. The causes of
                                             and unwell. He was afebrile.        prednisolone at monthly                                                 in some cases of Still’s disease    diagnosis.
             musculoskeletal pains are
                                             The rash on knees and elbows        intervals.                          What is the prognosis in juve-      it may be used to control
             often vastly different in
                                             was slightly scaly and there           Luke responded very well to      nile dermatomyositis — how          fever and serositis if other        With the present increase in
             the paediatric population;
                                             was a rather livid purplish         the treatment — the synovitis       likely is Luke to remain in         measures have failed. I have        childhood obesity, as well as
             strains and sprains are
                                             background to it. He had            resolved and he no longer had       remission/cure?                     not recommended use of sali-        the decreased physical activity
             very uncommon causes
                                             non-scaly patches on his face.      any detectable effusions on his        Two-thirds of cases resolve      cylates for more than two           caused by computers, TV, etc,
             of joint pain(s) or swelling
                                             There was a suggestion of           knees or ankles. The interpha-      over three years, as Luke has       weeks in more than 10 years.        is there any increase in the
             and limp.
                                             some erythema in a periorbital      langeal swelling of the proxi-      done, and it is unlikely he will    Anti-inflammatory doses are         prevalence of Perthes disease
         ■   Any musculoskeletal pain
                                             distribution.                       mal interphalangeal joints of       relapse after this time.            70-100mg/kg/day in divided          and/or slipped femoral epi-
             that persists for more
                                                He had slight redness over       both his hands subsided.                                                doses.                              physes?
             than four weeks needs
                                             his finger joints but the most      Repeat MRI showed remark-           Is he at risk of developing                                                There are no data on this.
             further evaluation.
                                             striking feature was a fusiform     able improvement, with only a       other autoimmune diseases in        Can you comment on the use
         ■   Although paediatric
                                             swelling of the proximal inter-     small high-signal focus in the      adulthood?                          of COX-2 inhibitors in chil-        What is the long-term out-
             rheumatology is a rela-
                                             phalangeal joints of his fingers    right rectus femoris muscle.          No.                               dren and their relative safety      come of slipped upper femoral
             tively new paediatric sub-
                                             and toes. I was tempted to             The primarily elevated                                               compared with NSAIDs?               epiphysis treated with pins?
             speciality, there are now
                                             settle for the diagnosis of reac-   aldolase level returned to          Would Luke’s treatment today           COX-2 inhibitors are not         Do these patients eventually
             paediatric rheumatolo-
                                             tive arthritis and send him         normal. The abnormal find-          still be based on steroids or are   approved for use in children        require hip surgery?
             gists in most Australian
                                             home with Nurofen and topi-         ing on Luke’s nailfold capil-       there other immunosuppres-          by the TGA. Overseas experi-           If the slippage is diagnosed
             major capital cities. Out-
                                             cal steroid cream, but I knew       laroscopy cleared. He had           sive or anti-inflammatory           ence indicates they are effec-      and treated early, before there
             come studies show early
                                             the problem was more serious.       some post-vasculitic staining       medications with greater            tive and safe. Generally            is much movement and associ-
             referral, in the case of
                                                On further questioning,          over the knees but his muscle       potency and fewer side effects?     NSAIDs are better tolerated         ated cartilage damage, the out-
             juvenile arthritis, improve
                                             Luke’s mother admitted that         strength was perfectly normal.         Initial treatment consists of    by children than by adults but      come is very good. There is a
             long-term outcome.
                                             he had been eating poorly           Luke’s mother noticed that he       steroids. Response in terms of      the side-effect profile is the      risk of early-onset osteoarthri-
                                             and had much less than his          could run up and down the           muscle strength can be slow         same.                               tis leading to total hip replace-
         Useful web sites for                usual energy, now having            stairs again.                       and may take 6-8 weeks                 Since the manufacturer’s         ment, but not until the fifth or
         doctors                             difficulties climbing the stairs       The specialist began reduc-      before there is any apparent        withdrawal of naproxen sus-         sixth decade.
         http://www.wch.org.au/              at home. My old habit of            ing Luke’s steroid dose and at      change. Other agents may be         pension from the market, there         However, even mild cases
         clinicalguide/pages/                charting weight and height          eleven months after starting        used in conjunction for those       is no liquid form of NSAID          can be complicated by spon-
         hippain.php                         each time I saw him now             treatment, the oral prednisone      who respond inadequately to         approved for use in children.       taneous chondrolysis result-
                                             proved useful — there was a         was stopped. His parents were       steroids alone, or to allow         The options are Vioxx sus-          ing in severe joint damage.
         http://www.wch.org.au/              significant dip on his weight       warned about the risk of            lower doses of steroids for         pension or Feldene D, but par-      The more severe the damage
         clinicalguide/pages/                graph.                              relapse of his disease.             patients requiring long-term        ents must be informed that this     at the time of diagnosis and
         joint_acute.php                        It was not too difficult to         Luke is now nine; it is five     treatment. Such agents include      is off-label use.                   initial treatment, the more
         Useful web site for                 find a vein in Luke’s rather        years since his initial presenta-   methotrexate, cyclosporine-A,                                           rapid will be the joint deterio-
         patients/families                   skinny precubital fossa. The        tion. He has caught up with         gamma-globulin infusions,           In your experience, how fre-        ration and the need for
         http://www.chw.edu.au/              urgent blood tests arrived on       his growth percentiles and is       azathioprine and cyclophos-         quent is serum sickness after       surgery.
         parents/factsheets                  Saturday morning and con-           doing extremely well at             phamide (particularly for vas-      the use of certain antibiotics         Accurate placement of
                                             firmed my suspicions — Luke         school. He had one episode of       culitis).                           (eg, Cefaclor)?                     the pins is important in
                                                                                                                                                                                             terms of long-term out-
                                                                                                                                                                                             come, and subsequent
         HOW TO TREAT                        NEXT WEEK                                                                                                                                       removal is frequently rec-
         Editor: Dr Lynn Buglar              The next How to Treat begins a two-part series on insomnia. The authors are Dr Gerard A Kennedy, lecturer in psychology
                                                                                                                                                                                             ommended to avoid migra-
         Co-ordinator: Julian McAllan        at Victoria University and senior consultant psychologist to the departments of respiratory and sleep medicine at Monash
                                                                                                                                                                                             tion and other complica-
                                             Medical Centre and Austin and Repatriation Medical Centre, Melbourne; and Dr Peter Solin, honorary clinical chair of the
                                                                                                                                                                                             tions that may lead to joint
                                             Australasian Sleep Association, in research at the department of respiratory and sleep medicine at Monash Medical Centre,
                                                                                                                                                                                             damage and the need for
                                             and in private practice at The Cabrini and Cedar Court Sleep Laboratories, Melbourne.
                                                                                                                                                                                             further surgery.

    40       | Australian Doctor | 26 March 2004                                                      www.australiandoctor.com.au

				
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