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					Samatha Madhavarapu
21 m/o F with limping
  HPI
• Intermittent limp of R leg started 6 weeks ago.
• Constant limp since 3 days
• Worse upon awakening
• Stiffness in R knee.
• Transient warmth and redness +
• Not able to bear weight initially, improves over 2 hrs.
• Was outdoors in Upstate at BBQ 8 weeks ago
• No fever, rash, recent URI
• No trauma, diarrhea,
  HPI Contd…
 PMH: None
 FH: grand father has seizure disorder. No bone/joint
  problems
 Immunizations: UTD
 NKDA
 Meds: Tylenol PRN pain.
 Labs done 6 weeks ago: wbc7.6, 33.9/66.3, ESR 18, xray
  R knee Normal.
  Physical Exam
Vital signs:
HEENT : Normal
 Heart , Lungs ,Abdomen: Normal
Skin: No rash
R knee: decreased extension, no swelling, no redness,
 no warmth.
Other Jts: FROM.
  LABS
BMP: 136/4.9/ 101/22/12/0.4/139/10.5
Total Protein/ Albumin: 7.5/4.7
LFT: 0.2/01, 38/19, 244
CBC: 7.3/ 12/36.3/392
ESR: 15
  Other Labs
 CRP: 0.2
 ANA negative
 Lyme Ab titre: 1.2
 Anti CCP antibody: 8.3
 HLA B 27: Negative
 RF: 20.0
 Ultrasound of Knees: Small R knee jt effusion
  DD of Arthritis in Children
 Rheumatic &              Congenital & Metabolic
  Inflammatory Diseases     Disorders
 Seronegative             Bone & Cartilage
  Spondyloarthropathies     Disorders
 Infectious Illnesses     Neoplastic Disorders
 Reactive Arthritis       Hematologic Disorders
 Immunodeficiencies       Pain Syndromes
   Juvenile Rheumatoid Arthritis
 JRA most common rheumatic disease of childhood
 Synovitis of peripheral joints manifested as swelling
 JRA is not a single disease, but a category of diseases.
  Criteria for Classification of JRA
 Age of onset: < 16 yrs
 Arthritis in > or = 1 joint
 Duration of the Disease: > or = 6 weeks
 Onset type is defined by type of articular involvement
  in the first 6 months after onset:
 Poly arthritis: > or = 5 inflamed joints
 Oligo arthritis: < or = 4 inflamed joints
 Systemic Disease: arthritis with intermittent fever
  Classification of Chronic Arthritis in
  Children
 ACR, ELAR & ILAR classification
 Only ACR criteria have been statistically
 validated.
Characteristic                 ACR        ELAR       ILAR

Onset Types                    3          6          6

Age of onset of arthritis      < 16 yr    <16 yr     <16 yr

Duration of arthritis          >= 6 wk    >= 3mn     >= 6wk

Juvenile Ankylosing             Doesn’t   includes   Includes
Spondylitis                    include

Juvenile Psoriatic Arthritis   Doesn’t    includes   includes
                               include

Inflammatory Bowel             Doesn’t    includes   includes
Disease                        include

Exclusion of other diseases    YES        YES        YES
  Etiology
• Unknown
• Immunogenetic Susceptibility (Specific HLA
  subtypes)
• External Triggers
  - Viruses( EBV, Parvo virus B19, Rubella)
  - Hyperreactivity to self antigens(type 2 collagen)
  - Enhanced T-cell reactivity to heat schock proteins
  of bacteria.
  Epidemiology
 Incidence of JRA: 13.9/100,000
Sex:
 Pauci and poly articular disease more in girls
 Systemic onset –equal frequency in boys and girls
Race:
 Prevalnce of JIA lower in Urban African – American
  compared to Caucasians
 Oligo 40% newly diagnosed cases in Caucasians.
 Blacks with JRA were older and less likely to test positive
  for ANA or to have uveitis, more likely to test positive for
  Ig M RF
    Pathogenesis
• Synovitis: Villous hypertrophy & edema of subsynovial
    tissues.
•   Vascular endothelial hyperplasia
•   Infiltration of mononuclear and plasma cells.
•   Pannus formation with erosion of cartilage and bone.
•   Recriutment of T-cells specific to synovial non specific
    antigens., facilitated by specific HLA types.
  Clinical Features
 Onset insidious or abrupt
 Morning stiffness and gelling
 Easy fatiguability
 Joint pain (not very severe) and swelling, limited joint
  movt, not erythematous.
 May have preceding illness
  Oligoarthritis/Pauciarticular
• Affects 4 or fewer joints
• Typically larger joints (knees, ankles, wrists).
• Starts with 1 joint
• Monoarticular involvement of hip, upper extremity
  large joints never presenting sign in JRA.
• If knee is affected-limping+, esp morning
• Chronically- atrophy of extensor muscles of thigh,
  tight hamstrings & knee flexion contractures.
• Associated with HLA-DR8
  Polyarthritis/Polyarticular
• Minimum 5 joints should be effected.
• Both large & small jts of upper and lower extremities
• Resembles adult RA and HLA profile.
• Associated with HLA –DR4
• Rheumatoid nodules in severe form
• Micrognathia- chronic TM joint disease
• C-Spine involvement- atlantoaxial subluxation
    Systemic Onset
• Arthritis with visceral involvement
• Characteristic intermittent spiking fevers to >/= 39c
    for >/= 2 weeks.
•   Febrile episode assoc with evenescent (< 1 hr)
    macular rash, linear or circular, 2-5 mm, over trunk &
    proximal extremities.
•   Arthralgia, myalgia
•   Hepatosplenomegaly,Lymaphadenopathy
•   Serositis/pericardial effusion
•   Photophobia (uveitis), irregular iris due to synechiae
Oligoarthritis   Chr. uveitis
  Labs
 CBC with diff:
  Lymphopenia,Thrombocytosis,microcytic anemia.
  Neutropenia is uncommon.
 ESR:
  - Always elevated with systemic JRA.
  - Usually elevated with polyarticular but within
  reference range in pauci articular.
   - When elevated, ESR helps to monitor success of
  medical treatment
  Labs contd
 ANA:
Positive in 40-85% with oligo/poly articular
Unusual with systemic onset
Titres do not correlate with disease severity.
Associated with incraesed risk of uveitis
 RF: Rare in systemic JRA.
 Marker of persistence of polyarticular JRA into
  adulthood., devpt of rheumatoid nodules and poor
  functioning.
 Total protein and albumin: levels are often decreased
  during active disease
 ALT test: to exclude hepatitis (viral or autoimmune)
  prior to starting NSAIDS
 U/A: to r/o infection (trigger of JRA or transient
  postinfectious arthritis) and nephritis (seen in pts with
  SLE)
    Imaging
X-ray:
   When 1 jt is affected , to r/o osteomyelitis or
  septic arthritis.
 Soft tissue swelling, regional osteoporosis,
  osteopenia, sub chondral bone erosions, narrowing
  of cartilage spaces, fusion of nueral arches.
MRI
 synovial inflammation, early minimal changes seen
Echo cardiography: Serositis
DEXA: osteopenia
X rays
  Management
 Multidisciplinary Team for care of children
 Main goal: maximize daily functioning , minimize
  drug toxicity.
 Key predictor of long term outcome is early
  diagnosis and referral to rheumatology team.
 Diet: Include 3 servings of calcium rich foods
 Activity: more active, better the prognosis
    NSAIDS
• Used to treat all subtypes of JRA (40-60% children
    show improvement).
•   Mean duration for anti-inflammatory effect in JRA-
    30 days
•   Most with pauci and few with poly respond to NSAID
    alone .
•   Rofecoxib , celecoxib (selective Cox-2 inhibitors) ~
    similar to naproxen effectiveness
•   Adverse : nausea, decresead appetite, abd pain. Less
    gastritis Naproxen induced pseudoporphyria
    Methotrexate
• Safest, most efficacious, least toxic of of 2nd line
    agents for JRA.
•   Used in 60% patients with poly JRA
•   Inhibits DHFR, purine synthesis.
•   Pts unresponsive to PO MTX benefit from SC or IM
    administration.
•   Well tolerated and less toxicity in children.
•   Pts who respond well have improved growth,
    functionality, radiographic improvement.
    Glucocorticoids
• For overwhelmingly inflammatory
        or systemic illness.
•   Bridge therapy for those who did
          not respond to conventional therapy
•   Ocular control of uveitis (drops or injections)
•   Intra articular use :initial therapy in pts with only 1 or
    2 joint involvement
•   Improvement in symptoms in 2-3 days, which last for
    at least 6 mo in 60% and 1yr in 45%
    Anti TNF alpha
• Etanercept,: Only one approved for
                   children
•   Fusion protein with TNF receptor
     monomer to Fc portion of Ig G1.
•   Administered SC twice weekly used in active
    polyarticular JRA who fail MXT therapy.
•   JRA assoc-chronic uveitis that is inadequately
    responsive to steroid therapy.
•   Not to be used with h/o chronic infections
•   R/o TB before starting rx
  Sulphasalazine
 Improved joint inflammation & labs compared to
  placebo.
 GI irritation and rash.
 Steven johnson in pts with active systemic JRA.
 CI in porphyria and G6PD deficiency
    Prognostic features
• Child with oligo: esp. girls, onset< 6yrs age –chronic
    uveitis risk
•   Polyarticular: RF, rheumatoid nodules
•   Systemic onset: number of joints involved, duration
    of inflammation, severity of arthritis.
•    Limb length discrepancy, contractures,
•   Disability continues into adulthood in 20%
•   Chronic pain syndromes.
•   Psychological complications.
 http://www.youtube.com/watch?v=NqyB-cTxvs8
A 16-month-old boy is brought to your clinic because his mother says he
is "walking funny" today. She states that he has been walking for 4
months and is very active, but she is unaware of any trauma or falls. She
denies fever or other symptoms. He appears well and has normal vital
signs. Physical examination reveals mild tenderness to palpation over
the medial aspect of the lower leg just above the ankle. There is no
overlying bruising, erythema, or edema, and you can elicit full range of
motion in the hips, knees, and ankles.

Of the following, the MOST likely diagnosis is
a. Aneurysmal Bone Cyst
b. Ankle Sprain
c. Fracture
d. Osteomyelitis
e. Transient Synovitis
An 11-year-old girl presents 2 weeks after an office visit for a presumed viral
illness characterized by fever, malaise, and flushing of the cheeks. Today, her
mother notes that she no longer has a fever, but she complains of pain in her
knees and elbows. On physical examination, the left knee is slightly swollen and
warm but not erythematous. The girl reports pain on movement of both
elbows, but there are no physical findings on examination of the elbows or
other joints. The remainder of the physical examination findings are normal,
except for an oral temperature of 100.6°F (38.1°C). Results of laboratory studies
include a white blood cell count of 8.9x103/mcL (8.9x109/L) with 40%
polymorphonuclear leukocytes, 45% lymphocytes, and 15% monocytes;
hemoglobin of 11.0 g/dL (110.0 g/L); platelet count of 472.0x103/mcL
(472.0x109/L); and erythrocyte sedimentation rate of 20 mm/hr.
 Of the following, the MOST likely pathogen to cause this child's
joint complaints is

a. Borrelia burgdorferi

b. Coxsackievirus

c. group A beta-hemolytic streptococci

d. influenza A virus

e. parvovirus B19
Acknowledgement
Dr. Jillian Parekh
Dr. Karen Sawitz
Questions???

				
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