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					                                                                           Disclosures

           Rheumatology                                                      Biogen/Idec
                                                                             Genentech
                                                                             Medimmune
                  Seth Mark Berney, MD                                       Pfizer
                  Professor of Medicine                                      Roche
                  Center of Excellence for Arthritis
                    and Rheumatology
                  Louisiana State University School
                    of Medicine in Shreveport




                                                        Arthropathies Can Be Classified As:
                                                       Noninflammatory            Inflammatory (arthritis)
                                                       Primary osteoarthritis     Noncrystalline:
       Rheumatology part 1:                            Secondary osteoarthritis
                                                       Trauma                     Rheumatoid Arthritis
         RA/OA/Crystalline                             Neuropathic
                                                       + Metabolic disease
                                                                                  Seronegative Spondyloarthropathy
                                                                                           (SNSA)
                                                                                  Systemic Lupus Erythematosus
                                                                                  Scleroderma
                                                                                  Acute Rheumatic Fever

                                                                                  Crystalline:

                                                                                  Gout
                                                                                  Pseudogout




                                                        Classification Of Synovial Effusions

                                                       Type of fluid              Color             WBC/mm3
Therapy depends on whether the disease                                           Clear                 <200
                                                            Normal
is noninflammatory or inflammatory                                            (colorless)           (<25% PMN)
and whether noncrystalline on crystalline.             Noninflammatory       Clear-Yellow
                                                                                                     200-2,000
                                                                                                    (<25% PMN)
                                                                                                    2,000-100,000
                                                         Inflammatory       Cloudy Yellow
                                                                                                     (>50% PMN)
                                                                                                      >50,000
                                                             Septic               Purulent
                                                                                                    (>75% PMN)




                                                                                                                     1
                                                    60 y/o wm former Professional Football player
                                                         with a H/O multiple knee injuries c/o
                                                         bilateral knee pain x 10 years.

                                                    The patient also complains of bilateral wrist
                                                        pain, and 15-20 minutes AM stiffness.

                                                    He denies joint swelling, Raynaud’s
                                                        phenomena, sicca symptoms, fever or
                                                        chills.




 On PE:                                                                    Definition
                                                    Osteoarthritis (degenerative joint disease) is
Non-ill looking wm.                                      characterized by:

Nontender nodules of the right index, right long           1.   asymmetric joint involvement,
                       joints.
     and left ring DIP joints                              2
                                                           2.                           narrowing
                                                                asymmetric joint space narrowing,
Tenderness on palpation at the base of                     3.   joint hypertrophy and
     bilateral thumbs (CMC joints).                        4.   new bone formation (osteophyte).
Enlargement of bilateral knees with pain and
                                                    Rheumatoid Arthritis is a systemic disease with:
     crepitus on passive range of motion.               1. symmetric joint inflammation (arthritis) and
Normal wrists, MCPs, PIPs, elbows, hips and             2. autoantibody formation.
     ankles.




               Epidemiology                                                Etiology:
                                                    1o- no obvious underlying cause.
Osteoarthritis:                                           idiopathic
     Affects an estimated 12% of the US                   erosive osteoarthritis (eoa)
population        between the ages 25-75 years.
     80% of individuals over age of 75 years have   2o- resulting from p
                                                                g            joint disorders or systemic disease:
                                                                       prior j                   y
            radiographic evidence.                         1) Joint trauma-
                                                                        congenital defect
Rheumatoid Arthritis:                                                   developmental defect-
    Affects 1-2% of the population worldwide.                                   chondrodysplasias
    Male : Female= 1 : 2.5                                              injury
                                                                        excessive loads
    Peak age at onset: 25-55
                                                                        Charcot joint




                                                                                                                    2
                Etiology cont.:                                  Etiology cont.:
                                                        4) Endocrinopathies-
      2) Prior inflammatory arthropathy-
                                                              diabetes mellitus
            Gout
            CPPD                                              acromegaly
            RA                                                sex hormone abnormalities
                                                              hyperadrenocorticism
      3) Metabolic disorders-                           5) Collagen abnormalities
           hemochromatosis                                    (inherited or acquired)-
           ochronosis                                         Ehlers Danlos disease
           Alkaptonuria                                 6) Miscellaneous:
           Wilson’s disease                                   avascular necrosis
           Paget’s disease
                                                              hemophilia
           Gaucher’s disease
                                                              Osteopetrosis




 Typical OA History/ Physical Exam
History
Joint pain without inflammation which is worse after
      use and relieved by rest.
Morning stiffness < 1 hour
NO constitutional symptoms (fever, malaise, fatigue)

Physical Exam
Non ill-appearing
Bony joint enlargement
Heberden’s and Bouchard’s nodes
Joint tenderness with passive ROM
+ noninflammatory effusion                               (c) 1972-2004 American College of Rheumatology
                                                         Clinical Slide Collection. Used with permission.




  Typical RA History/Physical Exam
History:
Symmetric joint swelling, warmth, redness
Morning stiffness > 1 hour
+ constitutional symptoms

Physical Exam:
Ill-appearing
Ulnar deviation
Boutonniere and swan neck deformity
Limited joint range of motion
Joint swelling, redness, warmth, and pain (arthritis)
Extraarticular manifestations:
      Rheumatoid nodule   Ocular    Pulmonary
      Nervous system      Cardiac    Vasculitis         (c) 1972-2004 American College of Rheumatology
                                                        Clinical Slide Collection. Used with permission.




                                                                                                            3
  Typical OA laboratories and
    radiographic changes:
    Labs
           ESR-nl
           Joint fluid <2000 WBC
           CBC-nl
           SMA-20-nl

    Radiographs
         Asymmetric/nonconcentric joint
              space narrowing
         Osteophytes
         Bone enlargement




                                                        Typical RA Laboratory Abnormalities
                                                        Inflammatory
                                                        1.Normochromic normocytic anemia-
                                                                        low HCT, low normal MCV, low iron and
                                                        low/normal TIBC
                                                        2.
                                                        2 Elevated platelet count
                                                        3. Elevated ESR
                                                        4. Elevated C-reactive protein
                                                        5. Elevated immunoglobulin concentration
                                                        6. Joint fluid analysis- inflammatory
                                                        7. Rheumatoid factor (RF)- antibodies directed against the Fc
                                                               of IgG. Most common is IgM rheumatoid factor.
                                                               75-95% of patients are seropositive (RF+).
                                                        8. CCP antibody- supposed to be VERY specific but not
                                                               sensitive.




           Diseases with + RF                          Serologic Factors in RA: Anti-CCP and RF
Chronic bacterial infections
                     Subacute bacterial endocarditis                                      Sensitivity    Specificity
                     Leprosy
                     TB                                                                     (%)            (%)
                     Syphilis
                     Lyme disease                            Anti-CCP*                          41%         98%
Viral infection
                     Cytomegalovirus
                     EBV                                     RF                                 62%         84%
                     Hepatitis B, C
                     HIV                                     Anti-CCP +
                     Influenza                                                                  33%        99.6%
                     Rubella                                 RF
Parasitic infection
Sarcoidosis
Pulmonary interstitial disease
Liver disease                                             * anti-cyclic citrullinated peptide
Mixed Cryoglobulinemia                                  Bizzaro N et al. Clin Chem. 2001;47:1089-1093.
Malignancy




                                                                                                                        4
Typical RA Radiographic Abnormalities

1. Periarticular Osteopenia
2. Marginal erosions- where synovium attaches to bone.
3. Target joints-
                  a. MCPs
                  b. PIPs
                  c. Wrists (Carpal bones)
                  d. Cervical Spine
                  e. MTPs
4. Soft tissue swelling




                                                                                           OA                          RA
                                                                                        Asymmetric
                                                                  Joint                monoarticular,
                                                               distribution            oligoarticular,       Symmetric polyarticular
                                                                                        polyarticular
                                                                                       No, only Bony                    Yes
                                                                Synovitis              enlargement
                                                             Inflammation                    No                         Yes
                                                                                    DIP, PIP, CMC, Hips,        PIP, MCP, Wrists,
                                                               Target joint         Knees, Spine, Feet
                                                                                    Knees Spine Feet,        Elbows, C-spine, Knees
                                                                                                             Elbows C spine Knees,
                                                                                            Toes             Hips, Ankles, Toes
                                                             Joints spared           MCP, Wrist, Ankles       DIP, CMC, T/L/S spine

                                                              AM Stiffness              < 45 minutes                  >1 hour
                                                                                                              Inflammatory: elevated
                                                                   Labs               Noninflammatory          Plt, anemia of chronic
                                                                                                              disease, elevated ESR
                                                                          Bony Enlargement with               Bone loss: periarticular
                                                             Bone changes new bone formation                   osteopenia, marginal
                                                                              (osteophytes)                           erosions




               OA Management

       Goal:

       Relieve symptoms (pain) thus maintain
                          q     y
             function and quality of life.

             1. PT/OT
             2.Analgesia
             3.Surgery
                                                         American College of Rheumatology Subcommittee on Osteoarthritis Guidelines.
                                                         Recommendations for the medical management of Osteoarthritis of the hip and knee.
                                                         2000 Update. Arthritis and Rheumatism 2000;43:1905-1915.




                                                                                                                                             5
                RA Management                                             New Management
Goal:   Suppress inflammation (synovitis) to minimize
           joint damage and preserve joint function.     Drug Combinations: Use lower doses of individual drugs
                                                               resulting in lower toxicity but with increased inflammation
Inflammation suppression medications-                          suppression.
      DMARDs= Disease-Modifying anti-Rheumatic Drugs &
      Immunosuppressants/Cytotoxics                      Prosorba: Protein A Immunoadsorption column

DMARDs
DMARD                                                    IL-1                     IL-1Ra
                                                         IL 1 antagonist: soluble IL 1Ra [anakinra (Kineret)]
            Methotrexate (MTX)
            Gold (Au)                                    TNF- antagonists:
            Sulfasalazine                                     soluble TNF receptor [TNFR:Fc; Etanercept (Enbrel)]
            Hydroxychloroquine                                anti-TNF mAb [cA2; Infliximab (Remicade)]
            Minocycline                                       anti-TNF mAb [Adalimumab (Humira)]
            Leflunomide (Arava)
                                                         B cell antagonist: anti-CD20 mAb [Rituximab (Rituxan)]
Immunosuppressants/Cytotoxics
           Azathioprine                                  T cell antagonist:
           Mycophenylate mofetil                                 T cell costimulator blocker [CTLA4-Ig; Abatacept (Orencia)]




                                                                          Definition of Gout

                                                               An acute arthritis caused by the inflammatory
                                                                    response to monosodium urate crystals
         Crystalline Arthropathy                                    in the joint.

                                                               Neutrophils phagocytose the crystals and
                                                                           degranulate. The enzymes
                                                               released          cause the clinical
                                                               manifestations of            inflammation
                                                               which include severe joint inflammation,
                                                               systemic fever and     peripheral
                                                               leukocytosis.




                                                                             Patient History
                                                            A 56 y/o man with a h/o HTN, obesity and regular
                                                            alcohol intake who presents to the ER on Sunday
                                                            morning with a severely painful, red, swollen left
                                                            great toe, which awakened him from a drunken
                                                                                                   didn t
                                                            sleep. The pain is so severe that he didn’t even
                                                            want the bedsheet touching it. He has had 5
                                                            similar episodes in the past last year that resolved
                                                            without treatment. He just completed 7
                                                            consecutive days of being too drunk to go to work.
                                                            He takes a diuretic and one aspirin per day.




                                                                                                                               6
        Patient Physical Examination                               Common Symptoms of Gout
                                                              Acute onset.
    He is an obese male in obvious pain with a                Severe inflammation (pain, swelling, warmth, and
    fever of 101.0° F.                                              erythema).
                                                              Monoarticular (initially) or pauciartiular.
    On musculoskeletal examination, the left 1st
    MTP joint is purple-red and exquisitely tender
                  purple red                tender.           Involvement of 1st MTP joint (Podagra) – occurs
    The erythema, swelling and pain extends up                      in 75% of patients.
    to the ankle.                                             Onset often at night.
                                                              Early attacks subside spontaneously in 3-10 days.
    The remainder of the physical examination is
    normal.                                                   Attacks may be triggered by alcohol, trauma,
                                                                  drugs, surgical stress or acute medical illness.




    Common Physical Examination
         Findings in Gout
1. Arthritis (Joint inflammation):
                   redness
                   warmth
                   tenderness
                   swelling

2. Surrounding soft tissue inflammation (cellulitis)

2. Tophi (deposits of monosodium urate crystals)
   can occur anywhere, cause the characteristic
   gout erosions and can only be distinguished from
   rheumatoid nodules by aspirating and identifying
   MSU crystals under polarizing microscopy.                           (c) 1972-2004 American College of Rheumatology
                                                                       Clinical Slide Collection. Used with permission.




                                                                    Differential Diagnosis for
                                                                Acute Onset Monoarticular Arthritis

                                                                            Crystal-induced arthritis
                                                                            Trauma
                                                                            Septic arthritis




           (c) 1972-2004 American College of Rheumatology
           Clinical Slide Collection. Used with permission.




                                                                                                                          7
     To Diagnose a Patient with
     Acute Monoarticular Arthritis
   Joint aspiration and inspecting synovial fluid
      or tophaceous material and
      demonstrating characteristic MSU
      crystals (intracellular if joint fluid) is the
      only way to definitively diagnose gout.
      This will also allow gram stain and culture
      to evaluate infectious arthritis.

   Foot radiographs should be done to evaluate
     for possible fracture.

   Serum uric acid level is not helpful in making
     the diagnosis of gout.                                     (c) 1972-2004 American College of Rheumatology
                                                                Clinical Slide Collection. Used with permission.




                                                       Typical Laboratory Findings in Gout
                                                       Inflammatory synovial fluid (can be sterile pus)
                                                              a. Cloudy
                                                              b. 20,000 to 100, 000 WBC/mm
                                                              c. Predominately PMN
                                                       Monosodium urate crystals in synovial fluid
                                                              a. Needle-shapedp
                                                              b. Strong, negative birefringence with
                                                                    compensated polarized light
                                                       Serum uric acid is elevated at some time in almost
                                                                    all patients. However it is NOT
                                                            DIAGNOSTIC.
                                                       Urine uric acid >750 to 1000 mg/day suggests
                                                              overproduction of uric acid.
                                                       May have leukocytosis, high ESR, increased
                                                                    C-reactive protein during acute attack.




Typical Radiographic Findings in Gout

      Soft tissue swelling during acute
          attack.

      Soft tissue density if tophi are present.

      Oval bone erosions with overhanging
         edge is classic abnormality.




                                                                                                                   8
                Acute Treatment                                      Acute Treatment cont.
Treats the inflammation:                                   Oral or intramuscular corticosteroids are also
NSAIDs                                                      effective.
  are a treatment of choice for acute gout,
  preferably an NSAID with a short half-life such as       Oral high dose colchicine is rarely used as
                         p
  indomethacin or ibuprofen.                                initial treatment of acute gout due to the high
                                                            frequency of adverse effects and generally
Intraarticular corticosteroid injection
  is effective for alleviating acute gouty arthritis in     excellent response of acute gout to NSAID
  most cases. Injection into a small joint can be           with lower incidence of adverse effects.
  difficult and rarely the inflammation can recur
  when the steroid effect subsides. BUT, this is my        Intravenous colchicine should be avoided
  (and most rheumatologists’) treatment of choice if
  only one or two joints are affected.                     NEVER increase or begin a urate lowering
                                                            agent during an acute attack.




       Classification of Hyperuricemia                             Underexcretion (90%)
Overproduction (metabolic) (10%)
                                                          Primary
Primary                                                        1. Idiopathic
  1. Idiopathic
  2. Specific Enzyme Defects (<1% of Primary)             Secondary
                                                               1. Acute or chronic renal failure
                    y
        a. PRPP synthetase overactivity
                                      y                                      p
                                                               2. Volume depletion
        b. Partial deficiency of HGPRTase                      3. Altered renal tubular handling of uric acid
        c. “Complete” deficiency of HGPRTase                         due to drugs, volume status or
Secondary: Increased cell turnover or destruction
                                                                     endogenous metabolic products:
     (which increases nucleic acid release)                                diuretic therapy,
                                                                           low-dose salicylate therapy,
  a.   Lymphoproliferative or myeloproliferative                           lactic acidosis,
  b.   Chemotherapy                                                        ketoacidosis,
  c.   Acute or chronic hemolysis                                          ethanol
  d.   Psoriasis




                Chronic Therapy                             Allopurinol vs Uricosuric Agents
                                                          Uricosurics (probenecid, sulfinpyrazone or high
       Colchicine and NSAIDs                                   dose salicylate)
       prevent flares associated with changes in          may be used in patients who are underexcretors
       uric acid. We treat for one month prior to         of uric acid and who do not have renal
       initiating hypouricemic therapy and                insufficiency, hi t
                                                          i    ffi i              f kidney stones, or t hi
                                                                         history of kid     t         tophi.
       always begin urate lowering drugs while
       on antiinflammatory medication.                    However, their use requires patients drink > 1
                                                          gallon water/day.

                                                          The presence of tophi (or erosions) would indicate
                                                          that the treatment of choice is allopurinol.




                                                                                                                9
                                                  Clinical Pattern in CPPD Disease
     Calcium Pyrophosphate
                                                      Pseudo-osteoarthritis – 50%
    Dihydrate (CPPD) Crystal                          Pseudo-gout – 25 %
       Disease Including                              Lanthanic (asymptomatic) – 20%
           Pseudogout                                 Pseudo-rheumatoid
                                                      Pseudo rheumatoid arthritis – 5%
                                                      Pseudo-neurotropic joints – rare

                                                      Associated with aging




   Pseudogout Clinical Features
 Similar to gout
 Acute onset
 Initially monoarticular but becomes oligo or
         polyarticular
 Self-limiting attacks last from 1 day to weeks
 Knee is most common joint
 May be precipitated by surgery, medical
 illness   or trauma.
 CPPD crystals- rhomboid, weak, positive
     birefringence
                                                       (c) 1972-2004 American College of Rheumatology
                                                       Clinical Slide Collection. Used with permission.




Pseudogout Radiographic Findings


       Chondrocalcinosis in 75%

       Calcification of cartilage

       Often associated with osteoarthritis




                                                                                                          10
            Pseudogout Therapy                                   CPPD Associated Diseases
   Similar to gout therapy

   Arthrocentesis alone may be adequate                                      Hypothyroidism
                                                                                   y
                                                                             Parathyroid disease
   Intra articular corticosteroids
   Intra-articular
                                                                             Hemochromatosis
   Non-steroidal Anti-Inflammatory Drugs (NSAID)
   Colchicine may be effective

   Maintenance therapy with NSAID and/or
        colchicine




                                                            30 year old white female cashier with 5 year H/O
                                                                 arthralgias, fatigue, +ANA.
 SLE/Vasculitis/Other syndromes

                                                            22 year old black female nursing student with 1
                                                                 year H/O arthralgias, alopecia, fatigue with
                                                                 sun exposure, +ANA, +DS-DNA, hematuria
                                                                 with RBC casts and proteinuria.




   SLE: 1982 Classification Criteria                          SLE: 1982 Classification Criteria
                                                          Serositis:
Serositis         Blood dyscrasias         Malar rash                  Pleuritis, Pericarditis or peritonitis.
Oral ulcers       Renal disorder           Discoid rash
                                                          Oral ulcers:
Arthritis         ANA
Photosensitive    Immunologic disorder                                 Painless oral or nasopharyngeal ulcerations
 rash             Neurologic disorder                     Arthritis:
                                                                       Nonerosive arthritis involving > 2 peripheral
                                                                          joints (clinically indistinguishable from RA
                                                          Photosensitivity:
 Patient must have 4 of 11 criteria observed by MD.
                                                                       Skin rash from UV irradiation.




                                                                                                                         11
      SLE: 1982 Classification Criteria                          Frequency of positive ANA (%)
Blood Dyscrasias:                                                               Rodent epithelial Tissue      Hep 2
       Coombs + Hemolytic anemia with reticulocytosis    Cells
 or
       Leukopenia- <4,000 WBC on > 2 occasions or        SLE                                 90-95        98
       Lymphopenia <1,500
       Lymphopenia- <1 500 on 2 occasions or             PSS                                 50-70        95
                                                         RA                                  30-50        50-75
       Thrombocytopenia- <100,000                        Primary Sjogrens       50                        75
                                                         Polymyositis                        30                   90
Renal:                                                   JRA                                 25                   ?
             Persistent proteinuria > 0.5 grams/day or   Chronic Active Hepatitis            50                   ?
             Cellular casts or                           Infectious Mono                     5-20                 ?
             > 10 RBC/HPF                                Leprosy                             5-20                 ?
                         but urine must be sterile       Vasculitis                          5-20                 ?
                                                         SBE                                 5-20                 ?
ANA




         Antinuclear antibody (ANA)                             SLE: 1982 Classification Criteria
      ANA is directed against nuclear components.        Immunologic disorder:
                                                                  anti-ds DNA antibody (anti-native DNA ab) or
                                                                  anti-SM antibody (anti-smith ab) or
      Significant titer is > 1:80                                 antiphospholipid antibody (2 positive tests performed
                                                           with
      Found in 98% SLE patients but also-                                       at least a 12 week interval) based on
           RA                                              either:
           PSS                                                          1. abnormal serum IgG or IgM anticardiolipin ab
                                                           or
           Polymyositis                                                 2. lupus anticoagulant or
           Sjogrens syndrome                                            3. false positive RPR or VDRL for > 6 months
           MCTD                                            and
           Procainamide/Hydralazine/Dilantin                                    confirmed by t. Pallidum immobilization or
                                                                                fluorescent treponemal antibody (FTA)
           Interstitial pulmonary fibrosis                                             absorption test.
           normal population
                                                         Neurologic disorder:
                                                                  Seizures or




              Immunologic                                       Antiphospholipid
              disorder                                            syndrome
                                                           antiphospholipid ab = anticardiolipin ab =
                                                                           anti-beta-2 glycoprotein ab
  Antibodies to DS-DNA or SM are not sensitive
        but are very specific for SLE.
                                                           Antibodies to phospholipid bind to and activate
  Anti-DS
  Anti DS DNA ab indicates patients at increased            p                 g     gg g
                                                            platelets resulting in aggregation and
        risk for significant SLE renal disease and          thrombosis.
        may parallel lupus activity in individual
        patients.                                          Antiphospholipid antibodies are procoagulants
                                                            despite the misnomer of also causing the
                                                            ‘lupus anticoagulant’ (falsely prolonged PTT).

                                                           Can occur as a 1o illness or as part of SLE.




                                                                                                                             12
   Antiphospholipid
    syndrome
           Arterial thrombosis
           Venous thrombosis
           Libman Sachs
           Libman-Sachs Endocarditis
           Livedo Reticularis
           Recurrent Fetal Loss
           Thrombocytopenia



                                                            (c) 1972-2004 American College of Rheumatology
                                                            Clinical Slide Collection. Used with permission.




SLE: 1982 Classification Criteria

Malar rash:
       Fixed erythema, flat or raised, over the
            malar eminences, tending to spare
 the
              nasolabial folds.

Discoid rash:
     Erythematous raised patches with scaling
          and follicular plugging.
     Atrophic scarring may occur in older
 lesions.
                                                            (c) 1972-2004 American College of Rheumatology
                                                            Clinical Slide Collection. Used with permission.




         (c) 1972-2004 American College of Rheumatology
         Clinical Slide Collection. Used with permission.




                                                                                                               13
                                                                          Subacute Cutaneous
                                                                          Lupus Erythematosus




                                                                          (c) 1972-2004 American College of Rheumatology
                                                                          Clinical Slide Collection. Used with permission.




                         Therapy                                                    Therapy cont.
      Serositis                                                          Renal
                           NSAID                                                         Corticosteroids
                           Corticosteroids                                               Immunosuppressive
                           Immunosuppressive
                                                                         Neurologic
      Arthritis                                                                           y p
                                                                                         Symptomatic
                           NSAIDs                                                        Corticosteroids
                           Hydroxychloroquine                                            Immunosuppressive
                           DMARDs
                           Corticosteroids                               Rash
                           Immunosuppressive                                             Topical Corticosteroids
        Drugs                                                                            Systemic Steroids
                                                                                         Hydroxychloroquine
      Blood dyscrasias                                                                   Methotrexate
                           Corticosteroids                                               Immunosuppressive Drugs
                           Immunosuppressive




                     Therapy cont.                                                 Patient #1 & 2
Antiphospholipid antibody syndrome: (Usually lifelong)
Pregnancy morbidity: 81 mg/day ASA + DVT prevention dose          22 year old black female nursing student with 1
 LMWH                                                                  year H/O arthralgias, alopecia, fatigue with
 1. > 1 unexplained deaths of a normal > 10 week fetus or              sun exposure, +ANA, +DS-DNA, hematuria
 2. > 1 births of a normal neonate before the 34th week of
             gestation because of eclampsia, severe
             g                           p ,                           with RBC casts and proteinuria.
 preeclampsia,
             or recognized features of placental insufficiency.
 3. > 3 consecutive spontaneous abortions before the 10th         30 year old white female cashier with 5 year H/O
             week of gestation, with maternal                          arthralgias, fatigue, +ANA.
 anatomy/hormonal
             and paternal/maternal chromosomal causes
 excluded.
First venous thrombosis: INR of 2.0-3.0
Recurrent venous thrombosis: INR >3.0
Arterial thrombosis: INR >3.0




                                                                                                                             14
 SLE is more than just a + ANA, arthralgias
 and fatigue.
                                                                          Vasculitis
 It is diagnosed when the patient has an
 appropriate history, physical exam and
 supportive laboratory abnormalities.




                  Definition                                  Patient Presentation
Vasculitis is a heterogeneous group of              50 y/o white male with a 3 month h/o:
conditions characterized by inflammation and        T=100.3, 10 lb unintentional weight loss, fatigue,
necrosis of blood vessel walls.                     achy joints and muscles, ? rash, indigestion and
                                                    breathless with minimal activity.
Arteries and veins, of various sizes and in
              veins                                 On PE:
                                                    Pt looks tired. BP=155/93, HR=92, T=99.
different locations may be involved resulting       Questionable numbness in hands with slightly
in a great diversity of symptoms and findings.      decreased grip strength o/w unremarkable exam.
                                                    Laboratory results:
Arteritis = angiitis= vasculitis of arteries.       HCT=31(39-44),
Venulitis = vasculitis of veins.                    ESR=55 (<20),
                                                    Plt=450 (160-350)
                                                    UA: 5-7 RBC/hpf




      Polyarteritis Nodosa (PAN)
Necrotizing angiitis affecting small-medium sized
     vessels characterized by granulomas and/or
     aneurysms at vessel bifurcations in
     potentially all organs except the lungs.

Most common age 40 -60 y/o.

Male : Female = 2:1

25% are caused by/related to hepatitis B virus
     antigenemia (Hep B sag+).

                                                            (c) 1972-2004 American College of Rheumatology
                                                            Clinical Slide Collection. Used with permission.




                                                                                                               15
     (c) 1972-2004 American College
     of Rheumatology Clinical Slide
     Collection. Used with permission.                                 (c) 1972-2004 American College of Rheumatology
                                                                       Clinical Slide Collection. Used with permission.




                                                                        Churg-Strauss
     Polyarteritis Nodosa (PAN) cont.                         (Allergic Granulomatosis/Angiitis)
      Diagnosis:                                               Eosinophilic vasculitis
           Obtain biopsy of any involved tissue
                 or angiogram.                                 Multiorgan disease involving small-medium
                                                                      sized vessels in patients with
      Treatment:                                               allergies
           steroids and cytoxan
                                                                      and/or asthma.
                    or
           steroids and antiviral agents (if Hep)
                                                               Most common in middle age.
      Prognosis:
           5 year survival when treated =60%                   Male > Female




            Churg-Strauss                                               Churg-Strauss
(Allergic Granulomatosis/Angiitis) cont.                    (Allergic Granulomatosis/Angiitis) cont.
Presentation:
                                                               Labs:
                                                                       eosinophilia >10% WBC
Initially:
        1. Increased allergic manifestations (esp asthma)
                                                                       Inflammatory

      2. Eosinophilia (>10% WBC)                               Chest x-ray-
                                                                    transient patchy or nodular infiltrates
Eventually:
     Vasculitis (typically develops as asthma/allergies        Sinus x-ray-
     improve)- especially in sinopulmonary tree.                    opacification




                                                                                                                          16
             Churg-Strauss
 (Allergic Granulomatosis/Angiitis) cont.                           Giant Cell Arteritis

    Diagnosis:                                         Takayasu’s arteritis (pulseless disease) and
         Obtain biopsy of any involved tissue.         Temporal arteritis (cranial arteritis) are chronic
                                                            vasculitis of aorta and large branches.
    Prognosis:
         Better than PAN
                                                       Histologically identical granulomatous inflammatory
    Treatment:                                               diseases (with multinucleated giant cells) of
         steroids                                            large/muscular arteries.




Takayasu’s arteritis (pulseless disease)                         Takayasu’s arteritis cont.
  Epidemiology- young asian (esp. Japan) females.      Vascular insufficiency (aortic arch syndrome):
                                                            Asymmetric BP- with differential >10mm
  Early Sx (days-weeks):                               Hg.
                                                            Angina
             constitutional symptoms                        Aortic insufficiency 2o aortic root dilatation.
                                                                               y
             Erythema Nodosum                               Bruits.
                                                            CNS ischemic symptoms-
  Weeks-Months:                                                    headache
                                                                   dizziness
       Narrowing occurs in carotid, cerebral,                      amaurosis fugax
       aorta, mesenteric, and subclavian                           diplopia
       arteries resulting in vascular insufficiency.
                                                              Hypertension 2o renal artery stenosis




          Takayasu’s arteritis cont.                                 Temporal arteritis
     Labs:
          Inflammatory                                    >50 years old (mean age = 70 years old)
     Diagnosis:
          Angiogram or                                    Female:Male = 2:1
          Magnetic resonance angiography
     Prognosis:                                           Uncommon in black population.
          > 90% 5 year survival with treatment
     Treatment:                                           North >> South
          Steroids
          Angioplasty (if not inflamed)                   50% of patients have polymyalgia rheumatica
          Bypass surgery




                                                                                                              17
             Temporal arteritis cont.
Onset- Gradual (weeks-months) or may be sudden onset with:
             flu-like symptoms,
             fever to 104
             weight loss.
Symptoms 2o to vascular involvement:
      headache
      scalp tenderness + skin necrosis
      enlarged fibrotic tender temporal artery
      jaw/tongue claudication
      visual symptoms/loss/diplopia
      sore throat/cough

Aortic arch syndrome with asymmetric BP/bruits.

Peripheral neuropathy




             Temporal arteritis cont.                                  Wegener’s Granulomatosis
                                                                Necrotizing granulomatous vasculitis involving small
                                                                     arteries/arterioles/venules potentially affecting
        Labs: Inflammatory with elevated ESR                         all organs with classic triad of:

                                                                1.Upper
                                                                1 Upper respiratory airway involvement (sinus)
        Diagnosis: Temporal artery biopsy                       2.Lower respiratory airway involvement (pulmonary)
                                                                3. Focal segmental glomerulonephritis (renal)
        Treatment: Steroids
                                                                Young to middle-aged adults
                                                                Male > Female




            (c) 1972-2004 American College of                              (c) 1972-2004 American College of Rheumatology
            Rheumatology Clinical Slide Collection. Used with              Clinical Slide Collection. Used with permission.
                  i i




                                                                                                                              18
  Wegener’s Granulomatosis cont.
Labs:
        Inflammatory
        Hematuria/Proteinuria/RBC casts
        Anti-neutrophil cytoplasmic antibody (ANCA)-
              80% of patients have a C-ANCA with
              specificity against proteinase-3.
Ch t x-ray:
Chest
     Nodules and cavitary lesions.
Diagnosis:
     Open lung biopsy or biopsy of other tissues
Treatment:
     Steroids
     Cytoxan
     Methotrexate (in absence of life threatening
            lung or kidney disease)




ANCA Immunoflourescence Patterns                                 Red Flags of Vasculitis
 1. Perinuclear staining pattern (pANCA).
    >70% of pANCA is directed against                      1.   Fever of unknown origin
    myeloperoxidase (MPO). Other pANCA                     2.   Unexplained multisystem disease
    antigens include cathepsin G, human                    3.   Unexplained glomerulonephritis
    leukocyte elastase and lactoferrin.                    4.   Unexplained cardiac/GI/CNS ischemia
                              Churg Strauss.
    Associated with PAN and Churg-Strauss                  5.                       p
                                                                Mononeuritis multiplex
                                                           6.   Suspicious rash-
 2. Cytoplasmic staining pattern (cANCA).                                palpable purpura
    >90% of cANCA is directed against                                    maculopapular
    Proteinase 3 (PR3).                                                  nodules
    Associated with Wegener’s                                            ulcers
    Granulomatosus.                                                      livedo reticularis

 However, both patterns are found in many
   conditions.




                                                       A 36-year-old female is referred by her OB-GYN
                                                       because she experiences 5 years of chronic
                                                       diffuse MSK pain. She states that her hands and
                                                       fingers frequently swell, making it difficult to
                                                       remove her rings. She has gained 30 pounds over
                                                       the last year, and is concerned about fatigue and
                                                       poor non-refreshing sleep.
         Miscellaneous Topics
              and Pearls                               On Physical exam:
                                                       She looks uncomfortable. Her vital signs are
                                                       normal and her exam is unremarkable except
                                                       diffuse tenderness on palpation of her upper and
                                                       lower extremities.

                                                       Labs:
                                                       CBC and Chemistry profile are normal.




                                                                                                           19
                 Fibromyalgia                           Differential Diagnosis of Chronic Pain
  Non-inflammatory disorder                                      Anxiety/stress/depression
  Diffuse pain
  Fatigue                                                        Drug Toxicity
  Non-restorative sleep                                          Folate/B12 deficiency
                                                                 Hepatitis B or C
  9 symmetrical pairs of tender points. Patients                 HIV
  h          t f       i t for
  have 11 out of 18 points f > 3 months.
                                       th                        Hypo or h
                                                                 H              th idi
                                                                          hyperthyroidism
  Diagnosis of exclusion                                         Malignancy
                                                                 Multiple Sclerosis
  Treatment:                                                     Obstructive Sleep Apnea
  Stress reduction                                               Polymyalgia Rheumatica
  Exercise                                                         (PMR)
  Non-narcotic analgesics                                        Syphilis
  SSRI                                                           Vasculitis
  Low-dose tricyclic antidepressants at HS                       Vitamin D deficiency




        Polymyalgia Rheumatica
Women > Men
Extremely uncommen < 50 years old                        A 72-year-old male complains 40 years of back
                                                              pain and recently his legs developed
Acute onset symmetric myalgias of neck,                       achiness and felt as if they were filled with
shoulders, low back, hips, thighs and occasionally            concrete after walking 3 blocks.
trunk with an elevated ESR.
P ti t may have t bl with ADL
Patients       h    trouble ith ADLs.                    These symptoms resolve with sitting and resting
                                                              but return with activity.
Weight loss, anorexia, depression, malaise are
       common. Fevers would suggest GCA.                 On physical examination: he has normal pulses
Men may have hand edema.                                      without bruits and a nonfocal neurologic
                                                              examination.
Traditionally patients improve dramatically after a
       few days on 15- 20 mg/day of prednisone.
If they do not respond as expected, question the
       diagnosis.




 Spinal Stenosis vs Protruding Disc
 Prototypic spinal stenosis patient is:
             A little old man hunched over a             An 80-year-old male presents with recent onset
   shopping                                              right shoulder pain. He recalls no specific injury.
             cart to relieve his                         Active abduction of the shoulder causes
   pseudoclaudication,                                   significantly more pain than does passive
             lower extremity neuropathy or back          abduction
                                                         abduction.
   pain
             which radiates to the legs.                 Radiograph is unremarkable.
             It is relieved by back flexion (such as
             ascending stairs or sitting in a chair).
 A protruding disc is relieved by back extension
             (such as descending stairs) (or
   worsened
             by ascending stairs).
 LBP surgery is indicated only when the patients




                                                                                                               20
         Bursitis vs Impingement                                               Osteoporosis

 Bursitis worse with:                                       Diagnostic criteria*                Classification
             Active movement                                T > -1                      Normal
             When subacromial, middle arc of                T = -1 to -2.5              Osteopenia (low bone mass)
                   abduction is painful
                                                            T < -2.5                         p
                                                                                        Osteoporosis
 Supraspinatus tendon impingement worse with:               Osteoporosis +              Severe or established
      Passive, full flexion                                    fracture(s)              osteoporosis
      Passive, internal rotation
      Active external rotation of the shoulder                *Measured in "T scores." T score indicates the number of
                  against resistance                          standard deviations below or above the average peak
                                                              bone mass in young adults.




                  Osteoporosis
                                                                An 25 y/o woman complains of severe right
By convention, these diagnostic criteria only apply             knee, wrist and left ankle pain. She has
     to postmenopausal women and men > 50                       several vesiculopustular skin lesions on her
     years old.                                                 arms and legs.
Therefore, premenopausal women and men < 50
     years old cannot be diagnosed as osteopenic                On h i l            i ti    h has
                                                                O physical examination, she h
     or osteoporotic by DXA.                                    tenderness and swelling of the tendons of
                                                                her right wrist without synovitis.
The T score correlates with fracture risk and is used
     for diagnosis.
The BMD is used to assess the response to therapy.




   Disseminated Gonococcus (GC)                                               Septic Arthritis
Migratory arthritis or tenosynovitis which settles          Monoarthritis is usually Staph aureus.
             in either a single joint or multiple joints.              WBC>100,000
In women, it tends to occur at menstration or post                     Patients with RA may have polyarticular
             menstration.                                      septic
                                                                              joints.
                                     25%. Therefore
Joint fluid cultures are positive < 25% Therefore,
             usually diagnosed on vaginal, throat or        Sickle cell anemia – Salmonella or staph
  anal                                                      IV drug use – sternoclavicular or sacroiliac MRSA or
             culture.                                             Pseudomonas
Most common cause of monoarticular arthritis                Lyme arthritis usually requires appropriate
             in sexually active adults < 35 years old.                  exposure (endemic area), tick bite and
Treatment:                                                                    appropriate rash.
Intravenous ceftriaxone                                           + ELISA can be seen in SLE, RA, RMSF
                                                                  + Western blot is used to confirm the dx




                                                                                                                         21
                                                               Office-Based Miscellaneous
                                                         Carpal tunnel syndrome:
                                                           Pregnancy/hypothyroid/oral contraceptives
                                                                  dialysis-related amyloid/wrist synovitis.
                                                                  Median nerve distribution numbness- esp
                                                                        nocturnal
                                                         Dupuytren contractures:
                                                           Diabetes/ETOH
                                                           Fibrous thickening of flexor tendons of the fingers
                                                                    which cause palmar pain and limited
                                                            extension                 of fingers.
                                                         AVN/(Osteonecrosis):
                                                           Steroids/Alcoholism/SLE/HIV/Pancreatitis
                                                           Hip pain (groin or buttock pain) with normal
                                                                          x-ray initially.




     Office-Based Miscellaneous                                                 Pearls
Genetic collagen disorders:                               1) Ferritin levels > 10x normal — think Still’s Disease.
Osteogenesis imperfecta – blue sclera                     2) Rotator cuff injuries are the most common cause
                                                             of shoulder pain.
Pseudoxanthoma elasticum – angioid streaks
                                                          3) Bursitis or tendonitis causes pain with ACTIVE
Marfan syndrome – High arched palate                         movement of the joint.
                            Long limbs
                            Aortic root dilatation        4) Osteosarcoma is a rare, late manifestation of
                            Ectopia lentis                   Pagets Disease (1%).
                            Pneumothorax                  5) Baker’s cysts (knee) may mimic a DVT.
Ehlers-Danlos – hypermobility                             6) Morton’s neuroma causes burning
         Classic- joint dislocation                          paresthesias between the 3rd and 4th toes.
                 skin scarring
         Hypermobility- hyperelasticity                   7) 90% of acute back pain without neurologic
         Vascular- vascular rupture                          deficits improve within two months- no bed
                                                             rest




                      Pearls                                                    Pearls
6) Premenopausal women do not suffer from gout.
   However, If they have gout, evaluate for underlying      10) Usually 1 autoimmune rheumatologic disease
   causes: renal failure, high cell turnover state,                per patient.
   enzyme deficiency.                                       11) Gout and RA do NOT coexist.
7) No sulfa antibiotics in SLE patients.                    12) Acute gout and septic arthritis are VERY rare
8) Q i l                   i t d ith tendon
   Quinolones are associated with t d ruptures,  t                               reportable).
                                                                   (and probably reportable)
   especially in SLE patients.                              13) No single blood test diagnoses ANY
9) Diff Diagnosis for hemarthrosis:                             rheumatologic disease.
        a. Trauma (fracture, tendon/cartilage
           injury)
        b. Traumatic arthrocentesis
        c. PVNS
        d. Coagulopathy
        e. TB/Fungus




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