Rheumatology wk3

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Rheumatology wk3
2011-04-18 15:02:00
scleroderma vasculitis SLE Sjogren

scleroderma and vasculitis
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  1. systemic sclerosis (scleroderma)
    • excessive extracellular matrix deposition
    • result of autoimmunity, vasculopathy, (early stages) and fibrosis (late stage)
    • limited and diffuse forms
    • vascular: endothelial cell damage/apoptosis, smooth muscle and pericyte proliferation leading to increased thickness, ROS, hypoxemia, vascular loss
    • fibrotic: fibroblast differentiation to myofibroblasts (contractile cells), collagen overproduction, increased deposition, remodeling
  2. limited systemic sclerosis
    • skin thickening/tightening only distal extremities (+face/neck)
    • 60% of cases
    • longstanding Raynaud's that can proceed onset
    • mild fatigue and athralgias
    • isolated pulmonary HTN, esophageal disease, telengectasias, calcinosis
    • anticentromere antibodies
  3. diffuse systemic sclerosis
    • 30% of cases
    • skin thickening/tightening over whole body
    • Raynaud's onset w/disease, sudden
    • more severe fatigue, arthralgias, tendon friction rubs often present
    • renal disease, interstitial lung disease, severe/diffuse GI, cardiac disease
    • anti-Scl-70 antibodies
  4. Diagnosis of systemic sclerosis
    • nailfold capillaroscopy: capillary loop dilatation and capillary loss
    • digital tip ulcerations secondary to ischemia, digital pits/scars, gangrene, distal phalangeal tuft resorption
    • anti-centromere Ab (for limited)
    • anti-Scl-70 Ab (for diffuse)
  5. Vasculitis
    • inflammation/autoimmunity targeted to endothelium
    • occlusion of vessel due to thrombosis or loss of integrity
    • immune complex deposition, ADCC, direct immune cell cytotoxicity
  6. small vessel vasculitis
    • neuropathy
    • non-blanching paplable purpuric lesions due to RBC extravasation
    • RBC casts due to glomeruli damage
    • Antineutrophil antibody (ANCA): activate neutrophils
  7. medium vessel vasculitis
    • skin ulcer secondary to occlusions
    • anneurysmal dilatations/stenosis
    • post-prandial pain, GI bleed, perforation
  8. large vessel vasculitis
    • Giant cell arteritis: disrupted internal elastic membrane, thickened temporal artery, limb claudication (ischemic pain w/exertion
    • Takavasu's arteritis: limb claudication, thickening of carotids
  9. SLE (systemic lupus erythermatosus): clinical presentation
    • Malar rash: sparing of nasolabial folds
    • Discoid rash: follicular plugging, raised
    • Photosensitivity: UV
    • Oral/nasal ulcers: painless
    • Arthritis: nonerosive, 2+ joints
    • Serositis: pleuritis or pericarditis
    • Renal disorder: proteinuria > 0.5mg/dL, 3+ casts
    • Neurologic disorder: seizures/psychosis
    • Hematologic: anemia, leukopenia, thrombocytopenia, lymphopenia
    • Immunologic: anti-dsDNA, anti-Sm, anti-PL
    • Antinuclear antibodies: ANA titer w/immunofluoresence
    • 4 criteria present = 98% specificity, 97% sensitivity
    • Class III, IV most severe
    • > anti-dsDNA, < C3/C4 = more severe
  10. What is ADCC?
    What are depleting/neutralizing antibodies?
    • Antibody-dependent cellular toxicity
    • antibodies directed against self-antigens, result in cell death
    • depleting/neutralizing Abs against plasma cells/proteins - can form immune complexes
    • Found in SLE
  11. SLE: epidemiology and onset
    • 51/100K in US, 9:1 female:male adults, 3:1 female:male children (sex hormone's role?)
    • 16-55YO, African Americans, Hispanic
    • UV light exposure, drugs
  12. SLE: ANA associations
    • ANA: useful to rule out SLE, need subsets for diagnosis
    • dsDNA: correlates w/disease activity, nephritis, specific and sensitive
    • histones: drug-induced lupus
    • Ro(SS-A): neonatal lupus, subacute cutaneous lupus
    • La(SS-B): neonatal lupus also
    • Sm: no associations, but very specific for SLE!
  13. SLE treatment
    NSAIDs, steroids, antimalarials, immunosuppressives
  14. Sjogren's Syndrome: pathophysiology
    • lymphocytic infiltration (CD4+, B cells) of exocrine glands
    • extraglandular involvement: arthritis, B cell lymphoma, lymphocytic infiltration of organs, fatigue
    • ANA, RF, anti-SSA, anti-SSB are not sensitive or specific diagnositically
  15. Sjogren's syndrome: glandular involvement
    • dry mouth: dental problems, dysphagia, difficulty speaking in 90% of pts
    • dry eyes (xeroophthalmia): keratoconjuctiva sicca - gritty sensation 95%
    • dry tracheobronchal mucosa (xerotrachea): hoarseness, bronchitis
    • dry skin, vaginal mucosa: puritis, increased infections
    • major salivary gland involvement
    • pancreatic exocrine, hypochlorhydria: epigastric pain, dyspepsia, atrophic gastritis