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SYSTEMIC VASCULITIDES

SYSTEMIC VASCULITIDES. Susan F. Massengill, MD January 15, 2002. Vasculitis. Inflammatory, destructive process affecting arteries and veins Vessels may be small, medium or large PMNs, lymphocytes or eosinophils Necrosis or granulomas

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SYSTEMIC VASCULITIDES

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  1. SYSTEMIC VASCULITIDES Susan F. Massengill, MD January 15, 2002

  2. Vasculitis • Inflammatory, destructive process affecting arteries and veins • Vessels may be small, medium or large • PMNs, lymphocytes or eosinophils • Necrosis or granulomas • May be focal (skin or single organ), BUT usually SYSTEMIC (skin and multiple organs)

  3. Common Presentatations

  4. Diagnostic Assessment • Labs: • ANCA • ANA (10-20% positive, but rarely dsDNA) • Complement studies, • RF • HBV and HCV • Lytes, BUN, Cr, urinalysis

  5. Antineutrophil Cytoplasmic Autoantibodies • Specific for neutrophils and monocytes • Two major staining patterns with indirect imunofluorescence • C-ANCA Proteinase 3 • P-ANCA Myeloperoxidase

  6. Diagnostic Assessment • Labs: • ANCA • ANA (10-20% positive, but rarely dsDNA) • Complement studies, • RF • HBV and HCV • Lytes, BUN, Cr, urinalysis

  7. Diagnostic Assessment • Radiology: • CXR • Sinus films • Chest CT • Histology (skin, kidney, lung)

  8. Small Vessel Vasculitis

  9. Henoch-Schonlein Purpura • Most common SVV seen in children (ANCA neg) • Peak incidence at 5 yrs, 2:1 male preponderance • URI prodrome (33%) • Palpable purpura, arthralgias, colicky abdominal pain • Renal involvement • Hematuria and proteinuria (50%) • ARF (<20%) • Pulmonary and peripheral neuropathy uncommon • Testicular/scrotal hemorrhage and edema

  10. Wegener’s Granulomatosis • C-ANCA • Necrotizing granulomatous inflammation • Upper/lower respiratory tract disease (90%) • Sinusitis, nasal ulcerations or erosions, saddle-nose deformity, subglottic stenosis, orbital pseudotumor • Glomerulonephritis (80%) • Tx: aggressive high dose steroids and cytoxan

  11. Microscopic Polyangiitis • P-ANCA • Non-granulomatous • Glomerulonephritis (90%) • Pulmonary-renal syndrome common • Tx: aggressive combination high dose steroids and cytoxan

  12. Churg-Strauss Syndrome • P-ANCA • Granulomatous • History of asthma and eosinophilia • Severe renal disease uncommon • Histologically identical to microscopic polyangiitis and Wegener’s

  13. Systemic Lupus Erythematousus

  14. “Classical” vs. “Non-classical” Lupus is defined by its clinical picture, together with antibodies directed against one or more nuclear components, particularly anti-double stranded DNA. However, a considerable number of patients are excluded who belong to the lupus “family,” but who do not satisfy strict criteria.

  15. Origins of Autoimmunity • Genetic factors • race, familial clustering, complement deficiencies • Female phenotype • Infective agents • Medicines

  16. GREATEST RISK • Delayed Diagnosis by clinician • Young children • Male child

  17. Diagnosis • High index of suspicion • Proteinuria • Membranous nephropathy • Henoch-Schonlein Purpura • Vasculitis

  18. Clinical Manifestations • Fever, malaise and weight loss (80-90%) • Rash (50%) • Arthritis (40-60%) • Nephritis (35-85%)

  19. Diagnosis • ANA (+) • “Lupus-like” patients rare • Anti-Sm antibodies (30%) • Hypocomplementemia (75%) • C4 and C1q >> C3 • ANCA

  20. Cutaneous • Malar rash • Vasculitis • hard palate • extremities, pressure points, ears, fingers • Photosensitivity

  21. Annular Papulosquamous Neonatal C2 deficiency SCLE SLE Malar rash Photosensitivity Bullous LE Generalized erythema ACLE CCLE-DLE Discoid LE Hypertrophic LE Lupus Panniculitis

  22. Arthritis/Arthralgias • occurs in 40-60% • polyarticular, symmetrical • small joints of hands, knees, ankles • pain out of proportion to exam • Non-deforming

  23. Renal Manifestations • Presenting feature in  25%, although 60% of adults and 80% of children later develop overt renal abnormalities • Proteinuria* • Microscopic hematuria • Hypertension (+/-) • Renal dysfunction (50%)

  24. Renal Histology • Renal biopsy worthwhile in all patients with SLE who have abnormal urine and/or reduced renal function • black race • Marked variability • Renal histology cannot be predicted from clinical picture • If untreated, WHO class predictive • Interstitial changes predictive • Transformation

  25. Therapeutic Interventions • Induction Phase • severe acute life threatening disease • Maintenance Phase • chronic disease • side effects of treatment • overtreatment vs. undertreatment

  26. PANCYTOPENIA GRANULOCYTOPENIA Sepsis Drug reaction Peripheral destruction ANEMIA Chronic disease Red cell aplasia Hypoplastic bone marrow Autoimmune hemolytic anemia THROMBOCYTOPENIA Sepsis Anti-platelet antibodies Lupus anticoagulant Antiphospholipid Abs

  27. Pulmonary • Pleuritis • Lupus pneumonitis • Pulmonary vasculitis • Infection • Pulmonary embolus • Pulmonary Hypertension

  28. Neurologic • CVA (hemorrhagic, embolic, thrombotic) • aPLs • hypercoagulability • vasculitis • Infection • Seizures • Movement disorders • HTN • Neuropathies • Organic brain syndrome/Psychosis • Withdrawal of Rxs (EtOH, steroids, illicit drugs)

  29. Cardiac • Pericarditis (25%) • Usually part of generalized serositis • Chest pain, cough, dyspnea, rub • Myocarditis (10%) • Libman-Sacks Endocarditis (15%) • tricuspid > mitral • Coronary Artery Disease / MI

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