II. Epidemiology

  1. Most common ANCA-associated Small Vessel Vasculitis
  2. Age of onset peaks at 40 to 60 years
  3. Gender: Men more commonly affected

III. Symptoms

  1. Fever
  2. Arthralgias and Myalgias
  3. Cough (Hemoptysis may be present)

IV. Signs

  1. Renal involvement most common (Glomerulonephritis)
  2. Palpable Purpura
  3. Lung disease (Pulmonary Hemorrhage)

V. Differential Diagnosis

  1. Polyarteritis Nodosa
  2. ANCA-associated Small Vessel Vasculitis
    1. Especially pulmonary-renal syndromes
    2. Granulomatosis with Polyangiitis (previously known as Wegener's Granulomatosis)
    3. Systemic Lupus Erythematosus
    4. Anti-glomerular basement membrane disease

VI. Labs

  1. Antineutrophil Cytoplasmic Antibody (ANCA)
    1. pANCA (MPO-ANCA) positive in 60-85% of cases
    2. cANCA (PR3-ANCA) positive in 40% of cases

VII. Diagnostics

  1. Biopsy shows minimal to no immune vascular deposits

VIII. Management

IX. References

  1. Calabrese in Ruddy (2001) Kelly's Rheum, p. 1176-8
  2. Schoen in Cotran (1999) Robbins Pathology, p. 521-2

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