- Necrotizing systemic vasculitis of capillaries, venules and medium-sized arteries
- Skin: Palpable purpura, splinter hemorrhages and ulcers
- Kidney: segmental & crescentic glomerulonephritis
- Lung: alveolar hemorrhage
- Constitutional symptoms
- p-ANCA (60%) > c-ANCA (30%) [main difference from Wegener]
Treatment and prognosis
- Treatment similar to granulomatosis with polyangiitis
- Initial: corticosteroids nd cyclophosphamide
- Maintenance: MTX, azathioprine, mycophenolate mofetil
- IVIg and anti TNF for refractory cases
- However, relapses frequent
- 5 yr survival 75%; 7yr: 62%
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