Overview
Definition
Necrotizing granulomatous inflammation of small/medium vessels (formerly Wegener's).
Epidemiology
Rare, incidence ~10 per 1 million.
Etiology & Risk Factors
- ANCA-associated autoimmunity
Clinical Symptoms
- Upper respiratory (sinusitis, saddle nose)
- Lower respiratory (hemoptysis, lung nodules)
- Renal (hematuria)
Clinical Approach
Diagnosis
- c-ANCA (PR3-ANCA) positive
- Tissue biopsy (granulomas and vasculitis)
Management
- High-dose Corticosteroids
- Cyclophosphamide
- Rituximab
Complications
- Rapidly progressive glomerulonephritis
- Alveolar hemorrhage
- Death if untreated