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- A disease characterized by granulomatous vasculitis involving multiple organs
- The characteristic triad of involvement includes the upper airway (e.g., otitis, sinusitis, nasal mucosa), lungs, and kidneys.
- Other organ systems involved include the skin, joints, and nervous system (peripheral/central).
- As the condition progresses untreated, upper airway erosions, necrotic pulmonary nodules, and renal failure are common.
- Without treatment, mortality rate is high. With treatment, survival rate is ~75–90% at 5 years.
- System(s) affected: upper airways (sinusitis, otitis); cardiovascular; gastrointestinal; nervous; pulmonary; renal/urologic; skin/exocrine
- Synonym(s): antibodies to neutrophilic cytoplasmic antigens (ANCA)-associated vasculitis (to include patients with Wegener and microscopic polyangiitis); granulomatosis with polyangiitis (recommended to replace Wegener as diagnostic term)
- ~0.4 to 1.2/100,000 persons per year and possibly increasing over the past decade
- Mean age of onset is the mid-40s, but the disease has been described in all age groups.
- Predominant sex: male > female (3:2)
Etiology and Pathophysiology
- Unknown etiology
- Autoimmune phenomena and immune-complex deposition in arterial walls are implicated, and the activation of neutrophils by bacteria or other infectious agents may be important as well.
- Role of antibodies directed against neutrophils is currently being investigated.
- A specific triggering infectious agent has not been identified.
Increased presence in HLA-B8 and HLA-DR2, more recently increase in HLA-DP and genetic variants involved in α1-antitrypsin and protease-3 (1)