musculoskeletal and connective tissue disorders
Eosinophilic Granulomatosis with Polyangiitis (EGPA)
Introduction
Eosinophilic granulomatosis with polyangiitis is a systemic small- and medium-vessel necrotizing vasculitis, characterized by extravascular granulomas, eosinophilia, and tissue infiltration by eosinophils. It occurs in people with adult-onset asthma, allergic rhinitis, nasal polyposis, or a combination. Diagnosis is best confirmed by biopsy. Treatment is primarily with corticosteroids plus another immunosuppressant. (See also Overview of Vasculitis .) Eosinophilic granulomatosis with polyangiitis (EGPA) occurs in about 3 people/million. Mean age at onset is 48. EGPA is characterized by extravascular necrotizing granulomas (usually rich in eosinophils), eosinophilia, and tissue infiltration by eosinophils. However, these abnormalities do not always coexist. The vasculitis typically affects small- and medium-sized arteries. Any organ can be affected, but the lungs, skin, sinuses, cardiovascular system, kidneys, peripheral nervous system, central nervous system, joints, and gastrointestinal (GI) tract are most commonly affected. Rarely, pulmonary capillaritis may cause alveolar hemorrhage. 