musculoskeletal and connective tissue disorders
Eosinophilic Granulomatosis with Polyangiitis (EGPA)
Key Points
Eosinophilic granulomatosis with polyangiitis is a rare small- and medium-sized vessel vasculitis. Phases include upper respiratory symptoms and wheezing, eosinophilic pneumonia and gastroenteritis, and life-threatening vasculitis. Phases may occur in or out of order and may overlap. Cardiac or neurologic involvement can occur and indicate a poor prognosis. Diagnose by clinical criteria, routine laboratory testing, and sometimes biopsy. Do a 2D echocardiogram in all patients. Treat with corticosteroids and other immunosuppressants, based on severity of disease, and use the same criteria for treatment of granulomatosis with polyangiitis or microscopic polyangiitis. Consider treatment withrituximabfor its possible high rates of response and reduced requirement for corticosteroids. Considermepolizumabin relapsing or refractory disease with primarily upper and/or lower respiratory tract manifestations. 