Eosinophilic granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic small- and medium-vessel vasculitis characterized by asthma, peripheral and tissue eosinophilia, and necrotizing granulomatous inflammation. It belongs to the group of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, though only about 30-40% of patients are ANCA-positive (typically perinuclear ANCA targeting myeloperoxidase). EGPA affects multiple organ systems and typically progresses through three phases, though not all patients follow this sequence. The prodromal phase features allergic disease including asthma and allergic rhinitis or nasal polyposis. The eosinophilic phase involves marked peripheral blood eosinophilia and eosinophilic tissue infiltration, particularly affecting the lungs and gastrointestinal tract. The vasculitic phase involves systemic small-vessel vasculitis that can affect the heart, peripheral nerves, skin, kidneys, and gastrointestinal tract. Cardiac involvement (myocarditis, cardiomyopathy, pericarditis) is the leading cause of mortality. Peripheral neuropathy, particularly mononeuritis multiplex, is one of the most common manifestations. Skin lesions including purpura and nodules are frequently observed. Treatment depends on disease severity. Glucocorticoids are the cornerstone of therapy and are effective in many patients. For severe or refractory disease, immunosuppressive agents such as cyclophosphamide or azathioprine are used. Mepolizumab, an anti-interleukin-5 monoclonal antibody, has been approved specifically for EGPA and has shown efficacy in reducing relapses and allowing glucocorticoid tapering. Rituximab is also used in refractory cases. With appropriate treatment, many patients achieve remission, though relapses are common and long-term management is often required.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Eosinophilic granulomatosis with polyangiitis