Eosinophilic granulomatosis with polyangiitis

This review aims to describe the epidemiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of eosinophilic granulomatosis with polyangiitis (EGPA). Eosinophilic granulomatosis with polyangiitis is a small to medium vessel necrotizing vasculitis, typically classified with granulomatosis with polyangiitis (GPA) and microscopic polyangitis (MPA) as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, less than 50% of patients with EGPA have a positive ANCA test. Among all the vasculitides, asthma and eosinophilia are unique features of EGPA. Eosinophilic granulomatosis with polyangiitis is very rare and the diagnosis may be missed as the disease evolves over time. Polyneuropathies are common and may be severe, requiring aggressive immunosuppressive therapy. Heart involvement is the most common cause of death in EGPA. Biopsy of involved tissue supports a clinically suspected diagnosis but is not always feasible. Treatment of EGPA is primarily dictated by the severity of disease and prognostic factors. More severe disease frequently requires the use of aggressive therapy such as cyclophosphamide. Once treatment is initiated, patients can achieve good control of symptoms; unfortunately, disease relapses are common and prolonged treatment with corticosteroids is often necessary for asthma management. A better understanding of the disease heterogeneity is needed for the development of better therapies.

Automated summary

This review provides an overview of the epidemiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of eosinophilic granulomatosis with polyangiitis (EGPA). EGPA is a rare small to medium vessel necrotizing vasculitis that is often misdiagnosed due to its evolving nature. The unique features of EGPA include asthma and eosinophilia. The disease commonly presents with polyneuropathies and heart involvement, which are often severe and require aggressive immunosuppressive therapy. The treatment approach depends on the severity of the disease, with more severe cases requiring aggressive therapies such as cyclophosphamide. Asthma management often requires prolonged treatment with corticosteroids due to the high rate of disease relapses. Improved understanding of the heterogeneity of the disease is necessary for the development of better therapies.