医学
肉芽肿伴多发性血管炎
嗜酸性
显微镜下多血管炎
血管炎
坏死性血管炎
嗜酸性粒细胞增多症
美波利祖马布
抗中性粒细胞胞浆抗体
皮肤病科
疾病
哮喘
病理
内科学
嗜酸性粒细胞
标识
DOI:10.1080/00325481.2022.2134624
摘要
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.
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