Guillevin Loïc
Assistance publique-Hôpitaux de Paris, hôpital Cochin, université Paris-Descartes, service de médecine interne, Paris, France.
Presse Med. 2012 Oct;41(10):1004-13. doi: 10.1016/j.lpm.2012.07.008. Epub 2012 Aug 23.
Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss), is a rare necrotizing vasculitis of small-sized vessels, associated to antimyeloperoxydase ANCA in 40% of patients. EGPA occurs in patients with asthma. Asthma is sever, associated with eosinophilia and extrapulmonary symptoms. Among them, mononeuritis multiplex is the most frequent symptom. When cardiac involvement is present, prognosis is poor. Despite a good overall prognosis, deaths are caused by vasculitis activity, gastrointestinal and cardiac involvement. Treatment is well codified based on steroids, which are quickly effective. Immunosuppressants combined with corticosteroids are compulsory to treat the most sever forms, mainly when cardiac and gastrointestinal or renal symptoms are present.
嗜酸性肉芽肿性多血管炎（EGPA）（韦格纳肉芽肿）是一种罕见的小血管坏死性血管炎，40%的患者与抗髓过氧化物酶抗中性粒细胞胞浆抗体（ANCA）相关。EGPA发生于哮喘患者。哮喘严重，伴有嗜酸性粒细胞增多和肺外症状。其中，多发性单神经炎是最常见的症状。当出现心脏受累时，预后较差。尽管总体预后良好，但死亡是由血管炎活动、胃肠道和心脏受累引起的。基于类固醇的治疗方案已得到很好的规范，类固醇起效迅速。对于最严重的类型，主要是出现心脏、胃肠道或肾脏症状时，必须联合使用免疫抑制剂和皮质类固醇进行治疗。
