Lhote François
Service de médecine interne, Centre hospitalier, Saint-Denis, France.
Presse Med. 2007 May;36(5 Pt 2):875-89. doi: 10.1016/j.lpm.2007.02.018. Epub 2007 Apr 3.
Churg-Strauss syndrome is a systemic and pulmonary vasculitis, defined by its association with severe asthma and with hypereosinophilia of the blood and tissues. The systemic vasculitis is a small-vessel vasculitis frequently associated with purpura, mononeuritis multiplex, and, more rarely, with rapidly progressive glomerulonephritis or diffuse alveolar hemorrhage. Its prevalence of 7 to 13 per million population makes it one of the rarest of the systemic vasculitides. Anti-MPO (antimyeloperoxidase) pANCA (ANCA with a perinuclear fluorescence pattern) is present in 35-40% of cases and appears to determine a subgroup of patients with a higher frequency of renal damage, alveolar hemorrhage, and central nervous system damage. Cardiac involvement is an important cause of morbidity and the leading cause of mortality in Churg-Strauss syndrome. Treatment is based on corticosteroid therapy and immunosuppressive drugs (cyclophosphamide and azathioprine) and is determined according to validated prognostic criteria (Five-Factor Score). Complete remission occurs in almost 90% of cases, and the 10-year survival rate has reached 79.4%. Relapses are frequent (25% of cases) and even after recovery from vasculitis, most patients (90%) still have asthma requiring corticosteroid treatment.
变应性肉芽肿性血管炎是一种系统性和肺部血管炎，其定义为与重度哮喘以及血液和组织嗜酸性粒细胞增多相关。系统性血管炎是一种小血管血管炎，常与紫癜、多发性单神经炎相关，较少与快速进展性肾小球肾炎或弥漫性肺泡出血相关。其在每百万人口中的患病率为7至13例，使其成为最罕见的系统性血管炎之一。35%至40%的病例存在抗髓过氧化物酶（MPO）的核周型抗中性粒细胞胞浆抗体（pANCA），且似乎决定了一组肾损害、肺泡出血和中枢神经系统损害发生率较高的患者亚组。心脏受累是变应性肉芽肿性血管炎发病的重要原因及主要死亡原因。治疗基于糖皮质激素疗法和免疫抑制药物（环磷酰胺和硫唑嘌呤），并根据经过验证的预后标准（五因素评分）来确定。几乎90%的病例可实现完全缓解，10年生存率已达79.4%。复发很常见（25%的病例），即使从血管炎中恢复后，大多数患者（90%）仍有需要糖皮质激素治疗的哮喘。
