Grau Rafael G
Indiana University School of Medicine, Division of Rheumatology, 1110 W. Michigan Street, LO-545, Indianapolis, IN 46202, USA.
Curr Rheumatol Rep. 2008 Dec;10(6):453-8. doi: 10.1007/s11926-008-0074-x.
Churg-Strauss syndrome (CSS) is a rare necrotizing small-vessel vasculitis associated with eosinophil-rich granulomatous inflammation of tissues and vessels and is also associated with asthma and eosinophilia. Epidemiologic studies continue to show that CSS is the rarest of the necrotizing small-vessel vasculitides. However, it is not possible to know with any certainty if there has been an increase in incidence. There has been an attempt to divide the patients with CSS into an antineutrophil cytoplasmic antibody-positive and cytoplasmic antibody-negative group. The former group has an increased frequency of renal involvement, parenchymal pulmonary disease, constitutional symptoms, and peripheral and central nervous system involvement, whereas the latter group has more frequent cardiac disease. The role of eosinophils and antineutrophil cytoplasmic antibodies remains poorly defined but provocative. Leukotriene receptor antagonists do not appear to induce CSS but facilitate the tapering of glucocorticoids, which unmasks the condition. Glucocorticoids and cyclophosphamide remain the foundation of treatment for vasculitis, but there are other promising and less toxic alternatives on the horizon.
变应性肉芽肿性血管炎（CSS）是一种罕见的坏死性小血管炎，与富含嗜酸性粒细胞的组织和血管肉芽肿性炎症相关，还与哮喘和嗜酸性粒细胞增多有关。流行病学研究持续表明，CSS是坏死性小血管炎中最罕见的一种。然而，无法确切知晓其发病率是否有所上升。曾有人尝试将CSS患者分为抗中性粒细胞胞浆抗体阳性组和阴性组。前一组肾脏受累、实质性肺部疾病、全身症状以及外周和中枢神经系统受累的频率增加，而后一组心脏病更为常见。嗜酸性粒细胞和抗中性粒细胞胞浆抗体的作用仍未明确，但颇具启发性。白三烯受体拮抗剂似乎不会诱发CSS，但有助于糖皮质激素逐渐减量，从而使病情显现出来。糖皮质激素和环磷酰胺仍是血管炎治疗的基础，但未来还有其他有前景且毒性较小的替代方案。
