Hellmich Bernhard, Gross Wolfgang L
Poliklinik für Rheumatologie, Universitätsklinikum Schleswig-Holstein, Ratzeburger Allee 160, 23538 Lübeck, Germany.
Expert Opin Pharmacother. 2004 Jan;5(1):25-35. doi: 10.1517/14656566.5.1.25.
Churg-Strauss syndrome (CSS) is a primary systemic vasculitis occurring primarily in patients with asthma. Unlike other small vessel vasculitides, CSS is characterised by blood and tissue eosinophilia. Corticosteroids are the therapy of first choice for all stages of the disease when active vasculitis needs to be treated rapidly. In patients with severe disease and organ- or life-threatening manifestation, the addition of cyclophosphamide appears to improve the outcome and reduces the incidence of relapses. In cases with an apparently better prognosis and less severe disease, methotrexate can be given as a corticosteroid-sparing agent in order to reduce the cumulative dose of corticosteroids, which is generally high in most cases as long-term administration of corticosteroids is often inevitable in order to control asthma, even if the vasculitis is inactive. In very severe cases of CSS, cyclophosphamide and corticosteroids may be insufficient to induce remission. In these cases, anti-TNF blocking agents such as infliximab or etanercept, may be added for a limited period of time. As this intense immunosuppression increases the risk for infections, a prophylaxis with sulfamethoxazole/trimethoprim is advised. Alternatively, the administration of recombinant IFN-alpha can be a effective when given on a short-term basis in otherwise refractory cases. Whether a continuous administration of immunosuppressive agents in addition to corticosteroids can reduce the frequency of relapses in CSS who are in remission is still unknown. As relapses occur in > 25% of all patients, studies addressing the prevention of relapses in CSS are highly desirable in the future.
变应性肉芽肿性血管炎（CSS）是一种主要发生于哮喘患者的原发性系统性血管炎。与其他小血管血管炎不同，CSS的特征是血液和组织嗜酸性粒细胞增多。当需要快速治疗活动性血管炎时，糖皮质激素是该病各阶段的首选治疗方法。对于患有严重疾病以及有器官或生命威胁表现的患者，加用环磷酰胺似乎可改善预后并降低复发率。对于预后明显较好且疾病不太严重的病例，可给予甲氨蝶呤作为糖皮质激素节约剂，以减少糖皮质激素的累积剂量，在大多数情况下，由于为控制哮喘通常不可避免地要长期使用糖皮质激素，即使血管炎处于非活动状态，糖皮质激素累积剂量一般也很高。在CSS的非常严重的病例中，环磷酰胺和糖皮质激素可能不足以诱导缓解。在这些情况下，可在有限时间内加用抗TNF阻断剂，如英夫利昔单抗或依那西普。由于这种强烈的免疫抑制会增加感染风险，建议使用磺胺甲恶唑/甲氧苄啶进行预防。或者，在其他难治性病例中短期给予重组干扰素-α可能有效。除糖皮质激素外持续给予免疫抑制剂是否能降低CSS缓解期患者的复发频率仍不清楚。由于超过25%的患者会复发，未来非常需要开展针对预防CSS复发的研究。
