Recent progress in the pharmacotherapy of Churg-Strauss syndrome
Recent progress in the pharmacotherapy of Churg-Strauss syndrome
复制标题
Churg-Strauss综合征的药物治疗新进展
DOI：
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发表时间：
2004
影响因子：
3.2
通讯作者：
W. Gross
中科院分区：
文献类型：
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作者：
B. Hellmich;W. Gross
Churg-Strauss综合征（CSS）是一种主要发生于哮喘患者的原发性系统性血管炎。与其他小血管炎不同，CSS的特征是血液和组织嗜酸性粒细胞增多。当活动性血管炎需要迅速治疗时，皮质类固醇是疾病各个阶段的首选治疗方法。在患有严重疾病和危及器官或生命的表现的患者中，添加环磷酰胺似乎可以改善结局并降低复发率。在预后明显较好且病情较轻的病例中，甲氨蝶呤可作为皮质类固醇保留剂给予，以减少皮质类固醇的累积剂量，在大多数病例中，皮质类固醇的累积剂量通常较高，因为长期给予皮质类固醇通常是不可避免的，以控制哮喘，即使血管炎是非活动性的。在非常严重的CSS病例中，环磷酰胺和皮质类固醇可能不足以诱导缓解。在这些情况下，可以在有限的时间内添加抗TNF阻断剂，如英夫利西单抗或依那西普。由于这种强烈的免疫抑制增加了感染的风险，因此建议使用磺胺甲恶唑/甲氧苄啶进行预防。或者，重组IFN-α的管理可以是一个有效的，当在短期的基础上，否则难治性病例。在缓解期的CSS患者中，除皮质类固醇外，持续给予免疫抑制剂是否可以降低复发频率仍是未知数。由于复发发生在> 25%的所有患者中，因此将来非常需要解决CSS复发预防的研究。
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-α can be a effective when given on a short-term basis in otherwise refractory cases. Whether a continous 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.
环磷酰胺治疗血管炎的感染性并发症。
影响因子：
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作者：
Bradley,JD;Brandt,KD;Katz,BP
通讯作者：
Katz,BP