Treatment of Churg-Strauss syndrome without poor-prognosis factors
Treatment of Churg-Strauss syndrome without poor-prognosis factors
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DOI：
10.1002/art.23198
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发表时间：
2008-02-01
影响因子：
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通讯作者：
Guillevin, Loic
中科院分区：
文献类型：
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作者：
Ribi, Camillo;Cohen, Pascal;Guillevin, Loic
目标。目的评价全身糖皮质激素(CS)作为一线治疗对无不良预后因素的Churg-Strauss综合征(CS)患者的疗效，并比较口服硫唑嘌呤(AZA)和静脉注射环磷酰胺(CyC)作为辅助免疫抑制治疗失败或复发的有效性和安全性。这项多中心、前瞻性、随机、开放标签的治疗试验包括72名新诊断的CS患者(FFS为0)，仅接受CS治疗。在治疗失败或复发时，患者随机接受6个月的口服AZA或6个周期的环磷酰胺。根据意向治疗策略进行分析。平均随访+/-SD为56.2+/-31.7个月。在被研究的72名患者中，93%的患者仅接受CS治疗就获得了缓解，35%的患者复发，主要是在治疗的第一年。在因治疗失败或复发而随机接受额外免疫抑制的19例患者中，10例接受AZA治疗的患者中有5例缓解，9例接受环磷酰胺冲击治疗的患者中有7例缓解，但差异无统计学意义。1、5年生存率分别为100%和97%。在随访结束时，79%的缓解期患者需要小剂量的CS治疗，主要是为了控制呼吸道疾病。72例患者中有31%发生了与CS相关的不良事件。在FFS为0的CS患者中，生存情况良好，证实了FFS在本病中的预测价值。CS的一线治疗在大多数患者中实现了缓解，但复发很常见，其中三分之一需要额外的免疫抑制治疗。AZA或PULSE CyC在治疗CS耐药疾病或重大复发方面相当有效。从长期来看，大多数患者继续服用CS，这可能解释了CS相关不良事件的高发生率。
Objective. To assess the efficacy of systemic corticosteroids (CS) alone as first-line treatment in patients with Churg-Strauss syndrome (CSS) without poor-prognosis factors, as defined by the Five-Factors Score (FFS), and to compare the efficacy and safety of oral azathioprine (AZA) versus intravenous pulse cyclophosphamide (CYC) as adjuvant immunosuppressive therapy for treatment failure or relapse.Methods. This multicenter, prospective, randomized, open-label therapeutic trial included 72 patients with newly diagnosed CSS (FFS of 0) treated with CS alone. At treatment failure or relapse, patients were randomized to receive 6 months of oral AZA or 6 pulses of CYC. Analyses were performed according to an intent-to-treat strategy.Results. The mean +/- SD followup was 56.2 +/- 31.7 months. Among the 72 patients studied, 93% achieved remission with CS therapy alone, and 35% relapsed, mainly during the first year of treatment. Among the 19 patients randomized to additional immunosuppression because of treatment failure or relapse, 5 of 10 receiving AZA and 7 of 9 receiving pulse CYC achieved remission, but the difference was not statistically significant. Survival rates in all patients at 1 and 5 years were 100% and 97%, respectively. At the end of followup, 79% of the patients whose disease was in remission required low-dose CS therapy, mainly to control respiratory disease. CS-related adverse events were observed in 31% of the 72 patients.Conclusion. In CSS patients with an FFS of 0, survival was excellent, confirming the predictive value of the FFS in this disease. First-line therapy with CS achieved remission in most patients, but relapses were common, and one-third of them required additional immunosuppressive therapy. AZA or pulse CYC was fairly effective in treating CS-resistant disease or major relapses. Over the long term, most patients continued to take oral CS, which might explain the high rate of CS-related adverse events.