A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients
A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients
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DOI：
10.1136/annrheumdis-2012-201531
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发表时间：
2013-06-01
影响因子：
27.4
通讯作者：
Gross, Wolfgang L.
中科院分区：
文献类型：
--
作者：
Moosig, Frank;Bremer, Jan Phillip;Gross, Wolfgang L.
目的评价血管炎转诊中心对嗜酸性肉芽肿和多血管炎（Churg-Strauss，EGPA）的治疗策略。纳入了1990年至2009年收治的所有EGPA患者。对诊断证据、疾病程度指数和伯明翰血管炎活动评分进行了结构化的跨学科检查。开始免疫抑制治疗并定期调整。根据欧洲抗风湿联盟和欧洲血管炎研究组给出的定义，治疗目标是诱导和维持缓解。结果269例疑似EGPA患者中，150例符合纳入标准。其中，104人进行了一次以上的随访，平均随访时间为53 +/- 4.9个月。通过使用其他数据源，将生存期随访延长至92 +/- 5个月。严重的器官表现发生在心脏（46%），肾脏（18%）和肺（10%）。107例患者（71%）使用环磷酰胺。所有患者的泼尼松龙剂量均在目标范围内（即，
Objective To evaluate a vasculitis centre based management strategy for eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA).Methods A retrospective cohort study at a vasculitis referral centre was performed. All EGPA patients admitted from 1990 to 2009 were included. A structured interdisciplinary work-up for proof of diagnosis, Disease Extent Index and Birmingham Vasculitis Activity Score was performed. Immunosuppressive therapy was initiated and regularly adapted. Treatment targets were induction and maintenance of remission according to definitions given by the European League Against Rheumatism and the European Vasculitis Study Group. Outcomes were mortality, rate of remission, relapses, adverse events and prednisolone-dose.Results Out of 269 patients with suspected EGPA 150 fulfilled the inclusion criteria. Of those, 104 had more than one follow-up visit resulting in a mean follow up of 53 +/- 4.9 months. By using additional data sources the follow-up concerning survival was extended to 92 +/- 5 month. Severe organ manifestations occurred at heart (46%), kidney (18%) and lungs (10%). Cyclophosphamide was used in 107 patients (71%). The prednisolone-doses of all patients were within the targeted range (i.e.