Della Rossa A, Baldini C, Tavoni A, Tognetti A, Neglia D, Sambuceti G, Puccini R, Colangelo C, Bombardieri S
Department of Internal Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy.
Rheumatology (Oxford). 2002 Nov;41(11):1286-94. doi: 10.1093/rheumatology/41.11.1286.
Churg-Strauss syndrome is a rare multisystem vasculitis of unknown aetiology. Due to the rarity of the disease, few single-centre case series have been described. The aim of this study was to evaluate a small series from a single Italian centre in order to describe the clinical features of the disease, the treatment and long-term follow-up.
Nineteen Churg-Strauss syndrome patients were selected from the medical records of all vasculitis patients attending the Immunology Unit at the Department of Internal Medicine of the University of Pisa in the decade between 1989 and 2000. Data were obtained retrospectively.
All the patients had asthma and hypereosinophilia. As in other case series, the lungs, skin and peripheral nervous system were the most commonly involved organs. The majority of our patient received i.v. pulses of methylprednisolone followed by i.v. pulses of cyclophosphamide. The outcome and long-term follow-up were good. There were no fatalities observed in this series during the follow-up period.
Churg-Strauss syndrome is a systemic vasculitis occurring in patients with a history of asthma and allergic rhinitis. The positive results of the treatment protocol used in this preliminary study deserve to be tested in controlled multicentre studies.
变应性肉芽肿性血管炎是一种病因不明的罕见多系统血管炎。由于该疾病罕见，很少有单中心病例系列报道。本研究旨在评估来自意大利单个中心的一小系列病例，以描述该疾病的临床特征、治疗及长期随访情况。
从1989年至2000年这十年间在比萨大学内科免疫科就诊的所有血管炎患者的病历中选取19例变应性肉芽肿性血管炎患者。数据为回顾性获取。
所有患者均有哮喘和嗜酸性粒细胞增多。与其他病例系列一样，肺、皮肤和周围神经系统是最常受累的器官。我们的大多数患者接受了静脉注射甲泼尼龙冲击治疗，随后接受静脉注射环磷酰胺冲击治疗。结局和长期随访情况良好。在该系列随访期间未观察到死亡病例。
变应性肉芽肿性血管炎是一种发生于有哮喘和变应性鼻炎病史患者的系统性血管炎。本初步研究中使用的治疗方案的阳性结果值得在对照多中心研究中进行验证。
