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目的韦格纳肉芽肿病(WG)是一病因不清的少见的系统性血管炎。通过分析病例系列以描述疾病的临床特征,治疗和病情随访。方法回顾性地收集11例来自1999—2005年我院风湿科住院WG患者,分析临床及实验室特征。结果所有WG患者都有上下呼吸道累及。肺、上呼吸道和肾是最常累及的器官。多数患者接受了静脉注射甲泼尼龙(MP)0．5g/d×3d,随后静脉注射环磷酰胺(CTX)750 mg/m~2,每月用1 g,共治疗6～8次,疗效好,长期随访病情稳定。结论WG是系统性血管炎,以上、下呼吸道及肾脏受累最多见；病理特点为坏死性肉芽肿和血管炎,联合免疫抑制治疗可取得较好的疗效。
Purpose Wegener’s granulomatosis (WG) is a rare systemic vasculitis of unknown etiology. By analyzing the case series to describe the clinical features of the disease, treatment and disease follow-up. Methods Retrospectively collected 11 cases of WG patients hospitalized in rheumatology department of our hospital from 1999 to 2005, and analyzed the clinical and laboratory characteristics. Results All WG patients had upper and lower respiratory involvement. The lungs, the upper respiratory tract and the kidneys are the most commonly involved organs. Most patients received intravenous injection of methylprednisolone (MP) 0.5g / d x 3d, followed by intravenous injection of cyclophosphamide (CTX) 750mg / m ~ 2, 1g per month, a total of 6 to 8 times the treatment effect Well, long-term follow-up was stable. Conclusion WG is a systemic vasculitis with the most common upper and lower respiratory tract and renal involvement. The pathological features are necrotizing granulomatosis and vasculitis, and combined immunosuppressive therapy can achieve better results.