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目的 研究韦格纳肉芽肿病 (Wegenergranulomatosis ,WG)的临床、病理特点以及对治疗的反应 ,提高对本病的认识。方法 分析 2 5例WG患者的临床资料和其中 2 0例的活检病理资料 ,对 9例病人进行随访。结果 发病平均年龄 (38± 16 )岁。从发病到确诊的平均时间为 (8± 9)个月。有多系统、器官受累 ,以耳、鼻和喉部受累最为常见 (96 % )。抗中性粒细胞胞质抗体阳性率为5 8%。病理表现有实质组织坏死、肉芽肿、多核巨细胞、微脓肿和血管炎等。多数患者接受糖皮质激素和环磷酰胺治疗后病情明显改善。结论 WG的基本病理改变为坏死性肉芽肿和小血管炎 ,有多系统、器官受累 ,而以上、下呼吸道及肾脏受累为常见 ,对糖皮质激素和环磷酰胺治疗反应较好。
Objective To study the clinical and pathological features of Wegener’s granulomatosis (WG) and its response to treatment to improve the understanding of this disease. Methods Clinical data of 25 patients with WG and 20 biopsy pathological data were analyzed. Nine patients were followed up. Results The average age of onset (38 ± 16) years. The average time from onset to diagnosis was (8 ± 9) months. There are multiple systems, organs involved, with ear, nose and throat involvement the most common (96%). The positive rate of anti-neutrophil cytoplasmic antibody was 58%. Pathological manifestations of parenchymal necrosis, granuloma, multinucleated giant cells, micro abscesses and vasculitis. Most patients receiving glucocorticoid and cyclophosphamide significantly improved after treatment. Conclusions The basic pathological changes of WG are necrotizing granulomatous and vasculitis, involving multiple systems and organs. Above and below the lower respiratory tract and kidneys are common and response to glucocorticoid and cyclophosphamide is good.