韦格纳肉芽肿病23例临床分析

The clinical analysis of 23 patients with Wegener granulomatosis

目的分析韦格纳肉芽肿病(Wegener granulomatosis,WG)的临床和病理特点,提高对本病的认识.方法对23例确诊的WG病人的临床表现、辅助检查及病理特点进行回顾性分析.结果发病年龄20～57岁,平均37.2岁,平均病程5.8个月.首发症状以上呼吸道为主(48%),可累及多个系统或器官,肺脏受累87%,肾脏受累78%.胞质型抗中性粒细胞胞质抗体(cANCA)阳性率100%,病理表现为坏死性肉芽肿性炎症、炎细胞浸润的血管炎.结论WG的临床表现复杂多样,有多系统或器官病变的症候群,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.

Objective To investigate the clinical and pathological features of Wegener granulomatosis to improve the management of it. Methods Twenty-three patients with Wegener granulomatosis were analyzed retrospectively. Results The average age at onset was 37.2 years (range from 20~57 years). The average interval from clinical symptoms presentation to diagnosis was 5.8 months. The initial symptom of 23 cases was the upper respiratory tract involvement (48%). The patients had multiple system and organ involvement. 87% patients with WG had lung involvement and 78% patients had glucocorticoids. Seventeen out of 23 were cANCA (74%) positive. The pathological manifestations included necrotizing granulomatosis and vasculitis. Conclusion Clinical manifestation of WG is complicate, including multiple system and organ involvement. The upper and lower respiratory tract and kidney are the most commonly involved organs. Necrotizing granulomatosis and vasculitis are the pathological features of WG.