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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 analyze the clinical and pathological features of Wegener granulomatosis (WG) and to improve the understanding of this disease. Methods The clinical manifestations, laboratory examinations and pathological features of 23 patients with confirmed WG were retrospectively analyzed. Results The age of onset was 20 to 57 years old, with an average of 37.2 years and a mean duration of 5.8 months. The first symptom of more than the upper respiratory tract (48%), can affect multiple systems or organs, 87% of lung involvement, kidney involvement 78%. Cytoplasmic anti-neutrophil cytoplasmic antibody (cANCA) positive rate of 100%, the pathological manifestations of necrotizing granulomatous inflammation, inflammatory cell infiltration of vasculitis. Conclusion The clinical manifestations of WG are complex and diverse. There are multiple systemic or organ lesion syndromes, with the most common upper and lower respiratory tract and kidney involvement. The pathological features are necrotizing granuloma and vasculitis.