韦格纳肉芽肿病的临床和病理分析

Clinical and pathological analysis of Wegener granulomatosis patients

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 granulomatosis (WG),and its response to therapy so as to improve management of this disorder.Methods Clinical data of 25 WG patients together with 20 biopsy specimens were reviewed.Nine patients were followed up.Anti neutrophil cytoplasmic antibodies (ANCA) were detected by indirect immunofluorescent method.Results The patients′ages at the beginning of the disease were from 13 to 72 years.The average was (38±16) years.The average course from the initiation of clinical symptoms to diagnosis was (8±9) months.Patients had multiple systems and organs involvements.Ear nose and throat involvements were the most common symptoms (96%).Seven out of 12 were positive in ANCA (58%).Pathological manifestations involvded parenchymal necrosis,granulomas,multinucleated giant cells,microabscesses,vasculitis and so forth.Most patients improved significantly after being treated with glucocorticoids and cyclophosphamide.Conclusion WG is a systemic necrotizing vasculitis different from others in its predilection to affect the upper and lower respiratory tracts and kidneys.Most patients had good response to glucocorticoids and cyclophosphamide and their prognosises were good.