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目的:探讨韦格纳肉芽肿的临床特点和诊治经验,提高对该病的诊治水平。方法:收集并分析20例韦格纳肉芽肿患者的临床资料。结果:发病年龄为8个月～79岁,中位年龄46岁,平均病程1.8年。临床表现:发热占95%,上呼吸道受累90%,肺部受累75%,肾脏受累70%,部分患者有关节、皮肤、眼、神经系统、心脏、消化系统受累。经肾上腺皮质激素及免疫抑制药治疗后19例疗效良好,1例表现为急进性肾炎综合征的患者死亡。结论:韦格纳肉芽肿临床表现多种多样,大部分有上呼吸道、肺、肾受累,首发症状多样化,常导致误诊、误治;早期诊治是改善预后的关键。
Objective: To investigate the clinical features, diagnosis and treatment of Wegener’s granulomatosis and to improve the diagnosis and treatment of the disease. Methods: The clinical data of 20 Wegener’s granulomatous patients were collected and analyzed. Results: The age of onset was 8 months to 79 years old, with a median age of 46 years and an average duration of 1.8 years. Clinical manifestations: fever accounted for 95%, upper respiratory tract involvement 90%, lung involvement 75%, kidney involvement 70%, some patients with joints, skin, eyes, nervous system, heart, digestive system involvement. Nineteen patients were treated well with corticosteroids and immunosuppressive drugs, and one patient with acute nephritic syndrome died. Conclusion: The clinical manifestations of Wegener’s granulomatosis are varied. Most of them have upper respiratory tract, lung and kidney involvement. The first symptom is diversified, which often leads to misdiagnosis and mistreatment. Early diagnosis and treatment are the key to improve prognosis.