Goodpasture综合征13例回顾分析

Clinical Analysis of 13-Case of Goodpasture Syndrome

目的:探讨Goodpasture综合征的临床特征及诊治要点。方法:对13例Goodpasture综合征患者的临床表现、实验室检查、诊断与治疗等资料进行回顾性分析。结果:13例患者中,全部患者(100%)均有咯血,12例(92.3%)有镜下血尿,大多数患者咯血症状先于肾小球肾炎症状出现。10例(83.3%)抗GBM抗体阳性。12例接受完整治疗方案的患者,有效7例,有效率58.3%。无效死亡5例,平均生存期为(91±100.1)月。结论:Goodpasture综合征是一组病变进展迅速,预后凶险的自身免疫性疾病,显效组在确诊天数上与有效组与死亡组间的差别具有统计学意义(P均<0.05),改善疾病预后的关键是早发现、早诊断、早治疗。

ABSTRACT Objective： To discuss the clinical characteristic, assistance inspection and points for the diagnosis and therapy. Methods： Retrospective analysis of the 13-case was made on the clinical manifestation of Goodpasture syndrome, laboratorial tests, diagnosis and treatment. Results： In this cohort, haemoptysis which usually happened before renal presentation occurred in all patients and microscopic hematuria occurred in 12 （92.3%） patients. Ten patients （83.3%） were anti-GBM body positive. Seven eases were effective in 12 patients who received treatments and the effective rate was 58.3%. Five patients died and the mean survival time was 91± 100.072 months. Conclusion： Goodpasture syndrome is a group of autoimmune diseases which progresses rapidly and has a bad prognosis. The obvious effect group has statistic difference in the diagnosed days with effect group and non-effect group （P both 〈0.05）. The key to improve prognosis is early discovery, early diagnosis and early treatment. Key words： Goodpasture syndrome; Anti-glomerular basement membrane antibody; Pulmonary hemorrhage; Acute glomerulonephritis