韦格纳肉芽肿临床特征与预后相关因素的初步分析

Initial Study of Clinical Features and Outcome of Patients in Wegener's Granulomatosis Patients

【目的】探讨韦格纳肉芽肿的临床特征和影响预后的因素。【方法】回顾性分析1999-2009年间在我科住院确诊为韦格纳肉芽肿的28例患者的临床特征和影响预后的因素,其中死亡4例。【结果】本研究随访的中位时间为31.5月(3～99月)。发生率较高的临床表现是非特异性症状(22/28,78.6%)、耳鼻喉表现(19/28,67.9%)、肺部表现(12/28,42.9%),肾小球肾炎(13/28,46.4%)。与生存组相比,死亡组诊断时急性肾损伤(P=0.005)、中枢神经系统受累(P=0.045)、血管炎器官损伤指数>4(P=0.011)的发生率高,可能提示预后不良。【结论】本研究显示韦格纳肉芽肿是一种多系统受累的疾病。诊断时伴有急性肾损伤、中枢神经系统受累、血管炎器官损伤指数>4可能提示预后不良,对这部分患者应使用更积极的免疫抑制治疗措施。

【Objective】To investigate the clinical features and outcome of Wegener＇s granulomatosis patients.【Methods】 Twenty-eight inpatients with Wegener＇s granulomatosis,diagnosed between 1999 and 2009 in Department of Rheumatology of The First Affiliated Hospital of Sun Yat-sen University,were retrospectively studied and their clinical features and outcomes were analyzed.Among them,4 cases had died.【Results】The median follow-up time was 31.5 months（3-99 months）.The most frequent clinical manifestation of this group was non-specific symptoms（22/28,78.6%）,followed by ENT involvement（19/28,67.9%）,lung involvement（12/28,42.9%）,glomerulonephritis（13/28,46.4%）.Compared with the survival group,the incidence of acute kidney injury（P=0.005）,central nervous system involvement（P=0.045）,vasculitis damage index4（P= 0.011）was higher than those in the group of death.Those features were possible predictors of poor prognosis.【Conclusion】This study showed that Wegener＇s granulomatosis was a multi-system involvement disease.The incidence of acute kidney injury,central nervous system involvement,vasculitis damage index4 might be possible predictors of poor prognosis,more aggressive immunosupressant needed to be employed in this kind of patients.