原发性小血管炎临床特征及其误诊原因分析

Clinical Characteristics and Causes of Misdiagnosis of Primary Vasculitis

目的分析原发性小血管炎的临床特征及其常见的误诊原因。方法对25例确诊为原发性小血管炎患者的临床资料进行回顾性分析。结果 25例原发性小血管炎患者入院时误诊、漏诊22例,首诊误诊率高达88.0%。原发性小血管炎临床表现多样,缺乏特异性,以多系统损害为主要特点,肾脏和肺脏受累最常见,其中2个脏器同时受累者19例,占76.0%。抗中性粒细胞胞浆抗体(ANCA)阳性者24例,阳性率为96.0%。25例患者均采用糖皮质激素或糖皮质激素联合环磷酰胺治疗,随访1年,21例患者(85.0%)治疗后血尿、蛋白尿完全消失;2例患者症状好转;死亡2例,病死率为8.0%。结论原发性小血管炎常出现多个系统损害,早期实验室重点筛查ANCA均能较早诊断,从而减少误诊、漏诊。

Objective To analyze the clinical characteristics of patients with primary vasculitis and the common reasons for misdiagnosis. Methods Clinical data of 25 patients diagnosed with primary vasculitis were retrospectively analyzed to summarize the characteristics and causes of misdiagnosis. Results Twentytwo of the 25 patients （88.0%） were misdiagnosed.The clinical manifestations of primary vascuhtis were vari- ous and non-specific.The main features of primary vasculitis were multi-system damage,especially in the kidney and lung （19 patient（76.0%） with both organs involved）.Twenty-four of the 25 patients （96.0%） were positive for serum anti-neutrophil cytoplasm antibodies （ANCA）.AU patients were treated with glucocorticoid or in combination with cyclophosphamide.After 1 year of follow-up,the hematuria and proteinuria disappeared in 21 patients （85.0%）,symptoms improved in 2 （8.0%） and death occurred in 2 （8.0%）. Conclusion Multi- system damage was often involved in primary vasculsitis.Early laboratory screening,especially ANCA screen- ing,can be used in early diagnosis of primary vasculitis and thus reduce the risk of misdiagnosis or missed di- agnosis.