显微镜下多血管炎的临床与病理特征及预后分析

Analysis of clinical,pathological features and prognosis of microscopic polyangiitis

目的分析显微镜下多血管炎(MPA)的临床特征,观察不同病理类型的临床转归。方法回顾性分析上海交通大学医学院附属新华医院2010年1月至2019年12月61例MPA患者的临床资料。根据年龄将患者分为≥60岁组(46例)和<60岁组(15例),根据初发时血肌酐水平分为≥500μmol/L组(18例)和<500μmol/L组(43例)。记录患者的基本资料和实验室检查结果等,采用伯明翰系统性血管炎活动评分(BVAS)评估疾病活动度。对23例病理资料完整的患者进行病理分型并随访其临床转归,以进展至终末期肾病(ESRD)或死亡为终点事件。结果≥60岁组铁蛋白明显高于<60岁组[452(289,792)μg/L比210(119,451)μg/L],差异有统计学意义(P<0.05)。肌酐≥500μmol/L组发热率、血红蛋白和血小板明显低于肌酐<500μmol/L组[3/18比48.8%(21/43)、77.5(62.8,86.0)g/L比85.0(77.0,104.0)g/L和192(147,234)×10^(9)/L比257(208,365)×10^(9)/L],胃肠道受累和BVAS明显高于肌酐<500μmol/L组[16/18比25.6%(11/43)和20.0(16.0,23.3)分比15.0(12.0,19.0)分],差异有统计学意义(P<0.05或<0.01)。Pearson相关分析结果显示,BVAS与肌酐呈正相关(r=0.42,P<0.01),与血红蛋白呈负相关(r=-0.42,P<0.01),与红细胞沉降率和血小板无相关性(r=0.05和0.04,P>0.05)。完成临床转归统计的23例患者中,6例肾脏病理表现为新月体型患者中5例发生终点事件,12例肾间质重度损伤患者中7例发生终点事件;Kaplan-Meier生存曲线分析结果显示,ESRD MPA患者平均生存期明显短于非ESRD MPA患者(41.2个月比63.5个月),差异有统计学意义(χ^(2)=0.48,P=0.028)。结论老年MPA患者与年轻MPA患者临床表现相近;初发时肌酐≥500μmol/L和贫血预示着血管炎活动度更高;病理表现为新月体型或重度间质损伤患者预后较差,ESRD较非ESRD患者生存率更低。

Objectives To analyze the clinical features of microscopic polyangiitis(MPA),and observe the clinical outcomes of different pathological types.Methods The clinical data of 61 patients with MPA in Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from January 2010 to December 2019 were analyzed retrospectively.According to age,the patients were divided into≥60 years old group(46 cases)and<60 years old group(15 cases).According to the initial serum creatinine,the patients were divided into≥500µmol/L group(18 cases)and<500µmol/L group(43 cases).The basic data and laboratory examination results of the patients were recorded,and the disease activity was evaluated by Birmingham systemic vasculitis activity score(BVAS).Twenty-three patients with complete pathological data were pathologically classified and followed up to assess their clinical outcomes.The progression to end-stage renal disease(ESRD)or death was defined as the endpoint.Results Ferritin in≥60 years old group was significantly higher than that in<60 years old group:452(289,792)µg/L vs.210(119,451)µg/L,and there was statistical difference(P<0.05).The fever rate,hemoglobin and platelets in creatinine≥500µmol/L group were significantly lower than those in creatinine<500µmol/L group:3/18 vs.48.8%(21/43),77.5(62.8,86.0)g/L vs.85.0(77.0,104.0)g/L and 192(147,234)×10^(9)/L vs.257(208,365)×10^(9)/L,the gastrointestinal involvement and BVAS in creatinine≥500µmol/L group were significantly higher than those in creatinine<500µmol/L group:16/18 vs.25.6%(11/43)and 20.0(16.0,23.3)scores vs.15.0(12.0,19.0)scores,and there were statistical differences(P<0.05 or<0.01).Pearson correlation analysis result showed that BVAS was positively correlated with creatinine(r=0.42,P<0.01),negatively correlated with hemoglobin(r=-0.42,P<0.01),but it had no correlation with erythrocyte sedimentation rate and platelets(r=0.05 and 0.04,P>0.05).Among the 23 patients with completed the clinical outcome statistics,endpoint events occurred in 5 of 6 patients with crescent renal pathology,and in 7 of 12 patients with severe renal interstitial injury.Kaplan-Meier survival curve analysis result showed that the average survival time in ESRD MPA patients was significantly shorter than that in non ESRD MPA patients(41.2 months vs.63.5 months),and there was statistical difference(χ^(2)=0.48,P=0.028).Conclusions The clinical manifestations of elderly MPA patients are similar to those of young MPA patients.Creatinine≥500µmol/L or anemia at initial onset indicate higher vasculitis activity in MPA.The prognosis of MPA patients with pathological manifestations of crescent or severe interstitial injury is poor,and the survival rate of ESRD is lower than that of non ESRD patients.