显微镜下多血管炎临床特点及其死亡危险因素分析

The clinical features of patients with microscopic polyangiitis and analysis of the risk factors of death

目的观察显微镜下多血管炎（microscopic polyangiitis,MPA）的临床特点,分析其预后危险因素。方法回顾性分析2012年9月至2016年4月复旦大学附属华东医院免疫风湿科住院治疗以及门诊随访的MPA患者。评价其临床特征,包括抗中性粒细胞胞浆抗体（anti-neutrophil cytoplasmic antibody,ANCA）、累及器官、治疗及生存等。Kaplan-Meier法分析计算累积生存率。结果纳入20例患者,男性9例,女性11例,中位发病年龄71岁,四分位范围（65~78岁）,病程2个月（1~6个月）。随访时间9个月（5.5~22.8个月）。以发热为首发表现13例,其中高热8例（体温≥39.1℃）;胸部CT提示肺部病灶12例,其中肺间质病变11例;髓过氧化物酶抗中性粒细胞胞浆抗体（myeloperoxidase anti-neutrophil cytoplasmic antibody,MPO-ANCA）阳性17例,蛋白酶-3抗中性粒细胞胞浆抗体（peroxidase-3 anti-neutrophil cytoplasmic antibody,PR3-ANCA）阳性4例;糖皮质激素初始平均剂量为61 mg（泼尼松或相当于泼尼松）;2例血液透析;免疫抑制剂治疗18例。随访发现死亡患者4例。发病时肌酐水平和PR3-ANCA阳性增加死亡风险。结论 MPA患者发病年龄较西方研究报道高,常以发热为首发症状,肺部累及多见,以间质性炎症为主。发病时肌酐水平高和PR3-ANCA阳性提示预后不佳。

Objective To observe the clinical characteristics and risk factors of patients with microscopic polyangiitis（MPA）in a Chinese cohort. Methods The medical records were reviewed of all hospitalized patients and outpatients with MPA in Division of Rheumatology,Huadong hospital,Fudan University from Sep.,2012 to Apr.,2016.We evaluated their clinical features including antineutrophil cytoplasmic antibody（ANCA）status and organ involvement,treatment and survival.Cumulative patient survival was calculated using the Kaplan-Meier method. Results A total of 20 patients with MPA included,male 9 and female 11.The median age at diagnosis was 71 years（interquartile range 65-78 years）,after a median follow-up of 9 months（interquartile range 5.5-22.8 months）.Thirteen patients presented with fever as the initial symptom,8 patients with high fever above 39.1 ℃.Chest computed tomography revealed 12 patients with lung involvement,and majority（11 cases）with interstitial inflammation changes.Seventeen patients were myeloperoxidase-ANCA（MPO-ANCA）positive,and 4 patients were peroxidase-3-ANCA（PR3-ANCA）positive.The mean initial daily glucocorticoid dose was 61 mg initially（prednisone or equivalent）.Hemodialysis was implemented in 2 patients.Eighteen cases received concomitant immunosupressants.Four deaths were reported during the observation period.Kaplan-Meier identified a higher serum creatinine at onset and positive PR3-ANCA as factors with higher risk of death. ConclusionsMPA mainly affect elderly people,age at onset was elder than reported in western country.Fever is one of common manifestations initially.Lung involvement is frequent,mainly as interstitial pneumonitis.High levels of serum creatinine and PR3-ANCA positivity are associated with risk of death.