10例显微镜下多血管炎的临床分析  

Clinical characteristics in ten microscopic polyangiitis patients

作　　者：戈霞晖[1] 韩锋锋[1] 李惠民[2] 管雯斌[3] 郭雪君[1] 

机构地区：[1]上海交通大学医学院附属新华医院呼吸科,200092 [2]上海交通大学医学院附属新华医院放射科,200092 [3]上海交通大学医学院附属新华医院病理科,200092

出　　处：《国际呼吸杂志》2012年第10期728-731,F0003,共5页International Journal of Respiration

摘　　要：目的对10例显微镜下多血管炎（microscopicpolyangiitis，MPA）患者各项临床资料进行分析，以便提高对该病的认识。方法回顾性分析了10例MPA患者的临床表现、实验室检查、影像学资料及病理特点，同时对每例患者进行随访。结果所有患者均有呼吸系统、肾脏及全身症状，3例有中枢神经系统累及，4例有消化系统症状，2例则伴有全身皮疹。胸部CT主要以肺间质纤维化及胸膜增厚为主，伴有斑片状实变影及磨玻璃影。肾脏及皮肤活组织检查病理的共同特点主要是小血管纤维素样坏死。对所有患者进行随访，4例死亡患者中2例分别死于蛛网膜下腔出血及脑出血。结论本研究MPA患者胸部CT主要表现为肺间质纤维化及胸膜增厚，肺及肾脏累及提示预后不佳，而中枢神经系统累及常预示预后凶险。Objective To improve our recognition of microscopic polyangiitis （MPA） by analyzing clinical data of ten MPA patients. Methods The clinical manifestations, pulmonary computerized tomography, laboratory test results, pathology and follow-up material were retrospectively analyzed in ten MPA patients. Results Respiratory, renal and constitutional symptom were presented in all patients. Moreover, three patients （30 % ） were involved central nervous system, four patients （40%） had digestive system symptom, and two patients （20%） were found severe rash. In pulmonary computerized tomography, fibrosis and pleural thickening were main kinds of patterns in MPA, companion with patchy consolidation and ground glass opacities. Fibrinoid necrosis in pathology was common characteristic in both renal and skin biopsy. All patients were followed up, and four patients died, of whom two patients died from subarachnoid hemorrhage and cerebral hemorrhage. Conclusions Fibrosis and pleural thickening in pulmonary computerized tomography are main kinds of patterns in MPA. However, respiratory and renal as well as central neurological involvement indicates poor prognosis.

关 键 词：显微镜下多血管炎 肺部表现 抗中性粒细胞胞浆抗体 中枢神经系统损害 

分 类 号：R593.2[医药卫生—内科学]