抗中性粒细胞胞浆抗体相关性肺血管炎的诊治(附19例报告)

ANCA-related vasculitis with lungs involvement (report of 19 cases)

目的探讨抗中性粒细胞胞浆抗体(antineutrophilcytoplasmicantibodies,,ANCA)相关性肺血管炎的临床表现及早期诊断要点。方法采用回顾性方法对19例ANCA相关性肺血管炎患者的临床资料进行分析。结果本组19例均有肺损伤,肾脏损害13例,肝脏损害5例,皮肤损害5例,心脏损害3例;胸部CT示11例有双肺弥漫性病变。血沉为(89.20±19.82)mm/1h,C-反应蛋白为(55.33±34.12)mg/L。ANCA及特异性靶抗原[髓过氧化物酶(MPO)及蛋白酶3(PR3)]检查阳性。应用糖皮质激素及免疫抑制剂治疗,7例肺部病变完全吸收,血尿、蛋白尿减轻6例,肾功能改善3例;其他脏器功能情况好转或趋于稳定,1例肾衰竭患者需血液透析支持治疗。结论ANCA相关性肺血管炎临床表现复杂,除肺部病变外,常累及多器官、多系统,ANCA及相应的特异性靶抗原检查有助于该病的早期诊断。早期给予糖皮质激素及免疫抑制剂干预治疗,能延长患者的生存时间,改善预后。

Objective To investigate the pulmonary clinical manifestations and the early diagnosis of ANCA- related vasculitis with lung involvement. Methods Data of 19 ANCA-related vasculitis cases with lung involvement were analyzed retrospectively. Results All 19 cases had not only lung involvement but also multi-system damages including kidney, liver, skin etc. Findings on chest X-Ray were multiple, diffuse patchy shadows in both lungs. Laboratory examination revealed elevated ESR and CRP increased, ANCA and the specific antigen （MPO, PR3） were positive. Administration of corticosteroid and immune suppressors helped the lung damaging manifestation disappear, while the function of other organs to he improved or stable. Few patients with renal failure had to receive hemedialysis. Conclusions The clinical manifestation of ANCA-related casculitis with lung involvement are complicated, multiple organs and systems damaging are often occurred besides the lung, ANCA and specific antigens tests may help the early diagnosis. Early administration of corticosteroid and immune suppressors can extend patients＇ live time and improve the prognosis.