抗中性粒细胞胞浆抗体相关性小血管炎维持性透析患者并发肺出血1例  

Anti-neutrophil cytoplasmic antibodies-associated vasculitis with lung hemorrhage in the patient on maintenance haemodialysis:a case report

作　　者：丁嘉祥[1] 王梅 

机构地区：[1]北京大学国际医院肾内科血液净化中心,北京102206

出　　处：《北京大学学报（医学版）》2017年第5期915-918,共4页Journal of Peking University:Health Sciences

摘　　要：抗中性粒细胞胞浆抗体（anti-neutrophilcytoplasmicantibodies，ANCA)相关性小血管炎(associatedvasculitis,AAV)是一个可累及全身多个脏器的系统性疾病，最常见肾脏和肺脏受累[1]，临床上常表现为急进性肾小球肾炎和肺出血。严重的弥漫性肺出血是AAV早期死亡的最常见原因[2]，发生率为10%~45%，其中5%的患者需要呼吸机辅助治疗，AAV患者出现肺出血提示患者的预后不良[3]。长期维持性血液透析的AAV患者合并肺出血的报道较少，本文报告1例既往无肺出血的AAV患者在维持性血液透析8年后出现肺出血的诊断治疗经过，并进行文献复习，以期提高对该病的诊断和治疗水平。Anti-neutrophil cytoplasmic antibodies( ANCA)-associated vasculitis( AAV) is usually a multisystem disorder,and pulmonary renal syndrome is a common presentation. Patients with AAV are less likely to experience relapse when they progress to end-stage renal disease( ESRD). We report a rare case of diffuse alveolar hemorrhage( DAH) in relapsing AAV after eight years of haemodialysis. A 58-year-old woman was admitted to our hospital with the chief complaints of dyspnea and hemoptysis accompanied by anemia,fever,fatigue,and weight loss. She had elevated anti-myeloperoxidase( MPO) titer.The computer tomoghraphy showed diffuse alveolar hemorrhage. After the recurrent episode of AAV was diagnosed,she underwent the following therapy: Plasmapheresis was initiated within 24 h after admission,3 000 m L of plasma was removed per session,and the anticoagulation of citrate was applied during plasmapheresis. Five plasmapheresis treatments were performed,and after three apheresis sessions,the pulmonary hemorrhage ceased. Other treatments included a methylprednisolone bolus,tapered to oral prednisone and cyclophosphamide. Regular hemodialysis was scheduled. These treatments resulted in resolution of the inflammatory symptoms,DAH improved. Her anti-MPO level decreased. The patient was discharged in good condition. AAV with DAH is usually acute at the onset and is generally a condition with high morbidity and substantial mortality. Therefore,prompt diagnosis and aggressive treatment are needed to improve survival.

关 键 词：抗体 抗中性粒细胞胞浆 血管炎 血液透析 出血 肺疾病 

分 类 号：R692[医药卫生—泌尿科学]