Ding J X, Wang M
Renal Division, Blood Dialysis Center, Peking University International Hospital, Beijing 102206, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2017 Oct 18;49(5):915-918.
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 mL 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.
抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)通常是一种多系统疾病,肺肾综合征是其常见表现。AAV患者进展至终末期肾病(ESRD)时复发的可能性较小。我们报告一例罕见的病例,一名患者在血液透析8年后复发性AAV出现弥漫性肺泡出血(DAH)。一名58岁女性因呼吸困难、咯血伴贫血、发热、乏力和体重减轻为主诉入院。她的抗髓过氧化物酶(MPO)滴度升高。计算机断层扫描显示弥漫性肺泡出血。在确诊AAV复发后,她接受了以下治疗:入院后24小时内开始血浆置换,每次置换3000毫升血浆,血浆置换期间应用枸橼酸盐抗凝。进行了5次血浆置换治疗,3次置换后肺出血停止。其他治疗包括甲泼尼龙冲击治疗,逐渐减量至口服泼尼松和环磷酰胺。安排了规律的血液透析。这些治疗使炎症症状消退,DAH改善。她的抗MPO水平下降。患者康复出院。伴有DAH的AAV通常起病急,一般发病率高、死亡率高。因此,需要及时诊断和积极治疗以提高生存率。