Department of Medical Education, Scripps Mercy Hospital, San Diego, CA, USA.
J Clin Rheumatol. 2012 Jun;18(4):185-8. doi: 10.1097/RHU.0b013e31825826ae.
We report a patient with a diagnosis of systemic lupus erythematosus who concurrently developed a syndrome of thrombotic microangiopathy that resembled thrombotic thrombocytopenic purpura. The patient underwent plasma exchange and immunosuppressive therapy for months before clinical improvement was finally achieved through bilateral nephrectomy. Ultimately, our patient died of disseminated aspergillosis from prolonged immunosuppression. We believe that recognition of bilateral nephrectomy as a potential treatment earlier in her course would have spared her this unfortunate demise. We hope that this review of current literature will help the reader to consider bilateral nephrectomy in patients with refractory systemic lupus erythematosus with clinical overlap of thrombotic microangiopathy resembling thrombotic thrombocytopenic purpura.
我们报告了一例系统性红斑狼疮诊断患者,其并发类似于血栓性血小板减少性紫癜的血栓性微血管病综合征。该患者接受了数月的血浆置换和免疫抑制治疗,最终通过双侧肾切除术才最终实现临床改善。最终,由于长期免疫抑制导致播散性曲霉菌病,我们的患者死亡。我们认为,如果在她病程的早期认识到双侧肾切除术是一种潜在的治疗方法,就可以避免她的不幸死亡。我们希望对当前文献的回顾将帮助读者在难治性系统性红斑狼疮患者中考虑双侧肾切除术,这些患者的临床表现重叠类似于血栓性血小板减少性紫癜的血栓性微血管病。