Zheng Tang, Chunlei Luo, Zhen Wang, Ping Liu, Haitao Zhang, Weixin Hu, Caihong Zeng, Huiping Chen, Zhihong Liu, Leishi Li
Research Institute of Nephrology, Jinling Hospital, Nanjing, People's Republic of China.
Am J Med Sci. 2009 Nov;338(5):343-7. doi: 10.1097/MAJ.0b013e3181b0c872.
We investigated the clinical-pathological features and the prognosis of thrombotic thrombocytopenic purpura (TTP) in patients with lupus nephritis (LN).
A retrospective analysis was performed on the clinical-pathological data and prognosis in 8 patients with LN complicating with TTP.
Thrombocytopenia and hemolytic anemia, neurologic symptoms, and renal dysfunction were the clinical manifestations in 8 patients. Six patients had fever. Eight patients presented with rapid progressive glomerulonephritis, and 1 patient with continuous gross hematuria. The histologic features of the 8 patients were thrombotic microangiopathy lesions. Immune-suppressive therapies were administrated in all patients, and blood purification therapy was applied in 7 patients. Three cases involved plasma exchange and/or immunoabsorption. Seven patients received a median follow-up of 12 months. One patient died, 3 cases received peritoneal dialysis, and 1 case failed to follow-up. During follow-up, 1 case was able to stop peritoneal dialysis, and 1 case changed to hemodialysis. The other 3 patients continued with stable renal function.
The patients with LN with TTP have severe clinical-pathological changes. Active treatment including renal replacement therapy, plasma exchange, and immunoabsorption are promising.
我们研究了狼疮性肾炎（LN）患者血栓性血小板减少性紫癜（TTP）的临床病理特征及预后。
对8例LN合并TTP患者的临床病理资料及预后进行回顾性分析。
8例患者的临床表现为血小板减少、溶血性贫血、神经系统症状及肾功能不全。6例患者有发热。8例患者表现为快速进展性肾小球肾炎，1例患者有持续性肉眼血尿。8例患者的组织学特征为血栓性微血管病病变。所有患者均接受免疫抑制治疗，7例患者接受血液净化治疗。3例患者接受了血浆置换和/或免疫吸附治疗。7例患者接受了中位12个月的随访。1例患者死亡，3例患者接受腹膜透析，1例患者失访。随访期间，1例患者能够停止腹膜透析，1例患者改为血液透析。其他3例患者肾功能持续稳定。
LN合并TTP的患者有严重的临床病理改变。包括肾脏替代治疗、血浆置换和免疫吸附在内的积极治疗前景良好。
