关键词: 系统性红斑狼疮, 血栓性微血管病

Abstract:

Ｏｂｊｅｃｔｉｖｅ：To retrospectively analyze the clinical,histological features and prognosis of renal injury characterized by renal thrombotic microangiopathy in systemic lupus erythematosus (SLE) (SLErenal TMA).
Ｍｅｔｈｏｄｏｌｏｇｙ：Patients with  glomerular TMA and/or interstitial vascular TMA in the absence of subendothelial or epithelial immune complex (IC) deposition were screened out from 2 332 SLE patients who underwent first renal biopsy from January 2005 to December 2016. Their clinical,histological features and outcomes were retrospectively analyzed.
Ｒｅｓｕｌｔｓ：Twenty patients (09% of SLE renal biopsy) had SLErenal TMA.All of them had acute kidney injury (AKI) and 11 cases needed renal replacement treatment (RRT).Twelve cases presented as nephrotic syndrome.Thirteen (65%) cases were in accord with  TMA triad.Pathologically,17 cases (850%) had both glomerular and interstitial vascular TMA.Eight patients had no IC deposit,twelve patients had IC deposites only in mesangium.Range of foot process fusion  is positively correlated with urinary protein quantification.All 11 patients requiring RRT recovered,15 (75%) achieved complete remission and 4 (20%) achieved partial remission.During a median followup of 580 (360,923) months,5 cases relapsed,no endstage renal disease or death occurred.
Ｃｏｎｃｌｕｓｉｏｎ：Renal injury characterized by TMA is not uncommon in SLE  cases.The clinical manifestation is special and the renal injury is serious.The renal outcome is good by intensive immunosuppressive therapy It should be considered as a unique type of renal injury in SLE.