系统性红斑狼疮相关血栓性血小板减少性紫癜的临床特点

回顾性总结北京协和医院2008年10月至2013年9月5年内共12例系统性红斑狼疮（SLE）相关血栓性血小板减少性紫癜（1TrP）患者的临床资料。SLE相关TTP患者占同期住院SLE患者的0．45％（12／2693）。其中男女比例1：5，中位年龄23岁。TTP诊断时疾病活动指数（SLEDAI）评分12—30分，多数为重度活动者（10／12）。患者诊断前均有显著的血小板降低及明确肾损害。12例患者中10例出现急性肾衰竭；9例心肌受累；8例有中枢神经系统受累症状；发病时8例有4个及以上脏器损害；3例做了肾穿刺活检，Ⅳ型狼疮肾炎2例，V型狼疮肾炎1例，病理均可见肾脏血栓性微血管病表现。外周血涂片中发现破碎红细胞是明确诊断的主要依据。11例接受了大剂量激素冲击治疗，6例最终好转（6／11）。6例接受了血浆置换（40ml·kg^-1·d^-1），4例好转。未接受血浆置换的6例，3例好转。12例患者最终死亡2例，恶化放弃治疗3例，7例好转。

Clinical characteristics of systemic lupus erythematosus associated thrombotic thrombocytopenic purpura

We retrospectively reviewed the clinical records of 12 systemic lupus erythematosus （SLE） patients with thrombotic thrombocytopenic purpura （TTP） admitted into Peking Union Medical College Hospital （PUMCH） during October 2008 to September 2013. TTP occurred in 0. 45% （ 12/2 693 ） of patients with SLE. The median age （2 males, 10 females） was 23 years. Their SLEDAI scores ranged from 12 to 30. All patients had significant thrombocytopenia and renal abnormalities. Ten of them had acute renal failure. Cardiac involvement and neurological deficits were observed in 9 and 8 of them respectively. Eight of them had injuries of more than 4 organs. Renal biopsy of 3 patients revealed lupus nephritis （ type IV, n = 2 ; type V, n = 1 ） and thrombotic microangiopathy. Eleven TIP patients received steroid pulse therapy （ 1 g/d, 3 days） and six of them survived. Six patients were rescued by therapeutic plasma exchange and 4 of them recovered. Among another 6 patients not on plasma exchange therapy, only 3 recovered. Finally 2 died, 3 gave uo with exacerbated symptoms after therapy and 7 survived.