Clinical features and prognostic factors of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a literature review of 105 cases from 1999 to 2011.
Clinical features and prognostic factors of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a literature review of 105 cases from 1999 to 2011.
复制标题
系统性红斑狼疮合并血栓性血小板减少性紫癜1999-2011年105例临床特征及预后因素文献复习
DOI：
10.1007/s10067-013-2312-5
复制
发表时间：
2014-03
影响因子：
3.4
通讯作者：
Tao, Juan
中科院分区：
文献类型：
--
作者：
Jiang, Honghao;An, Xiangjie;Li, Ya;Sun, Yi;Shen, Guanxin;Tu, Yating;Tao, Juan
关键词：
本研究旨在回顾与系统性红斑狼疮患者（sTTP）相关的血栓性血小板减少性紫癜（TTP）的临床特征、治疗和预后因素。收集1999 ~ 2011年世界文献中发表的sTTP病例报告，将105例病例分为死亡组和生存组。分析流行病学特点、临床表现、实验室检查、治疗及预后因素。我们发现，死亡组（100%）与生存组（56.5%）相比，肾脏和神经损伤共存的频率明显更高（P = 0.002）。肾病理损害以IV型为主，占57.7%，其次是V型（11.5%）、II型（5.8%）和血栓性微血管病变（TMA）（5.8%）。死亡组TMA发生率（50%）高于生存组（6.25%）（P = 0.042）。终末期肾病9例，其中5例为IV型（55.6%），1例为TMA型（11.1%），3例（33.3%）未明确。在32例患者中，40.6%表现出严重的ADAMTS13缺乏，缓解后恢复正常或轻度缺乏。sTTP总死亡率为12.4%，感染组死亡率（27.3%）显著高于未感染组（8.4%）（P = 0.028）。80%以上的病例给予血浆置换和糖皮质激素治疗，缓解率为65.7%，而对难治性sTTP多采用细胞毒或利妥昔单抗治疗，缓解率达90%以上。总之，肾和神经损伤、感染和肾损害与IV型或TMA共存可能表明sTTP预后不良。
This study aims to review clinical features, treatments, and prognostic factors of thrombotic thrombocytopenic purpura (TTP) associated with systemic lupus erythematosus patients (sTTP). The case reports of sTTP published in world literature from 1999 to 2011 were collected, and 105 cases were divided into death group and survival group. The epidemiologic characteristics, clinical manifestations, laboratory examinations, treatments, and prognostic factors were analyzed. We found that coexistence of renal and neurological impairments were significantly frequent in the death group (100 %) than in the survival group (56.5 %) (P = 0.002). Type IV was predominant in 57.7 % of renal pathological damage, followed by type V (11.5 %), type II (5.8 %), and thrombotic microangiopathy (TMA) (5.8 %). TMA appeared more frequently (50 %) in the death group than in the survival group (6.25 %) (P = 0.042). End-stage renal disease occurred in nine cases with type IV in five (55.6 %), type TMA in one (11.1 %), and unspecified in three cases (33.3 %). Of 32 cases, 40.6 % showed severe ADAMTS13 deficiency and returned to normal or mildly deficient after remission. The total mortality rate of sTTP was 12.4 % and the mortality rate of patients with infection (27.3 %) was significantly higher than those without infection (8.4 %) (P = 0.028). Plasma exchange and glucocorticoids were administrated in over 80 % of cases with 65.7 % remission rate, while additional cytotoxics or rituximab was mostly used in refractory sTTP and achieved over 90 % of remission rate. Above all, coexistence of renal and neurological impairments, infection, and renal damage with type IV or TMA might denote a poor prognosis of sTTP.
登录
查看更多内容
影响因子：
4.9
作者：
Lateef A;Petri M
通讯作者：
Petri M
影响因子：
2.4
作者：
Aleem, A;Al-Sugair, S
通讯作者：
Al-Sugair, S
影响因子：
3.1
作者：
Tang Zheng;Luo Chunlei;Li Leishi
通讯作者：
Li Leishi
影响因子：
20.3
作者：
Vesely, SK;George, JN;Raskob, GE
通讯作者：
Raskob, GE
影响因子：
3.1
作者：
Starck, M;Abedinpour, F;Nerl, C
通讯作者：
Nerl, C