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系统性红斑狼疮与血栓。

Systemic lupus erythematosus and thrombosis.

机构信息

Haematology and Thrombosis Unit, CMID Department, San Giovanni Bosco Hospital, Piazza Donatore di Sangue 3, 10154 Turin, Italy.

出版信息

Thromb J. 2015 Apr 23;13:16. doi: 10.1186/s12959-015-0043-3. eCollection 2015.

Abstract

Systemic Lupus Erythematosus (SLE) is an acquired, multiorgan, autoimmune disease. Clinical presentation is extremely variable and heterogeneous. It has been shown that SLE itself is an independent risk factor for developing both arterial and venous thrombotic events since SLE patients have an Odds Ratio (OR) for thrombosis that varies depending on the clinical and laboratory characteristics of each study cohort. The risk of developing a thrombotic event is higher in this setting than in the general population and may further increase when associated with other risk factors, or in the presence of inherited or acquired pro-thrombotic abnormalities, or trigger events. In particular, a striking increase in the number of thrombotic events was observed when SLE was associated with antiphospholipid antibodies (aPL). The presence of aPLs has been described in about 50% of SLE patients, while about 20% of antiphospholipid syndrome (APS) patients have SLE. While APS patients (with or without an autoimmune disease) have been widely studied in the last years, fewer studies are available for SLE patients and thrombosis in the absence of APS. Although the available literature undoubtedly shows that SLE patients have a greater prevalence of thrombotic events as compared to healthy subjects, it is difficult to obtain a definite result from these studies because in some cases the study cohort was too small, in others it is due to the varied characteristics of the study population, or because of the different (and very copious) laboratory assays and methods that were used. When an SLE patient develops a thrombotic event, it is of great clinical relevance since it is potentially life-threatening. Moreover, it worsens the quality of life and is a clinical challenge for the clinician.

摘要

系统性红斑狼疮(SLE)是一种获得性、多器官、自身免疫性疾病。临床表现极其多样且异质。已经表明,SLE 本身是发生动脉和静脉血栓事件的独立危险因素,因为 SLE 患者发生血栓的优势比(OR)因每个研究队列的临床和实验室特征而异。在这种情况下,发生血栓事件的风险高于普通人群,当与其他危险因素相关时,或存在遗传性或获得性促血栓异常或触发事件时,风险可能进一步增加。特别是当 SLE 与抗磷脂抗体(aPL)相关时,观察到血栓事件的数量显著增加。大约 50%的 SLE 患者存在 aPLs,而大约 20%的抗磷脂综合征(APS)患者患有 SLE。虽然近年来广泛研究了 APS 患者(无论是否患有自身免疫性疾病),但 SLE 患者和无 APS 的血栓形成的研究较少。尽管现有文献无疑表明 SLE 患者发生血栓事件的患病率高于健康受试者,但从这些研究中很难得出明确的结论,因为在某些情况下研究队列太小,在其他情况下则归因于研究人群的不同特征,或由于使用了不同(且非常丰富)的实验室检测和方法。当 SLE 患者发生血栓事件时,具有重要的临床意义,因为它可能危及生命。此外,它会降低生活质量,对临床医生来说是一个临床挑战。

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