Zöller Bengt, Li Xinjun, Sundquist Jan, Sundquist Kristina
Am J Cardiovasc Dis. 2012;2(3):171-83. Epub 2012 Jul 25.
Venous thromboembolism (VTE) is major health problem and is sometimes complicated by lethal pulmonary embolism (PE). Disturbances of the coagulation and anticoagulation systems are important risk factors for VTE. Comparative studies suggest that coagulation and innate immunity have a shared evolutionary origin. It is therefore unsurprising that the immune and coagulation systems are linked, with many molecular components being important for both systems. Systemic inflammation modulates thrombotic responses by suppressing fibrinolysis, upregulating procoagulant, and downregulating anticoagulants, and autoimmune disorders such as systemic lupus erythematosus (SLE), inflammatory bowel disease (IBD), and Behçet's syndrome have been linked to an increased risk of VTE. Recent reports have further shown that a majority of autoimmune and immune-mediated disorders are linked to an increased risk of venous thrombosis, PE, or VTE. For instance, a Swedish nationwide study found that the risk of PE was increased in the first year after hospitalization for 33 different autoimmune disorders. Especially high risks were noted for several autoimmune diseases such as immune thrombocytopenic purpura, polyarteritis nodosa, polymyositis/dermatomyositis, ulcerative colitis, and SLE. Another study from England, also based on hospitalization data, found that immune-mediated disorders were associated with an increased risk of VTE compared with other medical causes of hospitalization. Multiple mechanisms may operate and disease-specific factors, such as cardiolipin antibodies, have been identified. However, inflammation by itself appears to change the hemostatic balance in a thrombogenic direction. Recent epidemiological studies, together with previous experimental and clinical studies, indicate that autoimmune disorders should not only be viewed as inflammatory disorders, but also hypercoagulable disorders. Research to identify thrombotic risk factors, elucidate the mechanisms involved, and investigate prophylactic regiments is needed. The present review describes the epidemiological, clinical, and experimental evidence for the connection between VTE and autoimmune and immune-mediated disorders.
静脉血栓栓塞症（VTE）是一个主要的健康问题，有时会并发致命的肺栓塞（PE）。凝血和抗凝系统紊乱是VTE的重要危险因素。比较研究表明，凝血和先天免疫有着共同的进化起源。因此，免疫和凝血系统相互关联并不奇怪，许多分子成分对这两个系统都很重要。全身炎症通过抑制纤维蛋白溶解、上调促凝因子和下调抗凝因子来调节血栓形成反应，而自身免疫性疾病如系统性红斑狼疮（SLE）、炎症性肠病（IBD）和白塞病与VTE风险增加有关。最近的报告进一步表明，大多数自身免疫性和免疫介导的疾病与静脉血栓形成、PE或VTE风险增加有关。例如，一项瑞典全国性研究发现，33种不同自身免疫性疾病住院后的第一年发生PE的风险增加。几种自身免疫性疾病如免疫性血小板减少性紫癜、结节性多动脉炎、多发性肌炎/皮肌炎、溃疡性结肠炎和SLE的风险尤其高。另一项来自英国的研究同样基于住院数据，发现与其他住院的医学原因相比，免疫介导的疾病与VTE风险增加有关。可能有多种机制在起作用，并且已经确定了疾病特异性因素，如心磷脂抗体。然而，炎症本身似乎会使止血平衡朝着血栓形成的方向改变。最近的流行病学研究以及先前的实验和临床研究表明，自身免疫性疾病不仅应被视为炎症性疾病，还应被视为高凝性疾病。需要开展研究以识别血栓形成风险因素、阐明其中涉及的机制并研究预防方案。本综述描述了VTE与自身免疫性和免疫介导的疾病之间联系的流行病学、临床和实验证据。
