Am J Cardiovasc Dis 2012;2(3):171-183
Review Article
Autoimmune diseases and venous thromboembolism: a review of the
literature
Bengt Zöller, Xinjun Li, Jan Sundquist, Kristina Sundquist
Center for Primary Health Care Research, Lund University/Region Skåne, Clinical Research Centre, Floor 11, Building 28,
Jan Waldenströms gata 35, Skåne University Hospital, 205 02, Malmö, Sweden; Stanford Prevention Research Centre,
Stanford University School of Medicine, Medical School Office Building, 251 Campus Drive, Mail Code 5411, Stanford,
California 94305-5411, USA
Received May 7, 2012; accepted June 5, 2012; Epub July 25, 2012; Published August 15, 2012
Abstract: 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. (AJCD1205001).
Keywords: Autoimmune diseases, immunology, inflammation, rheumatic diseases, inflammatory bowel diseases,
venous thrombosis, venous thromboembolism, pulmonary embolism, blood coagulation disorders
Address all correspondence to:
Dr. Bengt Zöller
Center for Primary Health Care Research
Lund University/Region Skåne
Clinical Research Centre
Floor 11, Building 28, Jan Waldenströms gata 35
Skåne University Hospital, 205 02
Malmö, Sweden.
Tel: +46 70-6691476; Fax: +46 40-391370
E-mail: bengt.zoller@med.lu.se
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