Venous thromboembolism in systemic autoimmune diseases: A narrative review with emphasis on primary systemic vasculitides

Abstract

Venous thromboembolism (VTE) is a prevalent multifactorial health condition associated with significant morbidity and mortality. Population-based epidemiological studies have revealed an association between systemic autoimmune diseases and deep venous thrombosis (DVT)/VTE. The etiopathogenesis of increased risk of VTE in systemic autoimmune diseases is not entirely clear but multiple contributors have been explored, especially in the context of systemic inflammation and disordered thrombogenesis. Epidemiologic data on increased risk of VTE in patients with primary systemic vasculitides (PSV) have accumulated in recent years and some of these studies suggest the increased risk while patients have active diseases. This could lead us to hypothesize that venous vascular inflammation has a role to play in this phenomenon, but this is unproven. The role of immunosuppressive agents in modulating the risk of VTE in patients with PSV is not yet clear except for Behçet's disease, where most of the studies are retrospective. Sensitizing physicians to this complication has implications for prevention and optimal management of patients with these complex diseases. This review will focus on the epidemiology and available evidence regarding pathogenesis, and will attempt to summarize the best available data regarding evaluation and treatment of these patients.

Plasma ADAMTS13, von Willebrand Factor (VWF), and VWF Propeptide Profiles in Patients With Connective Tissue Diseases and Antiphospholipid Syndrome

“…The etiopathogenesis of the increased risk of VTE in systemic autoimmune diseases is not entirely clear but multiple contributors have been explored. 1 Antiphospholipid antibody syndrome (APS) 2,3 is a well-known systemic thrombotic diathesis that is associated with the presence of antiphospholipid antibodies (aPLs). The mechanisms underlying the development of thrombosis, including cerebral thrombosis 4 and VTE, 5 and obstetric morbidity 6 due to aPLs are poorly understood.…”

Intravenous Immunoglobulin as an Immunomodulating Agent in Antineutrophil Cytoplasmic Antibody–Associated Vasculitides: A French Nationwide Study of Ninety‐Two Patients

“…We did not observe any stroke or myocardial infarction, but 4 patients had venous thromboembolism. They all were older than 50 years and had other risk factors (including metastatic cancer in 2 of them) in addition to active vasculitis, which is also a known risk factor for venous thromboembolism (36,37). Five patients had an increase of .30% in the serum creatinine level, but imputing the cause to IVIG would be debatable, since 3 of them had active glomerulonephritis.…”

Inflammation and thrombosis – testing the hypothesis with anti- inflammatory drug trials

“…Systemic inflammation can participate in the pathogenesis of the increased rates of thrombosis documented in other autoimmune diseases, such as autoimmune haemolytic anaemia, immune thrombocytopenic purpura, systemic lupus erythematosus, systemic vasculitides, the antiphospholipid antibody syndrome, Sjögren's syndrome, and systemic sclerosis (66)(67)(68)(69)(70). Similarly, increased microvascular thrombosis is found in chronic and acute infections and in sepsis, where it contributes to multiple organ dysfunction.…”