Thrombosis in vasculitis: An updated review of etiology, pathophysiology, and treatment

Thromboembolic disease is a complication of many vasculitides. A common observation is that thromboembolic events coincide with the period of vasculitic disease, but the mechanism by which this occurs remains unclear. Inflammatory thrombosis is now recognized as a symptom of arteritis rheumatic, and vasculitides such as Behçet's syndrome (BS), and anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) or giant cell arteritis (GCA). This systematic review aimed to explain recent findings related to etiology, pathophysiology, and treatment methods for BS, AAV, and medium/large-vessel vasculitis.

A comprehensive literature search on English sources from PubMed, Scopus, MEDLINE, Science Direct, ProQuest, AIM, CINAHIL, and ELDIS databases was used to find the relevant articles and reports. The relevant papers (having full text) were obtained until June 2023. Two independent reviewers screened the titles and abstracts of the obtained articles, and a third arbitrator resolved disputes between the reviewers.

It is becoming increasingly clear that certain systemic inflammatory diseases, like vasculitis, are linked to a higher risk of both venous and arterial thrombosis. An increased incidence of thromboembolic disease in AAV has been noted, particularly during times of active disease. Growing evidence supports the use of immunosuppression in the management of venous thrombosis in vasculitis. These patients also have a higher risk of developing ischemic disease.