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ANCA-associated Vasculitis: A Prothrombotic State Even in Remission?

It is well established that active antineutrophil cytoplasmic antibody-associated vasculitis (AAV) — granulomatosis with polyangiitis (Wegener’s; GPA) or microscopic polyangiitis — is associated with a very high risk of venous thromboembolic events (VTE): about 7 per 100 person-years1,2,3,4, compared to 0.15–0.31 in the general population5,6,7. Risk probably remains elevated, although to a much lower degree, during remission. Such risk has been calculated (1.0 per 100 person-years) in only 1 study4 and was not formally compared to the published data on healthy persons available at the time5; but dissection of data from the other studies of VTE in AAV1,2,3 and addition of data from new and very large cohorts of healthy persons support the statement that the relative risk of VTE during quiescent AAV is elevated, probably to about the same degree as in rheumatoid arthritis or inflammatory bowel disease6,7,8. The increased risk of VTE in AAV, regardless of disease activity, cannot be attributed to the known risk factors for VTE2,4,9.

It is on this background that Hilhorst, et al looked for evidence of hypercoagulability in patients with quiescent AAV10. At first glance, it might appear that investigating active AAV would be more fruitful, but since abnormalities related to … 

Address correspondence to Dr. Monach. E-mail: pmonach{at}bu.edu