New Treatment Strategies in Large-vessel Vasculitis

Sebastian Unizony; John H. Stone; James R. Stone


Curr Opin Rheumatol. 2013;25(1):3-9. 

In This Article

Takayasu Arteritis

TAK is a relatively rare form of vasculitis with tropism for the aorta and its main branches as well as the pulmonary vasculature.[30] TAK primarily affects women of childbearing-age across all races and geographic locations, but Asians and South Americans demonstrate higher incidence.[31] As in GCA, glucocorticoids are still the mainstay of treatment for TAK.[5] Unfortunately, relapses, glucocorticoid dependence, and subclinical radiographic progression are seen in more than two-thirds of the patients, and, as in GCA, there is no proven glucocorticoid-sparing alternative. Conventional immunosuppression (e.g. methotrexate, azathioprine, mycophenolate mofetil) has shown limited potential for glucocorticoid sparing in uncontrolled series.[32–34] Retrospective and open-label studies[35–38] suggest that TNF-α-directed therapies might have a role in the treatment of TAK, but confirmatory controlled studies are lacking.

In TAK, early vascular lesions consist of T cells, natural killer (NK) cells, and macrophages. Granuloma formation and giant cells can subsequently be found in the media of elastic arteries. Late-stage ('burned out') damage demonstrates extensive fibrosis, and intimal hyperplasia, which may lead to aneurysm formation or arterial stenosis. In contrast with GCA, a pathogenic role for B cells has also been suggested in TAK by not only the identification of nonspecific polyclonal hypergammaglobulinemia and circulating antiendothelial antibodies,[39] but also the demonstration of increased numbers of plasmablasts in peripheral blood[40] and the presence of B cells infiltrating the adventitia of patients' aortic samples.[41]