Clinical diagnosis and management of large vessel vasculitis: Takayasu arteritis.

Takayasu arteritis (TA) is 1 of the 2 main causes of large vessel vasculitides (LVV), giant cell arteritis being the other. LVV can also develop in various other systemic diseases. In TA, a wide variety of symptoms result from vascular stenoses, occlusions, and dilation. Aneurysms may develop and may occasionally dissect or rupture. Disease activity can sometimes be difficult to assess clinically. Diagnostic modalities also have their shortcomings. Often, acute phase reactants do not accurately detect disease activity. Available vascular imaging modalities may be acceptable in defining vascular anatomy, but are notoriously inaccurate in delineating vascular inflammation. Glucocorticoids remain the cornerstone of therapy in TA, in spite of foreseeable long term side effects. In addition, several steroid-sparing agents are also being used, often based on promising results from small uncontrolled studies. Rarely, endovascular revascularization procedures are necessary. Resection of critical-sized aortic aneurysms and repair of aortic dissections are occasionally warranted as lifesaving procedures. The long term outcome of surgical intervention is often unfavorable and relapses are not uncommon. In addition to TA, other less commonly encountered causes of LVV are also briefly discussed in this review.