Large vessel vasculitides: update for the cardiologist

To evaluate new developments in the diagnosis and management of large vessel vasculitis, and to explore its relationship to atherosclerosis.

Although large vessel inflammatory disorders (giant cell arteritis and Takayasu arteritis) are the most common form of systemic vasculitis in adults, they remain incompletely understood and represent a challenge for rheumatologists and cardiologists. Diagnostic strategies rely on obtaining representative histological evidence of disease, or on using effective imaging techniques, that are only now being defined. The natural history of some of these conditions, such as Takayasu arteritis, includes a very long period of low level symptoms or even no symptoms, so that the diagnosis is made at an advanced stage of disease in many cases. Development of a robust system for evaluating disease activity is required in order to test the effectiveness of different therapies, ideally based on our understanding of their pathogenesis. A relatively newly recognized condition of IgG4-related disease includes inflammatory large vessel disease in a substantial number of cases; furthermore, better imaging techniques are being used to recognize the presence of isolated aortitis in patients with unexplained systemic disease. Long-term vascular complications are increasingly recognized, and this is blurring the margin between atherosclerosis and vasculitis, which may improve our understanding of both these conditions.

Large vessel vasculitis is the most common primary vasculitis. Better imaging and a wider understanding of its potential long-term complications should lead to improved quality of care in future.