Large vessel vasculitides

Abstract

Giant cell arteritis (GCA) and Takayasu's arteritis (TA) are the two primary large vessel vasculitides. They are idiopathic systemic inflammatory conditions characterized by granulomatous inflammation of large and medium-sized arteries. TA affects a younger age group, predominantly involves the aorta and its main branches, and tends to be a two-phase disease with inflammatory and occlusive stages. In contrast, GCA affects an older age group and classically involves the temporal and other cranial arteries, although extracranial GCA can affect the aorta and its primary branches. Constitutional symptoms are a feature of both diseases and vascular symptoms are dependent on the pattern of arterial involvement. Temporal artery biopsy and angiography remain the gold standard for diagnosis of GCA and TA, respectively. New imaging modalities are challenging this practice and have the potential to allow early diagnosis and monitoring of disease activity. Over the last decade treatment has seen major advances and, with the advent of vigorous immunosuppressive therapy, new means for intervention with percutaneous transluminal angioplasty supported by stenting and biological therapies on the horizon, the prognosis of patients with large vessel vasculitis seems likely to improve.