Large vessel vasculitides

Abstract 

The two major primary large vessel vasculitides are giant cell arteritis (GCA) and Takayasu's arteritis (TA). Both are idiopathic systemic inflammatory conditions characterized by granulomatous inflammation of large and medium-sized arteries. Although they share similar histological features, the demographics and clinical presentation of the two diseases are quite different. TA affects a younger age set, predominantly affects the aorta and its main branches and tends to be a two-phase disease with inflammatory and occlusive stages. GCA increases in prevalence with age and classically affects the temporal and other cranial arteries, although the aorta and its primary branches can be affected. Constitutional symptoms are a feature of both diseases. Vascular symptoms are dependent on the pattern of arterial involvement. Headache, jaw claudication and visual disturbance are typical symptoms of GCA. Limb claudication and diminished limb pulses are characteristic of TA. Temporal artery biopsy and angiography remain the gold standard for diagnosis of GCA and TA, respectively. New imaging modalities are challenging this practice; they also have the potential to allow monitoring of disease activity. Treatment has seen major advances in the last decade. With the advent of vigorous immunosuppressive therapy and new means for intervention with percutaneous transluminal angioplasty supported by stenting, it is hoped that the prognosis of patients with large vessel vasculitis will improve.

Keywords: angiography, giant cell arteritis, immunosuppression, large vessel vasculitis, Takayasu arteritis