Large vessel vasculitides

Abstract

Large vessel vasculitis encompasses two primary conditions: giant cell arteritis (GCA), the most common adult vasculitis in Europe, and Takayasu arteritis (TA). These are idiopathic systemic inflammatory conditions characterized by granulomatous inflammation of large and medium-sized arteries. TA typically 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 it can affect the aorta and its branches (large vessel/extracranial GCA). Constitutional symptoms are a feature of both diseases; vascular symptoms depend on the pattern of arterial involvement. Temporal artery biopsy and angiography remain the gold standards for diagnosis of GCA and TA, respectively. In recent years, validation of ultrasound Doppler imaging of the temporal and axillary arteries has facilitated the development of fast-track pathways to diagnose GCA without the need for invasive biopsy. Although corticosteroids remain the cornerstone of treatment for large vessel vasculitis, tocilizumab, a biologic therapy, is now considered to be part of the standard treatment for refractory/relapsing disease. Percutaneous and surgical interventions are largely confined to patients who develop complications of the disease.