Giant cell arteritis.

Abstract

Giant cell arteritis (GCA) is an immune-mediated vasculitis, affecting medium- to large-sized arteries, in individuals over the age of 50 years. Visual loss is a frequent complication of GCA, and once it occurs it tends to be both permanent and profound. Although major advances have been made in recent years in genetics, molecular biology and the description of the vessel wall morphology, the aetiology and pathogenesis of GCA are still incompletely understood. Over the years there has been much debate over whether polymyalgia rheumatica and GCA are separate or linked entities. Recent investigations support that polymyalgia rheumatica and GCA are two different expressions of the same underlying vasculitic disorder. A single cause or aetiological agent has not as yet been identified. Except for the histopathology of the arterial wall, there are no laboratory findings specific for GCA, and no particular signs or symptoms specific for the diagnosis. GCA typically causes vasculitis of the extracranial branches of the aorta and spares intracranial vessels. Transmural inflammation of the arteries induces luminal occlusion through intimal hyperplasia. Clinical symptoms reflect end-organ ischaemia. Branches of the external and internal carotid arteries are particularly susceptible. Corticosteroids remain the only proven treatment for GCA, the regimen initially involving high doses followed by a slow taper. However, early detection and treatment with high-dose corticosteroids is effective in preventing visual deterioration in most patients.