Giant cell arteritis: A review

Abstract

This excellent review covers all the important clinical and laboratory features of giant cell arteritis. Etiological aspects are considered but the conclusion is reached that there is little evidence for any of the theories which have been put forward, such as the precise relationship to aging, drug exposure, an infectious agent, an hereditary predisposition or a relationship to neoplasia. Support for a disturbance of immune mechanisms comes from observing the deposition of immunoglobulins in the walls of involved vessels and evidence that lymphocytes may be sensitized to a component of the arterial wall - most likely elastin. There has been increasing recognition of the disease so that the incidence in 1970-74 was 17.4 per year and histological evidence for the disease has been found in 1.7% of unselected post-mortems. All reviews have confirmed the age of onset to be in the older age groups with a slight predominance of women. The quite varied initial clinical manifestations are documented - these include both non-specific physical and mental changes and fever as well as the syndrome of polymyalgia rheumatica. It is noted that an overt synovitis is recognised in some patients. The symptoms and signs reflecting the more usual involvement of arteries arising from the arch of the aorta are described. The symptoms and signs of cranial arteritis - headache and scalp tenderness, the sequellae of ocular vessel involvement - ptosis, transient blurring, dipoplia and visual loss, compromised circulation to the skeletal musculature - claudication of the muscles of mastication, the tongue, calf and arm and actual necrosis of tissue including an extremity, scalp, or tongue have been described. The signs of the aortic arch syndrome including the angiographic appearances, with the occasional occurrence of cerebral infarction and even myocardial infarction are noted. The laboratory tests are reviewed including the characteristic rise in the sedimentation rate and non-specific changes in the formed elements of the blood, plasma proteins, immunoglobulins and complement fractions. Occasional reports of parenchymal disturbances involving the liver and kidney are reviewed. The authors highlight the important diagnostic signs and describe treatment with corticosteroids, emphasising the need for gradual reduction in dosage with reference to both any possible secondary rise in the sedimentation rate or relapse of symptoms.