Spectrum of giant cell vasculitis

Abstract

Giant cell arteritis (GCA) is the commonest primary systemic vasculitis in the United States. Severe outcomes include blindness and stroke, and death may result from aortic dissection. Temporal artery biopsy remains the gold standard for diagnosis. Magnetic resonance imaging (MRI) of involved vessels shows promise as a useful noninvasive method for diagnosis and assessment of disease activity. Corticosteroid therapy is effective but is associated with considerable morbidity. Longitudinal studies with large numbers of patients are required to identify appropriate steroid-sparing agents. New insights into the immunopathogenesis of GCA have allowed us to identify heterogeneous subsets of patients with varying clinical presentations corresponding to specific cytokine profiles. The concept of the involved artery as an active participant in the events leading to luminal obstruction has been realized and provides the opportunity to evaluate novel therapies to modify the course of the disease.

Giant Cell Arteritis as a Cause of First-Ever Stroke

“…GCA is the commonest primary systemic vasculitis in the USA [12]. The incidence of GCA worldwide is estimated to be 20 per 100,000 persons aged >50 years [3].…”

“…All authors emphasized that steroids are effective in GCA, and this is the main means of prevention of blindness [3,10,11,12]. However, during the first 2 weeks of steroid therapy for GCA, stroke can occur [23, 24].…”

“…The superficial temporal, vertebral, ophthalmic and posterior ciliary arteries are more often involved than the external and internal carotid arteries, while involvement of the intracranial arteries is rarely seen [3,10,11,12]. …”

“…GCA is a rare cause of stroke (3–7%), most often in the vertebrobasilar territory [14, 15]. Corticosteroid therapy is the basic and most common treatment, the main goal being prevention of blindness [2, 3, 12]. If transient ischemic attacks or strokes occur, a pulse of methylprednisolone (500–1,000 mg/day) for 3 days is the treatment of choice [2, 3].…”

Aortitis

“…The ideal radiologic tool in aortitis is one that not only evaluates vessel aperture but one that also distinguishes active from inactive sites of disease with a high degree of sensitivity and specificity. [3] Cogan's syndrome [4] Kawasaki disease [5] Sarcoidosis [6] Spondyloarthropathies [7] Systemic lupus erythematosus [8] Rheumatoid arthritis [9] Behçet's syndrome [10] Myelodysplastic syndrome [11] Relapsing polychondritis [12] Infectious agents Syphilis [13] Tuberculosis [14] Bacteria [15,16] Streptococcus (rheumatic fever) […”