Clinical practice. Giant-cell arteritis and polymyalgia rheumatica

Abstract

A 79-year-old woman presents with new-onset pain in her neck and both shoulders. She takes 7.5 mg of prednisone per day for giant-cell arteritis. Occipital tenderness and diplopia developed 11 months before presentation. At that time, her erythrocyte sedimentation rate was elevated, at 78 mm per hour, and a temporal-artery biopsy revealed granulomatous arteritis. The diplopia resolved after 6 days of treatment with 60 mg of prednisone daily. Neither headache nor visual symptoms developed when the glucocorticoids were tapered. How should this patient’s care be managed?

Figure 1. CTA and MRA of the Aortic Arch in Two Patients with Giant-Cell Arteritis

Computed tomographic angiography (CTA) (Panel A) shows the results of aortic-root repair and aortic-arch replacement with an “elephant trunk graft” in a 71-year-old woman who had biopsy-confirmed giant-cell arteritis. The graft terminates in the proximal descending thoracic aorta. Arrows indicate an aneurysm that is maximally dilated at the proximal descending thoracic aorta. The image was created with data from contiguous axial images reformatted in the sagittal and coronal planes. Contrast-enhanced magnetic resonance angiography (MRA) (Panel B) shows the aortic arch and its branches in a 72-year-old woman with biopsy-positive giant-cell arteritis. Arrows indicate stenotic lesions in the bilateral subclavian and axillary arteries, and arrowheads indicate long-segment occlusions of the proximal brachial arteries. Images courtesy of Dr. D. Fleischmann (Panel A) and Dr. F. Chan (Panel B), Department of Radiology, Stanford University.

Figure 2. Therapeutic Approaches to Giant-Cell Arteritis and Polymyalgia Rheumatica

The management of giant-cell arteritis (Panel A) depends on the disease stage. Therapeutic interventions for newly diagnosed disease (induction therapy), chronic disease (maintenance therapy), and disease flares (management of flares) are outlined. Glucocorticoids remain the cornerstone of therapy. Other immunosuppressive agents tested to date provide only marginal or no glucocorticoid-sparing benefits. The management of polymyalgia rheumatica (Panel B) has much lower glucocorticoid dose requirements. CRP denotes C-reactive protein, and ESR erythrocyte sedimentation rate.