Fatal renal failure in polymyalgia rheumatica caused by disseminated giant cell arteritis

A 62-year-old man with classical signs of polymyalgia rheumatica for one year suddenly developed fatal renal failure, despite treatment with steroids. Autopsy showed disseminated giant cell arteritis in the kidneys, pancreas, oesophagus, and larynx, with the most pronounced changes in the kidneys. All intrarenal arteries and arterioles were affected. The changes ranged from occlusive intimal proliferation to severe fibrinoid necrosis in the whole arterial wall. Fibrinoid necrosis was also found in the afferent and efferent arteries and several thromboses were seen in the glomeruli. It is not known why this apparently non-complicated case of polymyalgia rheumatica suddenly turned into a fatal case of disseminated giant cell arteritis. The only precipitating factor to be incriminated seems to be a reduction in the corticosteroid dose. This case adds further support to suggestions of the existence of a distinct type of systemic giant cell arteritis.