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Giant cell arteritis is a chronic, granulomatous inflammation of the temporal arteries that affects persons over the age of 50.1 Temporal arteritis is sometimes the first evident sign of a more disseminated disease and involvement of all the larger arteries including the aorta has been observed.2 Many studies describe appearance of murmurs and the formation of aortic aneurysms in patients suffering from temporal arteritis.3Weak extremity pulses, arm claudication and colour changes of Raynaud’s phenomenon type may occur, symptoms resulting from the narrowing or occlusion of branches of the thoracic aorta.2Death from rupture of aneurysms in giant cell arteritis is well documented.3 4 It has also been suggested that the extra-articular disease polymyalgia rheumatica5 is an expression of the generalised form of giant cell arteritis and the name polymyalgia arteritica may be more appropriate.6
A 74 year old woman was admitted to the university hospital in Lund because of tenderness over the right masseter muscle. It propagated towards the temporal region and spread similarly on the left side and over the back of the head. On admission the erythrocyte sedimentation rate (ESR) was 42 mm 1st h and serum electrophoretic analysis indicated slight inflammatory activity. The patient presented with a minor exophtalmus resulting from an earlier episode of Grave’s disease, but was otherwise previously healthy and in good physical condition. The subcutaneous temporal vessels were swollen and tender. On the diagnosis of probable temporal arteritis, 30 mg/day of prednisolone were prescribed. A biopsy, showing inflammatory cell infiltrates in the arterial wall, verified the diagnosis. The ESR decreased and the symptoms declined and the prednisolone was reduced to 5 mg/day. On several check ups over five years, there were no signs or symptoms of active disease and the ESR was at all times below 15 mm 1st h. At the age of 79, the patient collapsed during a walk and was declared dead upon arrival of the ambulance.
Necropsy revealed in the aortic trunk, 3 cm above the valvular plane, a rupture within a small area of pronounced wall thinning (< 0.5 mm) and haemopericardium. There were no aortic aneurysm formation or dissection and only minor atheromatotic plaques. Microscopically, the aorta and the left common carotide unexpectedly showed widespread infiltrates of lymphocytes, plasma cells, and scattered multinucleated giant cells (fig 1). The inflammation was intense in the aorta, showing cell infiltrates in all vessel wall layers, accompanied by oedema/necrosis and multifocal pronounced dissolution of the elastic fibres (fig 2), shown by staining for collagen IV and laminin. Close to the rupture, one arteriole was totally obliterated by inflammatory cells. Some minor arteriosclerotic lesions were seen and focal precipitation of metachromatic material, representing myxoid degeneration. The remaining temporal artery was not examined, as active arteritis had not been suspected at the time of necropsy.
Pathogenetically, we suggest that the intense aortic inflammation dissolved the elastic fibres, and attenuated the wall. It also obliterated some of the afferent arterioles (vasae vasorum), leading to multifocal ischaemic wall necrosis, followed by rupture of the vessel wall, without prior formation of an aneurysm. Despite focally intense inflammatory infiltrates, it is impossible to determine whether this was an acute exacerbation of the disease or part of a chronic, active inflammation. Clinically, however, there was no sign of disease activation. It is well known that giant cell arteritis can lead to death by rupture of aortic aneurysm.3 In this patient, there was inflammatory and ischaemic aortic wall necrosis without an aneurysm. This has, to our knowledge, not been reported before.
We are indebted to Professor Emeritus Nils Jonsson, for his critical examination of this report.
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