Selective Angiography in Arteritis of the Aorta and Its Major Branches

The Syndrome of aortitis and arteritis of unknown origin is recognized as a distinct entity although it has many different names: Takayasu's arteriopathy or pulseless disease (1), Takayasu's syndrome (2), young female arteritis (3), primary arteritis of the aortic arch (4), branchial arteritis or aortic arch arteritis (5), reversed coarctation (6), brachiocephalic arteritis (7, 8), Martorell's syndrome (9), chronic subclavian carotid obstruction (10), arteritis of the aorta in young women (11), idiopathic aortitis in young Africans (12), aortitis of obscure origin (13), idiopathic panarteritis (14), and arteritis of the aorta and its major branches (15). The disease occurs mainly in non-Western populations, in children, young adults of both sexes, and older women, but is rare in adult males (22, 30). It has been reported from many countries, including Japan (16), Singapore (17), India (18), Southern Rhodesia (19), South Africa (12–15), Nigeria (20), Spain (2), Scotland (21), Sweden (22), Germany (23), the U.S.A. (24), Cuba (10), Hungary (25), and Greece (26). The larger elastic arteries are usually affected, and the morbid anatomy is typical. There is focal or diffuse loss of muscular and elastic tissue with extensive fibrosis of the media and destruction of the elastic lamina. Recently involved areas show fibroelastic proliferation with ingrowth of new capillaries resembling gran-ulomata. Where the process is advanced, the vessel wall consists of homogeneous acellular collagen only. The intima is involved, and superadded thrombosis occurs. The gross fibrotic intimal thickening seen in the later stages is due partly to intimal hyperplasia and partly to organization of the. thrombi. The adventitia is also affected and is infiltrated with inflammatory cells. Perivascular cuffing may be seen around the vasa vasorum. Stenosis or obstruction is a result of the intimal proliferation and organization of thrombi, while dilatation and aneurysm formation take place where the media is destroyed and the residual intima and adventitia balloon outward (12, 15, 17). Superadded atheroma may result from incorporation of organized thrombus into the vessel wall or be part of premature atherosclerosis in a hypertensive patient. The atheromatous areas calcify and produce the linear shadow seen on the radiograph (18). Many sites are involved: the arch and descending thoracic aorta, the subclavian and carotid arteries, producing unequal pulses or cerebral syndromes (1, 9, 12–15, 20, 22, 24), and rarely the coronary arteries leading to myocardial infarction (4, 15). If the lumen of the thoracic aorta is narrowed sufficiently or if there is narrowing or occlusion of the abdominal aorta, the femoral pulses are absent and the clinical picture simulates congenital coarctation of the aorta (11, 13–15, 17, 28). Renal artery stenosis or obstruction produces severe hypertension (12, 17, 29).