Nasu, Tsuyoshi
May 1963
Angiology;May1963, Vol. 14 Issue 5, p225
Academic Journal
A systematic study and critical review were given on 21 autopsy cases reported in Japan, the cases clinically belonging to the category of pulseless disease. 1. Coinciding with the clinical manifestations, 18 out of the 21 eases proved to be young women or female infants. By Wasserman reaction of the serum before death all the 19 cases examined were negative and in the tuberculin test 11 of the 15 cases examined turned out to be positive. 2. This disease is essentially mesarteritis of the aortic arch and larger stem arteries branching out from the aorta, which shows granulomatous or diffuse productive inflammation of media and adventitia by way of vasa vasorum, complicated with a marked secondary-juvenile arteriosclerosis and culminating in severe stenosis of the arterial lumina. The histologic picture of arteritis resembles that of syphilitic arteritis but it is different from that of Buerger's disease. Those cases that are at the scarring stage of arteritic changes, develop thickening of the arterial wall and shortening of the artery's length, a state which might be called arteriosclerocirrhosis obliterans juvenilis. 3. The preferred sites of arteritic changes, as suggested by pulseless symptoms, are mainly aortic arch and cranial stem arteries such as subclavian or carotid arteries, but the changes further spread to thoracic and abdominal aorta and also caudal stem arteries such as coeliac or renal arteries and often they are found invading the pulmonary arterial trunk. Therefore, nosologically it would be more appropriate to call this disease ‘troncoarteritis productiva granulomatosa.’ 4. The cause of this disease is not established at present, although some cases ,show evidences suggestive of a tuberculous nature, which might indicate a multiple etiologic agent. 5. The fact that the incidence of disease is decidedly higher in young females seems to suggest that age and sex factors are concerned with the development of secondary juvenile arteriosclerosis.


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