Studies on the Pathogenesis of Pulseless Disease Especially on Experimental Angitis by Elastase

Twenty-three patients of pulseless disease were described with clinical and histopathological findings. Twenty-two cases whose ages range 16 to 41 were female and one was male. In nineteen of twenty-three cases surgical treatments were performed: removal of carotid body in 7, excision with Tetron graft in 4, by-pass prosthesis with Tetron in 5 and autogenous vein graft in 2 cases. Biop· sies were taken in 13 cases, and the histological features were classified into 4 types: adventitis type in 8 cases. tuberculoid granuloma type in one case, giant cell arteritis type and diffuse productive inflammation type in two cases. The main lesions were lirr.itted to the media and the adventitia, and the intimal changes seemed to be secondary reaction, and the vasa vasorum may be primary sites of involvement of pulseless disease. The lesion of the elastic fibres seems to play an important role in the pathogenesis. In order to clarify the exact etiologic relationship of primary elasticopathy to granulomatous reaction with elasticophagic giant cell, elastase suspension was infused in the common carotid artery of the rabbits intravascularly (37 cases) and extravascularly (21 cases), and elastolysis of the arterial wall was made, thereby, the elastogranulomous reaction and multinucleare giant cells phagocy· tosing the fragments of disintegrated internal elastic lamina were recognized in 2 cases (Figs. 15 and 16 ). From the results of these experiments, it is clear that a causal relation exists between degenerating elastic fibres and granulomatous tissue reaction accompany· ing giant cell formation in vivo, and elastase has an elastolytic activity in vivo, and the fragments of the elastic fibres act as foreign bodies. The following conditions are necessary for the occurrence of elasticophagic giant cells: a specific destruction of the elastic fibres to provoke giant cells, for example, hard and slightly soluble fragments of the internal elastic lamina, and a mesenchymal activation brought about by some factors, one of which was typhoid-paratyphoid vaccination in this experiment. The change in early stage of the elastic fibre by elastase was not observed in vivo, but at the incubation before elastolysis, metachromasia of the elastica became negative and no initial increase of the metachromatic reaction was observed. A newly formed elastic fibre was presented in early stage in the intimal thickening, but the regeneration of disintegrated elastic fibre was not seen in the 111[ Jl I 1'f Received for publication April 15, 1966.