Pulmonary vascular disease with pulmonary hypertension in collagen disease Takashi Sawai 1 , Masahisa Kyogoku 1 11st Department of Pathology, Tohoku University School of Medicine pp.59-67
Published Date 1986/1/15
DOI https://doi.org/10.11477/mf.1404204805
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In collagen disease, pulmonary hypertension (PH) have been considered to be intimate relation with pulmonary fibrosis, but there are some cases with pulmonary vascular disease which show the severe pulmonary hypertension. So in relating to PH, his-topathological study was performed on arterial le-sion of collagen disease.

In 48 autopsy cases, it was proved that 10/33 in SLE, 6/7 in PSS, 1/3 in MRA, 1/3 in PM (DM), 1/1 in UCTD and 1/1 in MCTD had the pulmonary vascular lesions (VL), such as intimal thickening, fibrous occlusion of lumen, medial hy-pertrophy, plexiform lesion, angiomatoid lesion, gran-ulation in adventitia, and necrotizing angitis. On the other hand histometrical study was used for con-firmation of medial hypertrophy of arteries. In ac-cordance with VL and PH, the cases which were used in this study were classified into four groups, PH (+) VL (+), PH (+) VL (-), PH (-) VL (+) and PH (-) VL (-).

For inspecting the distribution and early stage of vascular lesion, the two types of arterial lesion, ex-udative and sclerotic type of PSS and SLE, were followed by serial section. From these results, though the features in advanced stage of two groups are fairly different, the arterial lesion in beginning sta-ge occurred about the same order of small arteries.

As for the pathogenesis of vascular lesion in col-lagen disease, Raynaud's phenomenon and Immune Complex are thought to be one of main causes, but it is not directly confirmed until now.

Copyright © 1986, Igaku-Shoin Ltd. All rights reserved.


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