多発性筋炎にPlexogenic Arteriopathyを伴う肺高血圧症を合併した1症例

An autopsy case of pulmonary hypertension with plexogenic arteriopathy in polymyositis

　膠原病にみられる肺高血圧症（以下PH）には肺線維症に伴う二次的なものと^1），肺線維症はみられないが，中小の血管，特に動脈にさまざまな病変を伴うものがある。後者についてはSLEの壊死性血管炎やplexiformlesion^2），PSSの線維性内膜肥厚がよく知られている^3）。しかし，他の膠原病での報告は少なく，その特徴は把握しにくい。今回，私どもは多発性筋炎に合併したPHの剖検例を経験し，肺動脈病変を中心に解析したので報告する。

A 59-year-old woman had been under management for polymyositis about 5 years. In the course of treatment, pulmonary hypertension was pointed out by X-ray, cardiac cathetelization and so on. She died by cardiac insufficiency in spite of intensive care and therapy. Autopsy revealed the right ven-tricular hypertrophy, 8 mm in thickness (2 mm in mean thickness) and luminar dilatation of pulmona-ry arteries and that histologically in the absence of pulmonary fibrosis, plexogenic arteriopathy, di-latation lesion and medial hypertrophy of small pulmonary arteries.

In collagen diseases, two forms of pulmonary hypertension have been known, one is secondary occurred to pulmonary fibrous and the other shows pure arterial changes such as plexogenic pulmonary arteriopathy. In latter case, necrotizing angitis in SLE and fibrotic occulusion in PSS have been ob-served. So this case is interesting but rare that, in addition to SLE and PSS, the polymyositis have the plexogenic arteriopathy with severe pulmonary hypertension.