4例原发性肺动脉高压症(从状肺动脉病)的病理形态学观察

A MORPHOLOGICAL OBSERVATION OF PRIMARY PULMONARY HYPERTENSION (PLEXIGENIC PULMONARY ARTERIOPATHY)

原发性肺动脉高压症是一种罕见的原因不明的进行性疾病。系指原发于肺动脉的增生性病变,伴有丛状病变形成,以致肺动脉广泛阻塞,肺动脉压持续增高,继而右心肥大。本病常易误诊,预后不佳。本文报道4例典型原发性肺动脉高压症尸检病例,其病理组织学所见是:肌型肺动脉中膜肥厚;细动脉肌化;肌型肺动脉内膜纤维化及弹力纤维增生,分裂呈典型“洋葱皮样”结构;以及特征性丛状病变形成等。其丛状病变形成,可能是肺动脉高压形成后的继发性晚期改变(即反应性形态改变),而不是先天性畸形或血栓栓塞的后果。

Four autopsy cases of typical primary pulmonary hypertension were reported. The histopathological features were as follows: medial hypertrophy of muscular pulmonary arteries, muscularization of arterioles, intimal fibrosis and fibroelastosis of muscular pulmonary arteries having typical 'onion-skin' configuration, fibrinoid necrosis of the media or necrotizing arteritis of muscular pulmonary arteries and formation of highly characteristic plexiform lesions and dilatation lesions. It is thought that the plexiform lesions probably were the morphological changes responding to the pulmonary hypertension rather than being the congenital abnomality or organization of thrombi.