Fujinami M, Morimoto S, Nishikawa T, Sato A, Yamada Y, Kajita A
Acta Pathol Jpn. 1987 Mar;37(3):401-12.
Eight autopsy cases of primary pulmonary hypertension were studied. All cases were diagnosed as primary plexogenic arteriopathy. Microscopical study of the lungs showed in every case hypertensive arterial changes of Grade II-VI after Health & Edwards' criteria. Truncal pulmonary artery of three cases revealed aneurysm or severe dilatation with thrombus, and dilation was also recorded in another three cases. Histology of gross pulmonary artery in several cases presented disruption and defect of elastic fiber and alcian-blue positive microcystic change in media, lymphoid cell infiltration in outer media and inner adventitia, adventitial thickening, and intimal fibrosis. The liver of 7 cases showed secondary congestive changes and in one case intrahepatic portal vasculitis.
对8例原发性肺动脉高压尸检病例进行了研究。所有病例均诊断为原发性丛状动脉病。肺部显微镜检查显示，根据Health & Edwards标准，每个病例均有II - VI级高血压性动脉改变。3例患者的肺动脉主干出现动脉瘤或严重扩张并伴有血栓形成，另外3例也有扩张记录。几例大体肺动脉的组织学表现为弹性纤维断裂和缺损，中膜有阿利新蓝阳性微囊性改变，外膜和内膜有淋巴细胞浸润，外膜增厚和内膜纤维化。7例患者的肝脏出现继发性充血性改变，1例出现肝内门静脉血管炎。
