THE HISTOPATHOLOGY OF 36 CASES OF PLEXOGENIC PULMONARY ARTERIOPATHY
THE HISTOPATHOLOGY OF 36 CASES OF PLEXOGENIC PULMONARY ARTERIOPATHY
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DOI：
10.1111/j.1365-2559.1990.tb01054.x
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发表时间：
1990-01-01
期刊：
影响因子：
6.4
通讯作者：
SMITH, P
中科院分区：
文献类型：
--
作者：
CASLIN, AW;HEATH, D;SMITH, P
本文对36例因肺动脉丛形成而需行心肺联合移植术的肺进行了详细的组织病理学观察。它揭示了两个不同的过程参与这种疾病的发病机制。一种是组织学表现与肌性肺动脉收缩一致，这种情况可能会被肺血管扩张剂逆转。另一个是肺动脉内膜和管腔中肌成纤维细胞的增殖，这种生长障碍不太可能受到这种类型的治疗的影响。在以前的超微结构研究中，我们已经表明，这些细胞的肌肉谱系的来源是肌肉细胞从内一半的媒体迁移到内膜通过内弹性膜的间隙。在本研究中，我们发现了一个类似的增殖肌成纤维细胞在内膜，不仅肺动脉，而且肺静脉，在丛性肺动脉病。动脉血栓被认为是一种并发症，而不是原因的丛性肺动脉病。认为肺中的噬铁体、胆固醇肉芽肿和局灶性纤维化是病程早期肺内出血的结果。结论是，虽然丛性肺动脉病有一个重要的血管收缩元素，它也是基于一个障碍的细胞生长的肌肉peidgree。这一观点对原发性丛源性肺动脉病的治疗具有明确的指导意义。
A detailed histopathological study was made of the lungs of 36 cases of plexogenetic pulmonary arteriopathy coming to combined heart-lung transplantation. It revealed two dissimilar processes involved in the pathogenesis of this disease. One comprised histological appearances consistent with constriction of muscular pulmonary arteries, a condition that would be likely to be reversed by pulmonary vasodilators. The other was the proliferation of myofibroblasts in the intima and lumen of pulmonary arteries, a disorder of growth unlikely to be influenced by this type of therapy. In previous, ultrastructural studies we have shown that the source of these cells of muscular pedigree is muscle cells from the inner half of the media which migrate into the intima through gaps in the inner elastic lamina. In the present study we found a similar proliferation proliferation of myofibroblasts in the intima, not only of pulmonary arteries but also of pulmonary veins, in plexogenic pulmonary arteriopathy. Arterial thrombi found were considered to be a complication rather than a cause of plexogenic pulmonary arteriopathy. Siderophages, cholesterol granulomas and focal fibrosis in the lung were considered to be a consequence of intrapulmonary haemorrhage early in the course of the disease. It is concluded that, while plexogenic pulmonary arteriopathy has an important vasoconstrictive element, it is also based on a disorder of growth of cells of muscular peidgree. This view has clear implications for therapy of primary plexogenic pulmonary arteriopathy.