Loyd J E, Atkinson J B, Pietra G G, Virmani R, Newman J H
Department of Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee 37232.
Am Rev Respir Dis. 1988 Oct;138(4):952-7. doi: 10.1164/ajrccm/138.4.952.
We analyzed lung specimens from 23 affected members of 13 families with known familial primary pulmonary hypertension. In this heritable disease, the pathogenetic basis of disease should be distinct within families, and the pathologic lesions should reflect the underlying biologic mechanisms. Two pathologic types of primary pulmonary hypertension have been proposed to exist, "plexogenic" and "thromboembolic." The purpose of this study was to determine the nature and variety of pathologic lesions in families with familial primary pulmonary hypertension and to discover whether the pattern of pathologic lesions found supported the existence of two types of disease. We found marked heterogeneity of pathologic lesions within and among families, including frequent coexistence of thrombotic and plexiform lesions. We conclude that the lesions found in primary pulmonary hypertension are not specific but represent different manifestations of the same pathologic process. Thus, plexiform and thrombotic lesions of the pulmonary arterioles may not arise from distinctly different biologic processes but may simply be different manifestations of the same process. Further insights in primary pulmonary hypertension must come from study of the biologic aspects of the intimal lesion.
我们分析了来自13个已知家族性原发性肺动脉高压家族的23名患病成员的肺标本。在这种遗传性疾病中，疾病的发病机制在家族内部应该是不同的，并且病理病变应该反映潜在的生物学机制。原发性肺动脉高压已被提出存在两种病理类型，即“丛状型”和“血栓栓塞型”。本研究的目的是确定家族性原发性肺动脉高压家族中病理病变的性质和种类，并发现所发现的病理病变模式是否支持两种疾病类型的存在。我们发现家族内部和家族之间病理病变存在明显的异质性，包括血栓形成和丛状病变的频繁共存。我们得出结论，原发性肺动脉高压中发现的病变并非特异性的，而是代表同一病理过程的不同表现。因此，肺小动脉的丛状和血栓形成病变可能并非源于明显不同的生物学过程，而可能仅仅是同一过程的不同表现。对原发性肺动脉高压的进一步认识必须来自对内膜病变生物学方面的研究。
