The pathology of pulmonary veno-occlusive disease
The pathology of pulmonary veno-occlusive disease
复制标题
肺静脉闭塞性疾病的病理学
作者：
C. Wagenvoort;N. Wagenvoort
描述了 13 名肺静脉闭塞性疾病患者的肺部组织病理学。患者大多是儿童或年轻人，没有原发性肺动脉高压所见的性别偏好。特征是，小肺静脉和小静脉因内膜纤维化而变窄或闭塞，但较大的静脉也受到影响。这些阻塞性病变几乎肯定是由血栓组织引起的。肺动脉的变化通常继发于肺静脉血流阻塞，但动脉血栓性变化的发生率也很高。常见的肺实质病变包括间质性肺炎和纤维化、铁沉积和细胞增殖，以及支气管粘液腺和支气管上皮粘液细胞活性增加。这些变化可能表明感染（可能是病毒性感染）是某些患者的病因之一。与这种原因相一致的是，在肺静脉闭塞性疾病引起的症状出现之前，通常会观察到传染性发热呼吸道疾病。
The histopathology of the lungs is described in 13 patients with pulmonary veno-occlusive disease. The patients were mostly children or young adults and there was no sex preference as is seen in primary pulmonary hypertension. Characteristically the small pulmonary veins and venules were narrowed or occluded by intimal fibrosis, but larger veins were also affected. These obstructive lesions almost certainly resulted from organization of thrombi. Changes in the pulmonary arteries were generally secondary to obstruction of pulmonary venous flow but there was also a high incidence of thrombotic changes in the arteries. Pulmonary parenchymal lesions commonly found included interstitial pneumonia and fibrosis, siderosis and cellular proliferation, together with increased activity of bronchial mucous glands and bronchial epithelial mucous cells. These changes may have indicated an infection, possibly of viral nature, as an aetiological factor in some of the patients. Consistent with such a cause is the common observation of an infectious febrile respiratory illness preceding the onset of symptoms due to pulmonary veno-occlusive disease.