Heath D, Reid R
Br J Dis Chest. 1985 Jul;79(3):284-94.
Invasive pulmonary haemangiomatosis is a recently described disease in which exceedingly thin-walled vessels of capillary or venous dimensions infiltrate the lung parenchyma and pulmonary blood vessels. The angiomatous vessels, of obscure origin, infiltrate the media and intima of muscular pulmonary arteries, pulmonary veins and venules. The occlusion of the veins and venules by the thin-walled vessels, and the reactive intimal fibrosis they provoke, leads to pulmonary capillary dilatation, collections of intra-alveolar siderophages, fibrosis of alveolar walls and osseous nodules. This secondary pulmonary veno-occlusive disease in turn leads to hypertensive pulmonary vascular disease. Hence invasive pulmonary haemangiomatosis represents a fourth cause of 'unexplained pulmonary hypertension', the other three being unexplained plexogenic pulmonary arteriopathy, recurrent pulmonary thromboembolism, and pulmonary veno-occlusive disease. Two previously reported cases of invasive pulmonary haemangiomatosis presented with recurrent haemoptysis and the gradual development of chronic respiratory insufficiency associated with diffuse infiltrates in the chest radiograph. In one of these cases a haemothorax had developed. Such clinical features may be of importance in coming to the correct diagnosis.
侵袭性肺血管瘤病是一种最近才被描述的疾病，其中具有毛细血管或静脉管径的极薄壁血管浸润肺实质和肺血管。这些来源不明的血管瘤样血管浸润肌性肺动脉、肺静脉和小静脉的中膜和内膜。薄壁血管对静脉和小静脉的阻塞以及由此引发的反应性内膜纤维化，导致肺毛细血管扩张、肺泡内含铁血黄素巨噬细胞聚集、肺泡壁纤维化和骨结节形成。这种继发性肺静脉闭塞性疾病继而导致高血压性肺血管疾病。因此，侵袭性肺血管瘤病是“不明原因肺动脉高压”的第四个病因，其他三个病因是不明原因的丛状肺血管病、复发性肺血栓栓塞和肺静脉闭塞性疾病。先前报道的两例侵袭性肺血管瘤病患者表现为反复咯血以及慢性呼吸功能不全的逐渐发展，并伴有胸部X线片上的弥漫性浸润。在其中一例中还出现了血胸。这些临床特征对于正确诊断可能具有重要意义。
