Langleben D, Heneghan J M, Batten A P, Wang N S, Fitch N, Schlesinger R D, Guerraty A, Rouleau J L
Veterans Administration Medical Center, St. Louis, Missouri.
Ann Intern Med. 1988 Jul 15;109(2):106-9. doi: 10.7326/0003-4819-109-2-106.
We describe the first cases of familial pulmonary capillary hemangiomatosis, a disorder in which capillaries in the lungs proliferate. Three siblings died from primary pulmonary hypertension. One developed pulmonary congestion preterminally after vasodilator treatment. The inheritance pattern seems autosomal recessive. Lung specimens obtained in two siblings showed extensive pulmonary capillary hemangiomatosis, with normal capillaries proliferating into veins and alveoli. Including our patients, four of the nine patients with pulmonary capillary hemangiomatosis have presented with the clinical picture of primary pulmonary hypertension. Thus, pulmonary capillary hemangiomatosis should be considered as a histologic pattern of primary pulmonary hypertension. Most other cases of pulmonary capillary hemangiomatosis have been similar to pulmonary veno-occlusive disease. Recently, disorders involving the proliferation of cytologically normal capillaries have been termed angiogenic diseases. Pulmonary capillary hemangiomatosis may be an angiogenic disease.
我们描述了首例家族性肺毛细血管瘤病，这是一种肺部毛细血管增生的疾病。三名兄弟姐妹死于原发性肺动脉高压。其中一人在接受血管扩张剂治疗后临终前出现肺充血。遗传模式似乎为常染色体隐性遗传。在两名兄弟姐妹身上获取的肺标本显示有广泛的肺毛细血管瘤病，正常毛细血管增生进入静脉和肺泡。包括我们的患者在内，9例肺毛细血管瘤病患者中有4例表现出原发性肺动脉高压的临床症状。因此，肺毛细血管瘤病应被视为原发性肺动脉高压的一种组织学类型。大多数其他肺毛细血管瘤病病例与肺静脉闭塞性疾病相似。最近，涉及细胞学正常的毛细血管增生的疾病被称为血管生成性疾病。肺毛细血管瘤病可能是一种血管生成性疾病。
