Lippert J L, White C S, Cameron E W, Sun C C, Liang X, Rubin L J
Department of Radiology, University of Maryland Medical System, Baltimore 21201, USA.
J Thorac Imaging. 1998 Jan;13(1):49-51. doi: 10.1097/00005382-199801000-00012.
Pulmonary capillary hemangiomatosis is a rare disorder that is characterized pathologically by formation of capillaries along alveolar walls that may ultimately invade the pulmonary parenchyma and pulmonary arteries. The clinic presentation overlaps that of primary pulmonary hypertension, but the natural history of pulmonary capillary hemangiomatosis is one of rapid deterioration. The typical radiographic appearance is a diffuse bilateral reticulonodular pattern associated with enlarged central pulmonary arteries.
肺毛细血管瘤病是一种罕见的疾病，其病理特征是沿肺泡壁形成毛细血管，这些毛细血管最终可能侵犯肺实质和肺动脉。临床表现与原发性肺动脉高压重叠，但肺毛细血管瘤病的自然病程是迅速恶化。典型的影像学表现是与中央肺动脉增粗相关的双侧弥漫性网状结节状影。
