Ito Koji, Ichiki Toshihiro, Ohi Keiji, Egashira Kensuke, Ohta Mituhiko, Taguchi Kenichi, Takeshita Akira
Department of Cardiovascular Medicine, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan.
Circ J. 2003 Sep;67(9):793-5. doi: 10.1253/circj.67.793.
Pulmonary capillary hemangiomatosis (PCH) is a rare idiopathic lung disorder that occurs in young patients and leads to pulmonary hypertension (PH). It is difficult to diagnosis in the early stage and is often mistaken for primary PH; in almost all cases of PCH, the correct diagnosis is not made until autopsy. In the present case of PCH, the patient had severe pulmonary hypertension and died of respiratory failure. Pathologically, PCH is characterized by proliferation of benign thin-walled capillary sized blood vessels in the lung parenchyma.
肺毛细血管瘤病（PCH）是一种罕见的特发性肺部疾病，发生于年轻患者，可导致肺动脉高压（PH）。该病早期难以诊断，常被误诊为原发性PH；几乎在所有PCH病例中，直至尸检才做出正确诊断。在本PCH病例中，患者患有严重的肺动脉高压并死于呼吸衰竭。病理上，PCH的特征是肺实质内良性薄壁毛细血管大小的血管增生。
