Yacoub M H, Thompson V C
Thorax. 1971 Jul;26(4):365-75. doi: 10.1136/thx.26.4.365.
Idiopathic pulmonary hilar fibrosis is a condition related to mediastinal fibrosis, characterized by localization of the fibrosing process to one or both pulmonary hila. This results in pulmonary hypertension and bronchial narrowing. Three patients suffering from this disease, in whom the diagnosis has been confirmed by thoracotomy, are reported. The clinical and pathological features are described and previously reported cases are reviewed. The syndrome is classified into two types, according to whether the obstruction affects mainly the pulmonary artery or veins. The disease is a self-limiting one but may lead to organic changes in the lungs causing severe disability.
特发性肺门纤维化是一种与纵隔纤维化相关的疾病，其特征是纤维化过程局限于一侧或双侧肺门。这会导致肺动脉高压和支气管狭窄。本文报告了3例经开胸手术确诊为此病的患者。描述了其临床和病理特征，并对既往报道的病例进行了回顾。根据梗阻主要影响肺动脉还是静脉，该综合征分为两型。本病为自限性疾病，但可能导致肺部器质性改变，引起严重残疾。
