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本病是指弥漫性肺间质纤维化,因其确切病因尚不清楚,故又称特发性或隐原性肺间质纤维化,也有人称之为弥漫性纤维肺泡炎。现已认为这是一种比较常见的肺部疾病,虽有急性发病者,但绝大多数为慢性过程。病因迄今未明,可能与自身免疫反应,遗传因素、过敏等因素有关。主要病理特点为肺泡炎演变为肺间质纤维化。肺功能检查示限制性通气功能障碍、弥散功能障碍和肺顺应性下降。表现为肺容置减低,CO弥散量显著减低,但最大通气量与一秒钟用力呼气置的相对值基本正常。
The disease refers to diffuse pulmonary fibrosis, because of its exact etiology is not clear, it is also known as idiopathic or cryptogenic pulmonary interstitial fibrosis, also known as diffuse fibrosis alveolitis. Now that this is a more common lung disease, although acute onset, but the vast majority of chronic processes. Etiology so far unknown, may be related to autoimmune reactions, genetic factors, allergies and other factors. The main pathological features of alveolar inflammation of the lung into interstitial fibrosis. Pulmonary function tests showed restrictive ventilatory dysfunction, diffuse dysfunction and decreased lung compliance. Manifested as reduced lung capacity, CO dispersion significantly reduced, but the maximum ventilation and forced expiratory pressure of one second set the relative normal.