气道中心性间质纤维化

气道中心性问质纤维化是新近报道的目前认为不同于现有已知任何一型的间质性肺疾病.其病因不明,性质不清,女性多见,以慢性咳嗽和缓慢进展的呼吸困难为主要症状,肺功能显示限制性通气功能障碍,支气管肺泡灌洗液有轻到中度的淋巴细胞增加,其病理特点为以膜性及呼吸性细支气管为中心的间质纤维化和细支气管上皮化生,影像学显示病变累及大气道,激素治疗效果差,预后不佳.现就气道中心性间质纤维化作一综述.

Airway-centered interstitial fibrosis

Airway-centered interstitial fibrosis is recently reported and different from any type of interstitial lung disease known at present.The etiology is unknown,the nature is unclear,and it is more common in women.Chronic cough and slowly progressive dyspnea are the main symptoms.Pulmonary function tests show restrictive ventilation dysfunction.Lymphocytes have a mild to moderate increase in bronchoalveolar lavage fluid.The pathological features are membranous and respiratory bronchioles centered interstitial fibrosis and bronchiolar epithelial metaplasia.The imaging shows larger airways are also involved.The therapeutic efficacy of hormone and prognosis are poor.This article reviews on airwaycentered interstitial fibrosis.