气道中心性间质纤维化的诊断及鉴别诊断

目的介绍一种新近发现的特发性间质性肺炎--气道中心性间质纤维化及其鉴别诊断.方法分析北京协和医院确诊的1例以及文献报道的12例气道中心性间质纤维化患者的临床表现、肺功能、支气管肺泡灌洗液、影像学及病理学特点.结果气道中心性间质纤维化患者主要表现为慢性咳嗽和缓慢进展的呼吸困难,肺功能表现为限制性通气功能障碍,支气管肺泡灌洗液多有淋巴细胞增加,影像学表现为弥漫性网状结节影,伴气道壁增厚及周围纤维化,病理表现为明显的以膜性和呼吸性细支气管为中心的间质纤维化.结论气道中心性间质纤维化不同于其他间质性肺病,但其是否为一个独特的疾病有待进一步明确.

Diagnosis and differential diagnosis of airway-centered interstitial fibrosis

Objective To describe a form of interstitial lung disease pathologically characterized by small airway-centered interstitial fibrosis(ACIF). Methods We analyzed the clinical, pulmonary functional, radiographic, and histologic characteristics of one ACIF case in Peking Union Medical College Hospital and reviewed 12 cases in literatures. Results Clinically, patients presented with chronic cough and progressive dyspnea. Pulmonary function tests showed restrictive ventilatory pattern. Broncho-alveolar lavage showed a mild increase in lymphocytes in most cases. Chest radiography revealed diffuse reticulonodular infiltrates, with thickening of the bronchial walls and surrounding fibrosis. The key finding in histopathology was a distinctive pattern of ACIF centered on membranous and respiratory bronchioles. Conclusions ACIF is a disease that do not fit into any known category of interstitial lung disease. Whether it is a unique disease remains to be determined.