[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. Bronchoalveolar 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.
Xu
Ling
L
Department of Respiratory Medicine, PUMC Hospital, CAMS and PUMC, Beijing 100730, China. qlingxu@yahoo.com.cn
Cai
Bai-qiang
BQ
Liu
Hong-rui
HR
Zhu
Yuan-jue
YJ
D000328:Adult; D001706:Biopsy; D001980:Bronchi / Q000473:pathology*; D003937:Diagnosis, Differential; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D011658:Pulmonary Fibrosis / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed / Q000379:methods