[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.