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Updated: Aug 19, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Diagnosis and differential diagnosis of airway-centered interstitial fibrosis]
Ling Xu1, Bai-qiang Cai, Hong-rui Liu
1Department of Respiratory Medicine, PUMC Hospital, CAMS and PUMC, Beijing 100730, China. qlingxu@yahoo.com.cn
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.
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