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Updated: Jun 30, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Published on: June 16, 2020

Centrilobular fibrosis: an underrecognized pattern in systemic sclerosis.

Romy B C de Souza1, Claudia T L Borges, Vera L Capelozzi

  • 1Division of Rheumatology, University of São Paulo, São Paulo, Brazil. romy.c@uol.com.br

Respiration; International Review of Thoracic Diseases
|September 19, 2008
PubMed
Summary

Centrilobular fibrosis (CLF) is a distinct pattern in scleroderma lung disease, often linked to gastroesophageal reflux. Intensive antireflux treatment stabilized lung function in patients with isolated CLF.

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Area of Science:

  • Pulmonary Medicine
  • Rheumatology
  • Gastroenterology

Background:

  • Gastroesophageal reflux is associated with lung involvement in scleroderma.
  • This study investigates the relationship between lung disease and reflux in systemic sclerosis (SSc).

Purpose of the Study:

  • To identify clinical, radiological, and histopathological features of SSc patients with and without centrilobular fibrosis (CLF).

Main Methods:

  • Open lung biopsy in 28 SSc patients with lung involvement to classify CLF.
  • High-resolution computed tomography (HRCT), pulmonary function tests, and esophageal analysis were performed.
  • Treatment included cyclophosphamide for nonspecific interstitial pneumonia and intensified antireflux therapy for isolated CLF.

Main Results:

  • Isolated CLF was found in 21% of biopsies, often associated with nonspecific interstitial pneumonia (84%).
  • Histopathology showed bronchocentric distribution and intraluminal matter; foreign bodies were noted in one-third.
  • HRCT revealed central, patchy lung involvement with ground glass and consolidation patterns.
  • Patients with isolated CLF presented with dyspnea, esophageal abnormalities, and moderate lung impairment, which remained stable after antireflux treatment.

Conclusions:

  • A novel CLF pattern in SSc lung disease has been described.
  • This pattern exhibits unique histological, tomographic, and clinical characteristics.
  • These findings may lead to improved therapeutic strategies for SSc-related lung disease.