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Updated: Jun 28, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Diffuse thickening of bronchial walls with multiple nodular mucosal protrusions]
1Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
Sjögren's syndrome can cause rare large-airway disease, including tracheal and bronchial nodules. This case highlights central airway involvement and successful treatment with glucocorticoids.
Area of Science:
- Rheumatology
- Pulmonology
- Otolaryngology
Background:
- Sjögren's syndrome is a chronic autoimmune disease primarily affecting exocrine glands.
- Airway involvement in Sjögren's syndrome typically manifests as peripheral airway disease.
- Central airway involvement, particularly with nodular lesions, is uncommon in Sjögren's syndrome.
Observation:
- A 58-year-old woman presented with chronic respiratory symptoms and xerostomia.
- Physical examination revealed enlarged salivary glands and lymph nodes, with lung rales.
- Sialography and biopsy confirmed Sjögren's syndrome with lymphocytic infiltration.
Findings:
- Chest CT and bronchoscopy identified diffuse thickening and macroscopic nodules in the trachea and main bronchi.
- Endobronchial biopsy confirmed lymphocytic infiltration of the bronchial submucosa.
- The patient responded dramatically to glucocorticoid therapy, with regression of airway lesions and systemic symptoms.
Implications:
- This case demonstrates a rare presentation of Sjögren's syndrome involving central large-airway disease with nodular formation.
- Early diagnosis and treatment with glucocorticoids can lead to significant improvement in airway manifestations.
- Highlights the importance of considering Sjögren's syndrome in patients with unexplained airway nodules and autoimmune symptoms.
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