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Updated: Jul 19, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Spontaneous pneumothorax in scleroderma.
Jiun Yoon1, David R Finger, Joseph S Pina
1From the *Department of Medicine, †Rheumatology Service, and ‡Pulmonary Service, Tripler Army Medical Center, Honolulu HI.
Summary
Scleroderma patients with advanced lung fibrosis and cysts may develop spontaneous pneumothorax. Treatment involves chest tubes, but recurrence is common, necessitating procedures like talc pleurodesis for long-term management.
Area of Science:
- Pulmonology
- Rheumatology
- Case Study
Background:
- Scleroderma is a complex autoimmune disease primarily affecting connective tissues.
- Pulmonary complications, including fibrosis, are significant causes of morbidity and mortality in scleroderma.
- Spontaneous pneumothorax is a rare but serious pulmonary manifestation in scleroderma patients.
Purpose of the Study:
- To report a case of spontaneous pneumothorax in a patient with advanced scleroderma.
- To review previously reported cases of spontaneous pneumothorax in scleroderma.
- To discuss the management and outcomes of spontaneous pneumothorax in this patient population.
Main Methods:
- Case presentation of a patient with advanced scleroderma and spontaneous pneumothorax.
- Literature review of similar cases in scleroderma patients.
- Description of treatment interventions including chest tube thoracostomy and talc pleurodesis.
Main Results:
- The patient experienced recurrent spontaneous pneumothorax secondary to cyst rupture in advanced pulmonary fibrosis.
- Initial chest tube thoracostomy was followed by recurrence.
- Talc pleurodesis resulted in no recurrence over 18 months of follow-up.
Conclusions:
- Spontaneous pneumothorax is a rare complication of advanced pulmonary fibrosis in scleroderma, linked to cyst rupture.
- Management requires prompt intervention, with a high recurrence rate after chest tube insertion.
- Pleurodesis or surgical options may be necessary for definitive treatment and prevention of recurrence in scleroderma patients.
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