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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.

Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases
|October 18, 2006
PubMed
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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.

Related Experiment Videos

  • 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.