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Related Experiment Videos

Pulmonary functions in children with progressive systemic sclerosis.

B Z Garty1, B H Athreya, R Wilmott

  • 1Department of Pediatrics, University of Pennsylvania, Philadelphia.

Pediatrics
|December 1, 1991
PubMed
Summary

Pediatric progressive systemic sclerosis frequently causes early lung disease. While lung involvement is common, its progression is often slow, highlighting the importance of assessing pulmonary disease severity in children.

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Area of Science:

  • Pediatric Rheumatology
  • Pulmonology
  • Systemic Autoimmune Diseases

Background:

  • Progressive systemic sclerosis (PSS) is a rare autoimmune disease affecting connective tissues.
  • Pulmonary complications are a leading cause of mortality in adult PSS.
  • Data on pulmonary involvement in pediatric PSS is limited.

Purpose of the Study:

  • To investigate the patterns and progression of pulmonary involvement in children with PSS.
  • To determine the frequency and types of lung disease in pediatric PSS.
  • To assess the long-term course and prognostic factors of pulmonary disease in this population.

Main Methods:

  • Retrospective analysis of 13 pediatric patients with PSS.
  • Evaluation of respiratory symptoms, chest roentgenograms, and pulmonary function tests (PFTs).

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  • Longitudinal follow-up of PFTs over a mean of 6.2 years.

    • Main Results:

    • High frequency of pulmonary involvement (92% abnormal PFTs, 61% respiratory symptoms).
    • Early detection of lung disease, often within the first year of illness.
    • Generally indolent progression of lung disease, with significant changes in only 2 patients.
    • Pulmonary hypertension and severe restrictive lung disease were causes of mortality.

    Conclusions:

    • Pediatric PSS frequently presents with significant pulmonary disease.
    • Lung involvement occurs early and may progress slowly.
    • The severity of pulmonary disease is a critical prognostic indicator in pediatric PSS, similar to adults.