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Related Experiment Videos

Interrelationships between pulmonary and extrapulmonary involvement in systemic sclerosis. A longitudinal analysis

D P Tashkin1, P J Clements, R S Wright

  • 1Department of Medicine, UCLA School of Medicine 90024-1690.

Chest
|February 1, 1994
PubMed
Summary

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Pulmonary involvement in systemic sclerosis (SSc) is not strongly linked to other organ damage, but declining lung function, particularly DCO, TLC, and FVC, may predict survival in SSc patients.

Area of Science:

  • Pulmonary Medicine
  • Rheumatology
  • Systemic Sclerosis Research

Background:

  • Systemic sclerosis (SSc) can affect multiple organ systems, including the lungs.
  • Understanding the relationship between pulmonary involvement and other organ systems is crucial for patient management and prognosis.

Purpose of the Study:

  • To evaluate the correlation between pulmonary involvement and other organ system involvement in SSc at baseline.
  • To assess if baseline lung function and its rate of change predict the progression of extrapulmonary disease.
  • To determine if lung function parameters can predict survival in SSc patients.

Main Methods:

  • Analysis of semiquantitative indices of pulmonary and extrapulmonary involvement and pulmonary function tests (PFTs) in 109 SSc patients over 3 years.

Related Experiment Videos

  • Calculation of annualized rates of change in PFTs and extrapulmonary involvement indices.
  • Use of Spearman rank correlations and Cox stepwise regression to assess relationships and predictive values.

    • Main Results:

    • Baseline PFTs correlated with right heart involvement but not other extrapulmonary systems.
    • Baseline PFTs did not predict subsequent lung function decline or worsening of extrapulmonary involvement.
    • Annual decline in lung function correlated with worsening skin and upper gastrointestinal involvement.
    • Cumulative survival was potentially related to the rate of decline in DCO, TLC, and FVC.

    Conclusions:

    • Pulmonary involvement in SSc, except for right heart involvement (cor pulmonale), does not correlate with the extent of extrapulmonary involvement.
    • Pulmonary involvement does not predict subsequent worsening of pulmonary or extrapulmonary disease, except for skin and upper GI tract.
    • A rapid decline in DCO or lung volumes may indicate poor survival in SSc patients.