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[Pulmonary functions in patients with progressive systemic sclerosis]

M Takaya1, Y Ichikawa, H Shimizu

  • 1Department of Internal Medicine, Tokai University School of Medicine, Kanagawa.

Ryumachi. [Rheumatism]
|February 1, 1994
PubMed
Summary

Progressive systemic sclerosis (PSS) often impairs lung function, with most patients showing reduced diffusing capacity (DLCO) and vital capacity (VC). Autoantibodies and comorbidities like Sjögren

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Area of Science:

  • Pulmonary Medicine
  • Rheumatology
  • Immunology

Background:

  • Progressive systemic sclerosis (PSS) is a multisystem autoimmune disease.
  • Pulmonary involvement is a major cause of morbidity and mortality in PSS.
  • Understanding the factors influencing pulmonary function is crucial for patient management.

Purpose of the Study:

  • To investigate the correlations between pulmonary function parameters and clinical symptoms or autoantibody profiles in PSS patients.
  • To identify predictors of pulmonary dysfunction in PSS.
  • To assess the impact of comorbidities on lung function in PSS.

Main Methods:

  • Retrospective analysis of pulmonary function tests (PFC) including percent vital capacity (% VC) and percent diffusing capacity of carbon oxide (% DLCO).

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  • Correlation analysis with clinical data, including esophageal hypomotility and Sjögren's syndrome (SS).
  • Assessment of autoantibody profiles, specifically anti-Scl-70 and anti-RNP antibodies.

    • Main Results:

    • 85% of PSS patients exhibited abnormal pulmonary function.
    • % DLCO was decreased in 76% and % VC in 40% of patients.
    • Lower % DLCO in males; lower PO2 in patients with esophageal hypomotility.
    • PSS with SS showed significantly decreased % VC and PO2.
    • Pulmonary fibrosis occurred in 63% of patients, more frequent with anti-Scl-70 antibody.
    • Annual VC decrease was 62 ml, more prominent in severe fibrosis or with anti-Scl-70.
    • Milder VC decrease observed in patients with anti-RNP antibody.

    Conclusions:

    • Pulmonary dysfunction is highly prevalent in PSS.
    • Esophageal hypomotility, Sjögren's syndrome, and anti-Scl-70 antibody are associated with worse pulmonary function.
    • Anti-RNP antibody may be associated with a milder decline in VC.
    • Pulmonary function monitoring and risk stratification are essential in PSS management.