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Related Experiment Videos

Longitudinal changes in lung function and respiratory symptoms in progressive systemic sclerosis. Prospective study.

G I Greenwald, D P Tashkin, H Gong

    The American Journal of Medicine
    |July 1, 1987
    PubMed
    Summary

    Progressive systemic sclerosis (PSS) lung disease progresses slowly overall, but with significant individual variation. Lung function tests reveal gradual declines, particularly in diffusing capacity and static lung compliance over time.

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    Area of Science:

    • Pulmonary Medicine
    • Rheumatology
    • Systemic Sclerosis Research

    Background:

    • Lung involvement is common in progressive systemic sclerosis (PSS).
    • The long-term progression of lung disease in PSS is not well understood.
    • Understanding PSS lung disease progression is crucial for patient management.

    Purpose of the Study:

    • To prospectively evaluate the natural history of lung function over time in patients with PSS.
    • To identify factors influencing the rate of lung function decline in PSS.
    • To assess changes in pulmonary function test abnormalities and respiratory symptoms.

    Main Methods:

    • Prospective, serial pulmonary function testing in 61 PSS patients from 1973-1982.
    • Assessment of functional indexes including vital capacity, total lung capacity, and diffusing capacity.

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  • Analysis of demographic, clinical, and treatment-related factors affecting lung function changes.

    • Main Results:

    • Significant annual rates of change observed in restriction and diffusion indexes.
    • Male subjects showed trends toward faster lung function decline compared to females.
    • Nonsmokers exhibited faster declines in total lung capacity and static lung compliance than smokers.
    • Longer disease duration at study entry correlated with slower lung volume decrease.
    • Increased frequency of abnormal diffusing capacity and static lung compliance over the study period.

    Conclusions:

    • PSS-related lung disease exhibits an overall indolent progression.
    • Substantial individual variability exists in the rate of lung function decline.
    • Pulmonary function abnormalities, especially in diffusing capacity, become more frequent over time.
    • Further research is needed to elucidate specific PSS lung disease trajectories.