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Pulmonary involvement in systemic sclerosis (scleroderma).

V D Steen, G R Owens, G J Fino

    Arthritis and Rheumatism
    |July 1, 1985
    PubMed
    Summary
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    Systemic sclerosis frequently causes lung disease, especially restrictive lung disease and reduced carbon monoxide diffusion. Smoking worsens pulmonary function in these patients, highlighting the need to discourage smoking.

    Area of Science:

    • Pulmonary Medicine
    • Rheumatology
    • Internal Medicine

    Background:

    • Systemic sclerosis (SSc) is a multisystem autoimmune disease with significant pulmonary involvement.
    • Pulmonary complications are a leading cause of morbidity and mortality in SSc patients.

    Purpose of the Study:

    • To evaluate pulmonary function abnormalities in a cohort of nonsmoking patients with systemic sclerosis.
    • To compare pulmonary involvement in SSc patients with and without CREST syndrome.
    • To assess the impact of smoking on pulmonary function in SSc.

    Main Methods:

    • Pulmonary function testing (PFT) was performed on 165 nonsmoking SSc patients.
    • Patients were categorized based on SSc subtype (CREST vs. diffuse) and presence of restrictive lung disease.

    Related Experiment Videos

  • PFT results were compared between nonsmoking SSc patients and a historical cohort of 137 smoking SSc patients.
  • Main Results:

    • Restrictive lung disease and reduced diffusing capacity for carbon monoxide (DLCO) were common.
    • Pulmonary involvement severity and frequency were similar in CREST and diffuse SSc.
    • Dyspnea correlated with restrictive disease; rales correlated with fibrosis.
    • Nonsmoking patients with restrictive lung disease had a 5-year survival rate of 58%.
    • Smokers exhibited more frequent and severe obstructive changes and lower DLCO compared to nonsmokers.

    Conclusions:

    • Pulmonary function abnormalities are highly prevalent in systemic sclerosis, including CREST syndrome.
    • Smoking exacerbates pulmonary disease in SSc patients, indicating an additive detrimental effect.
    • Pulmonary rehabilitation and smoking cessation are crucial for managing SSc-related lung disease.