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Small airways in progressive systemic sclerosis (PSS).

R D Bjerke, D P Tashkin, P J Clements

    The American Journal of Medicine
    |February 1, 1979
    PubMed
    Summary
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    Progressive systemic sclerosis (PSS) rarely causes peripheral airway obstruction. When observed, it is likely due to smoking, not the disease itself, as pulmonary function tests showed normal results in PSS patients who did not smoke.

    Area of Science:

    • Pulmonary Medicine
    • Rheumatology
    • Cardiovascular System

    Background:

    • Obstructive disease of peripheral airways is associated with interstitial lung diseases like sarcoidosis.
    • Necropsy findings in progressive systemic sclerosis (PSS) suggest potential small airway obstruction due to bronchiolectasis and peribronchial fibrosis.
    • Interstitial pulmonary involvement is common in PSS.

    Purpose of the Study:

    • To investigate the presence and functional evidence of peripheral airway obstruction in patients with PSS.
    • To differentiate between PSS-related lung changes and smoking-induced airway effects.

    Main Methods:

    • Pulmonary function studies were conducted on 39 subjects with PSS (22 nonsmokers, 17 smokers).
    • Evaluated parameters included FEV1:FVC ratio, maximal mid-expiratory flow rate, closing volume, closing capacity, volume of isoflow, delta Vmax50, dynamic to static lung compliance ratio, and upstream airway conductance.

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  • Subjects with PSS often exhibited evidence of interstitial lung disease, such as increased static recoil pressure and reduced diffusing capacity.

    • Main Results:

    • The forced expiratory volume in 1 second to forced vital capacity (FEV1:FVC) ratio was normal in all PSS subjects.
    • Nonsmokers with PSS showed mostly normal peripheral airway function tests.
    • Smokers with PSS frequently presented abnormal results in tests assessing peripheral airway function, despite similar restrictive lung disease severity compared to nonsmokers.

    Conclusions:

    • Diffuse interstitial lung disease in PSS typically does not cause functional peripheral airway obstruction.
    • Abnormalities in peripheral airway function in PSS patients are more likely attributable to the effects of cigarette smoking.
    • This study highlights the importance of considering smoking as a confounding factor in assessing lung disease in PSS.