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Progressive systemic sclerosis subgroups: variable pulmonary features.

V J Taormina, W T Miller, W B Gefter

    AJR. American Journal of Roentgenology
    |August 1, 1981
    PubMed
    Summary
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    Chest X-ray findings in progressive systemic sclerosis (scleroderma) differ by subtype. Overlap syndromes show higher rates of interstitial lung disease compared to classic PSS.

    Area of Science:

    • Rheumatology
    • Pulmonary Medicine
    • Radiology

    Background:

    • Progressive systemic sclerosis (scleroderma) is a complex autoimmune disease.
    • Previous studies often analyzed scleroderma without considering specific subtypes.
    • Chest film manifestations in scleroderma require further investigation across its varied forms.

    Purpose of the Study:

    • To analyze chest film manifestations in subgroups of progressive systemic sclerosis (scleroderma).
    • To compare the incidence and severity of radiographic abnormalities among classic PSS, CRST/CREST syndrome, and PSS-overlap.
    • To identify distinct patterns of interstitial lung disease and pleural effusion in scleroderma subtypes.

    Main Methods:

    • Retrospective analysis of chest films from 73 patients with progressive systemic sclerosis (scleroderma).

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  • Patients were categorized into classic PSS, CRST/CREST syndrome, and PSS-overlap groups.
  • Radiographic abnormalities, including interstitial lung disease and pleural effusion, were assessed.

    • Main Results:

    • Radiographic abnormalities were observed in 18% of classic PSS, 0% of CRST/CREST, and 75% of PSS-overlap patients.
    • Interstitial lung disease in the PSS-overlap group was typically mild and basilar.
    • In contrast, lung disease in the classic PSS group, though less frequent, was more diffuse and severe.

    Conclusions:

    • Chest film manifestations in progressive systemic sclerosis (scleroderma) vary significantly based on patient subgroup.
    • PSS-overlap patients exhibit a substantially higher prevalence of interstitial lung disease on chest X-rays.
    • Subgroup analysis is crucial for understanding the spectrum of pulmonary involvement in scleroderma.