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[Respiratory function profile in scleroderma. 20 cases (author's transl)]

J P Kleisbauer, J M Feliciano, J Colonna

    Le Poumon Et Le Coeur
    |January 1, 1980
    PubMed
    Summary
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    Scleroderma patients often show normal chest X-rays but may have ventilatory disturbances. Pulmonary vascular lesions are common, even with normal carbon monoxide transfer (TLCO) in many cases.

    Area of Science:

    • Pulmonary Medicine
    • Rheumatology
    • Cardiovascular Research

    Background:

    • Scleroderma is a complex autoimmune disease affecting connective tissues.
    • Pulmonary involvement is a significant complication of scleroderma, impacting morbidity and mortality.
    • Understanding the spectrum of respiratory dysfunction is crucial for patient management.

    Purpose of the Study:

    • To investigate the prevalence and patterns of respiratory function abnormalities in patients with scleroderma.
    • To assess the correlation between different scleroderma subtypes and respiratory impairment.
    • To highlight the significance of pulmonary vascular lesions in scleroderma patients.

    Main Methods:

    • Recruitment of 20 scleroderma patients (13 female, 7 male; mean age 49).

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  • Classification of patients based on scleroderma subtype: localized, generalized without Raynaud's, with Raynaud's, and acrosclerosis.
  • Comprehensive respiratory function tests including spirometry, lung volumes, diffusing capacity for carbon monoxide (TLCO), and arterial blood gases.
  • Analysis of chest X-ray findings.

    • Main Results:

    • 38% of patients had normal chest X-rays.
    • 58% exhibited ventilatory disturbances, predominantly restrictive syndrome.
    • A decrease in TLCO was observed in only 25% of cases.
    • Pulmonary vascular lesions were noted to be extensive in these patients.

    Conclusions:

    • Despite potentially normal chest X-rays and TLCO, significant ventilatory disturbances are common in scleroderma.
    • Pulmonary vascular lesions represent a critical and often underestimated aspect of scleroderma-related respiratory disease.
    • Further research into early detection and management of pulmonary vascular complications is warranted.