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Diaphragm dysfunction in mixed connective tissue disease. A case report

J Martens, M Demedts

    Scandinavian Journal of Rheumatology
    |January 1, 1982
    PubMed
    Summary

    Mixed connective tissue disease (MCTD) can cause significant diaphragm dysfunction, leading to respiratory muscle weakness. This dysfunction results in restrictive lung defects and altered lung mechanics.

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    Area of Science:

    • Pulmonology
    • Rheumatology
    • Internal Medicine

    Background:

    • Mixed connective tissue disease (MCTD) is a rare autoimmune disorder with overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis.
    • Respiratory complications are known in MCTD, but specific diaphragm involvement is less frequently detailed.

    Observation:

    • A patient diagnosed with MCTD exhibited pronounced symptoms of respiratory muscle weakness.
    • Clinical examination revealed marked diaphragm dysfunction.

    Findings:

    • The patient demonstrated reduced maximal respiratory pressures (transrespiratory, transdiaphragmatic, transpulmonary) at various lung volumes (TLC, FRC, RV).
    • Diaphragm insufficiency was identified as the cause of a restrictive ventilatory defect.
    • A decrease in static lung compliance was observed, alongside a high diffusing capacity per unit lung volume.

    Implications:

    • This case highlights the potential for severe diaphragm dysfunction in MCTD patients.
    • Understanding diaphragm involvement is crucial for managing respiratory complications in MCTD.
    • Further research into the mechanisms and prevalence of respiratory muscle weakness in MCTD is warranted.

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