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Pulmonary function in relapsing polychondritis.

W S Krell, B A Staats, R E Hyatt

    The American Review of Respiratory Disease
    |June 1, 1986
    PubMed
    Summary
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    Relapsing polychondritis can cause airway obstruction, primarily due to airway abnormalities, not lung elasticity loss. Spirometry is crucial for diagnosing functional respiratory issues in affected patients.

    Area of Science:

    • Pulmonology
    • Internal Medicine
    • Rheumatology

    Background:

    • Relapsing polychondritis (RP) is a rare systemic autoimmune disease affecting cartilage.
    • Respiratory tract involvement in RP can lead to significant morbidity and mortality.
    • Understanding the mechanisms of airway obstruction in RP is critical for patient management.

    Purpose of the Study:

    • To investigate pulmonary mechanics and bronchoscopic findings in patients with relapsing polychondritis.
    • To elucidate the predominant mechanism of airway obstruction in relapsing polychondritis.
    • To assess the correlation between clinical presentation, bronchoscopic findings, and pulmonary function tests.

    Main Methods:

    • Evaluated 5 patients with relapsing polychondritis.

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  • Conducted pulmonary function tests, including maximal flow-static recoil curves.
  • Performed bronchoscopy to assess airway morphology.
  • Correlated findings with clinical symptoms and radiographic data.

    • Main Results:

    • Three of five patients presented with dyspnea and showed both expiratory and inspiratory obstruction.
    • Expiratory obstruction was primarily attributed to airway abnormalities, not reduced lung elastic recoil.
    • Bronchoscopic findings of the extrathoracic airway did not consistently correlate with pulmonary function, while intrathoracic findings correlated with expiratory flow rates.

    Conclusions:

    • Airway abnormality is the main cause of expiratory obstruction in relapsing polychondritis.
    • Spirometry is more valuable than bronchoscopic or radiographic findings for determining functional respiratory abnormalities.
    • Early detection and management of airway obstruction are vital for patients with relapsing polychondritis.