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Related Experiment Videos

Lung involvement in scleroderma.

G König, C Luderschmidt, C Hammer

    Chest
    |March 1, 1984
    PubMed
    Summary
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    Progressive systemic scleroderma (PSS) patients, particularly types 2 and 3 with inflammation, face a higher risk of severe lung involvement. Bronchoalveolar lavage findings can predict disease severity and progression.

    Area of Science:

    • Rheumatology
    • Pulmonology
    • Immunology

    Background:

    • Scleroderma, a connective tissue disease, can affect the lungs, leading to significant morbidity.
    • Lung involvement (LI) in scleroderma patients varies by disease subtype and systemic inflammation.
    • Assessing lung function (LF) and inflammatory markers is crucial for understanding LI progression.

    Purpose of the Study:

    • To investigate the prevalence and severity of lung involvement in scleroderma patients.
    • To correlate lung function with scleroderma subtypes and systemic inflammation.
    • To evaluate the prognostic value of bronchoalveolar lavage (BAL) findings in LI.

    Main Methods:

    • Studied lung function in 101 scleroderma patients, categorizing them by scleroderma type and systemic inflammation (form A vs. form B).

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  • Analyzed bronchoalveolar lavage (BAL) for differential cell counts and collagenase activity.
  • Correlated BAL findings with lung function impairment and disease severity.

    • Main Results:

    • Impaired lung function was observed in 18% of morphea, 32% of type 1, 56% of type 2, and 75% of type 3 scleroderma.
    • Progressive systemic scleroderma (PSS) form A patients showed more frequent and severe LI than form B patients.
    • Elevated lymphocytes/neutrophils and collagenase activity in BAL were associated with form A and severe LI, with worsening LF in those with inflammatory BAL patterns.

    Conclusions:

    • Scleroderma patients with PSS types 2 or 3, especially form A, have a higher risk of severe LI.
    • Differential cell counts and collagenase activity in BAL provide prognostic information for active scleroderma-related lung disease.
    • BAL analysis can help predict disease progression and guide therapeutic strategies.