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Pulmonary vascular changes in scleroderma.

R H Young, G J Mark

    The American Journal of Medicine
    |June 1, 1978
    PubMed
    Summary
    This summary is machine-generated.

    Pulmonary vascular changes, including intimal and medial hyperplasia, are significant in scleroderma patients. Some cases present as malignant pulmonary hypertension, a distinct clinicopathologic entity.

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    Area of Science:

    • Cardiovascular Pathology
    • Pulmonary Hypertension
    • Scleroderma Research

    Background:

    • Scleroderma (systemic sclerosis) is a complex autoimmune disease.
    • Pulmonary vascular complications are a known, yet not fully understood, aspect of scleroderma.
    • Assessing the incidence and pathological significance of pulmonary vascular changes is crucial.

    Observation:

    • A necropsy study analyzed 30 scleroderma patient reports.
    • Fourteen patients exhibited moderate to marked pulmonary arterial abnormalities.
    • Nine of these patients presented with predominant respiratory symptoms.

    Findings:

    • Pathological analysis revealed intimal and medial hyperplasia in pulmonary arteries of all sizes.
    • Severe arterial changes were observed in eight patients, five with minimal interstitial fibrosis.

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  • Three patients with severe changes experienced rapidly progressive respiratory failure and fatal pulmonary hypertension.

    • Implications:

    • These findings identify a distinct clinicopathologic entity: malignant pulmonary hypertension in scleroderma.
    • This entity is comparable to malignant renal hypertension in scleroderma.
    • Understanding these vascular changes is vital for diagnosing and managing scleroderma-related cardiopulmonary disease.