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Pulmonary hypertension in polymyositis

T W Bunch, R G Tancredi, J T Lie

    Chest
    |January 1, 1981
    PubMed
    Summary

    Pulmonary hypertension is a rare but fatal complication of polymyositis. Autopsy confirmed severe plexogenic pulmonary vascular disease in a patient with a history of polymyositis and cardiac failure.

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    Area of Science:

    • Cardiology
    • Rheumatology
    • Pathology

    Background:

    • Polymyositis is an idiopathic inflammatory myopathy.
    • Pulmonary hypertension (PH) is a known, albeit uncommon, complication of polymyositis.
    • Cardiac failure can coexist with polymyositis.

    Observation:

    • A 69-year-old woman with a 20-month history of polymyositis and cardiac failure presented with severe pulmonary hypertension.
    • The patient's severe pulmonary hypertension was noted terminally upon clinic admission.
    • She expired within 24 hours of admission.

    Findings:

    • Autopsy revealed severe plexogenic pulmonary vascular disease.
    • This indicates advanced vascular pathology in the pulmonary arteries.
    • The findings correlate pulmonary hypertension with polymyositis and cardiac failure.

    Implications:

    • This case highlights the lethal potential of pulmonary hypertension in polymyositis.
    • Early recognition and management of PH in polymyositis patients may be crucial.
    • Further research into the pathogenesis of PH in inflammatory myopathies is warranted.

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