• Ther Adv Respir Dis · Jun 2009

    Case Reports

    Sarcoidosis-associated pulmonary hypertension: a role for endothelin receptor antagonists?

    • Georgia G Pitsiou, Dionysis Spyratos, Ioannis Kioumis, Afroditi K Boutou, Chrysanthi Nakou, and Ioannis Stanopoulos.
    • Respiratory Failure Unit, 'G. Papanikolaou' Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece. gpitsiou@yahoo.gr
    • Ther Adv Respir Dis. 2009 Jun 1; 3 (3): 99-101.

    AbstractData on the treatment of sarcoidosis-associated pulmonary hypertension are scarce, while the variety of underlying pathophysiologic mechanisms are a major limitation in the implementation of a universal therapy. We report a 47-year-old male patient who presented with stage II sarcoidosis and associated severe pulmonary hypertension. Corticosteroid treatment resolved parenchymal lesions of the lung while vascular involvement did not respond, with the patient remaining in poor functional status. Addition of bosentan, a dual endothelin receptor antagonist, resulted in marked improvement in functional class and exercise capacity of the patient, allowing gradual tapering of steroids.

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