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

Respiratory Failure Unit, 'G. Papanikolaou' Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece gpitsiou{at}yahoo.gr

Dionysis Spyratos

Pulmonary Clinic, 'G. Papanikolaou' Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece

Ioannis Kioumis

Pulmonary Clinic, 'G. Papanikolaou' Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece

Afroditi K. Boutou

Respiratory Failure Unit, 'G. Papanikolaou' Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece

Chrysanthi Nakou

Respiratory Failure Unit, 'G. Papanikolaou' Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece

Ioannis Stanopoulos

Respiratory Failure Unit, 'G. Papanikolaou' Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece

Data 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.

Key Words: bosentan • pulmonary hypertension • sarcoidosis

This version was published on June 1, 2009

Therapeutic Advances in Respiratory Disease, Vol. 3, No. 3, 99-101 (2009)
DOI: 10.1177/1753465809341650


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