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

• Published in: Therapeutic advances in respiratory disease Vol. 3; no. 3; pp. 99 - 101
• Main Authors: Pitsiou, Georgia G., Spyratos, Dionysis, Kioumis, Ioannis, Boutou, Afroditi K., Nakou, Chrysanthi, Stanopoulos, Ioannis
• Format: Journal Article
• Language: English
• Published: London, England SAGE Publications 01.06.2009; SAGE PUBLICATIONS, INC; SAGE Publishing
• Subjects: Endothelin Receptor Antagonists; Humans; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - etiology; Male; Middle Aged; Pulmonary hypertension; Sarcoidosis; Sarcoidosis, Pulmonary - complications; Sulfonamides - therapeutic use; bosentan; pulmonary hypertension; sarcoidosis
• ISSN: 1753-4658; 1753-4666; 1753-4666
• DOI: 10.1177/1753465809341650

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.