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

TitleSarcoidosis-associated pulmonary hypertension: a role for endothelin receptor antagonists?
Publication TypeJournal Article
Year of Publication2009
AuthorsPitsiou, G. G., Spyratos D., Kioumis I., Boutou A. K., Nakou C., & Stanopoulos I.
JournalTher Adv Respir Dis
Volume3
Issue3
Pagination99-101
Date Published2009 Jun
ISSN1753-4658
KeywordsEndothelin Receptor Antagonists, Humans, Hypertension, Pulmonary, Male, Middle Aged, Sarcoidosis, Pulmonary, Sulfonamides
Abstract

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.

DOI10.1177/1753465809341650
Alternate JournalTher Adv Respir Dis
PubMed ID19638368

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