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

Ther Adv Respir Dis. 2009 Jun;3(3):99-101. doi: 10.1177/1753465809341650. Epub 2009 Jul 28.


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.

Publication types

  • Case Reports

MeSH terms

  • Bosentan
  • Endothelin Receptor Antagonists*
  • Humans
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology
  • Male
  • Middle Aged
  • Sarcoidosis, Pulmonary / complications*
  • Sulfonamides / therapeutic use*


  • Endothelin Receptor Antagonists
  • Sulfonamides
  • Bosentan