Pulmonary hypertension associated with sarcoidosis.
Pulmonary involvement is common in sarcoidosis, an immune-mediated inflammatory disorder that is characterized by non-caseating granulomas in tissue. Sarcoid patients with advanced pulmonary disease, especially end-stage pulmonary fibrosis, risk developing pulmonary hypertension (World Health Organization group III pulmonary hypertension secondary to hypoxic lung disease). Increased levels of endothelin (ET)-1 in plasma and bronchoalveolar lavage of some sarcoid patients suggest that ET-1 may be driving pulmonary fibrosis and sarcoidosis-associated pulmonary hypertension. Although a relationship between raised levels of ET-1 and clinical phenotype is yet to be identified, early evidence from studies of ET-1 blockade with drugs such as bosentan is encouraging. Such therapy possibly could be combined with standard anti-inflammatory agents to improve outcome.
Baughman
Robert P
RP
Department of Internal Medicine, Interstitial Lung Disease and Sarcoidosis Clinic, University of Cincinnati Medical Center, Holmes Eden Ave, Cincinnati, Ohio 45267, USA. baughmrp@ucmail.uc.edu
D000368:Aged; D000818:Animals; D065128:Endothelin Receptor Antagonists; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D017466:Receptors, Endothelin / Q000276:immunology; D012507:Sarcoidosis / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D017565:Sarcoidosis, Pulmonary / Q000150:complications* / Q000175:diagnosis* / Q000276:immunology
D065128:Endothelin Receptor Antagonists; D017466:Receptors, Endothelin