Pulmonary hypertension associated with sarcoidosis.
Baughman 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
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.
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PMID: 17767746 [PubMed - indexed for MEDLINE]PMCID: PMC2072890