This article is part of the supplement: Advances in systemic sclerosis and related fibrotic and vascular conditions
Pulmonary hypertension associated with sarcoidosis
Department of Internal Medicine, Interstitial Lung Disease and Sarcoidosis Clinic, University of Cincinnati Medical Center, Holmes Eden Ave, Cincinnati, Ohio 45267, USA
Arthritis Research & Therapy 2007, 9(Suppl 2):S8 doi:10.1186/ar2192Published: 15 August 2007
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.