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lüll Pulmonary hypertension associated with sarcoidosis Baughman RPArthritis Res Ther 2007[]; 9 Suppl 2 (Suppl 2): S8Pulmonary 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.|Aged[MESH]|Animals[MESH]|Endothelin Receptor Antagonists[MESH]|Female[MESH]|Humans[MESH]|Hypertension, Pulmonary/*complications/*diagnosis/immunology[MESH]|Male[MESH]|Middle Aged[MESH]|Receptors, Endothelin/immunology[MESH]|Sarcoidosis, Pulmonary/*complications/*diagnosis/immunology[MESH]|Sarcoidosis/complications/diagnosis/immunology[MESH] |