REVIEW

A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism

E Hachulla¹, J G Coghlan²

¹ Service de Médecine Interne, Hôpital Claude Huriez, Centre Hospitalier et Universitaire, 59037 Lille cedex, France
² Department of Cardiology, Royal Free Hospital, London NW3 2QG, UK

Correspondence to:
Correspondence to:
Professor E Hachulla
Service de Médecine Interne, Hôpital Claude Huriez, Centre Hospitalier et Universitaire, 59037 Lille cedex, France; ehachulla{at}chru-lille.fr

Evidence suggests that endothelin may have a fundamental role in scleroderma pathogenesis, including pulmonary arterial hypertension (PAH)—a leading cause of death in patients with scleroderma. Development of a new class of drug, endothelin receptor antagonists, heralds an improved outlook for patients with scleroderma and related diseases. Heightened vigilance towards early detection of PAH in scleroderma and a multidisciplinary approach to diagnosis and treatment may improve clinical outcomes for these patients.

Abbreviations: CCBs, calcium channel blockers; ERA, endothelin receptor antagonist; FVC, forced vital capacity; ILD, interstitial lung disease; mPAP, mean pulmonary arterial pressure; PAH, pulmonary arterial hypertension; TLCO, carbon monoxide transfer factor; VSMC, vascular smooth muscle cells

Keywords: scleroderma; pulmonary arterial hypertension; diagnosis; endothelin receptor antagonists

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

• Vachiery, J-L., Coghlan, G. (2009). Screening for pulmonary arterial hypertension in systemic sclerosis. ERR 18: 162-169 [Abstract] [Full Text]  
• Denton, C P, Pope, J E, Peter, H-H, Gabrielli, A, Boonstra, A, van den Hoogen, F H J, Riemekasten, G, De Vita, S, Morganti, A, Dolberg, M, Berkani, O, Guillevin, L, (on behalf of the TRacleer Use in PAH associated w, (2008). Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases. Ann Rheum Dis 67: 1222-1228 [Abstract] [Full Text]  
• Pauling, J. D., Gunawardena, H., Coghlan, J. G., Easaw, J., Suntharalingam, J., McHugh, N. J. (2008). Pulmonary artery hypertension as the presenting feature of systemic sclerosis sine scleroderma. Rheumatology (Oxford) 47: 1431-1432 [Full Text]  
• Girgis, R. E, Frost, A. E, Hill, N. S, Horn, E. M, Langleben, D., McLaughlin, V. V, Oudiz, R. J, Robbins, I. M, Seibold, J. R, Shapiro, S., Tapson, V. F, Barst, R. J (2007). Selective endothelinA receptor antagonism with sitaxsentan for pulmonary arterial hypertension associated with connective tissue disease. Ann Rheum Dis 66: 1467-1472 [Abstract] [Full Text]  
• Walker, U A, Tyndall, A, Czirjak, L, Denton, C, Farge-Bancel, D, Kowal-Bielecka, O, Muller-Ladner, U, Bocelli-Tyndall, C, Matucci-Cerinic, M, EUSTAR Co-authors, (2007). Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database. Ann Rheum Dis 66: 754-763 [Abstract] [Full Text]  
• Mok, M.Y., Tsang, P.L., Lam, Y.M., Lo, Y., Wong, W.S., Lau, C.S. (2007). Bosentan use in systemic lupus erythematosus patients with pulmonary arterial hypertension. Lupus 16: 279-285 [Abstract]  
• Collins, N., Bastian, B., Quiqueree, L., Jones, C., Morgan, R., Reeves, G. (2006). Abnormal pulmonary vascular responses in patients registered with a systemic autoimmunity database: Pulmonary Hypertension Assessment and Screening Evaluation using stress echocardiography (PHASE-I). Eur J Echocardiogr 7: 439-446 [Abstract] [Full Text]  
• Denton, C P, Humbert, M, Rubin, L, Black, C M (2006). Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis 65: 1336-1340 [Abstract] [Full Text]  
• Sticherling, M. (2006). The role of endothelin in connective tissue diseases. Rheumatology (Oxford) 45: iii8-iii10 [Abstract] [Full Text]  
• Battistini, B., Berthiaume, N., Kelland, N. F., Webb, D. J., Kohan, D. E. (2006). Profile of Past and Current Clinical Trials Involving Endothelin Receptor Antagonists: The Novel "-Sentan" Class of Drug.. Exp. Biol. Med. 231: 653-695 [Abstract] [Full Text]  
• Snyder, M. J., Jacobs, M. R., Grau, R. G., Wilkes, D. S., Knox, K. S. (2005). Resolution of Severe Digital Ulceration during a Course of Bosentan Therapy. ANN INTERN MED 142: 802-803 [Full Text]