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. 2010 Dec 1;1(2):11-20.

Pulmonary Arterial Hypertension-A Deadly Complication of Systemic Sclerosis

Edward A Pankey  1 , Matthew Epps, Bobby D Nossaman, Albert L Hyman, Philip J Kadowitz
Affiliations

Pulmonary Arterial Hypertension-A Deadly Complication of Systemic Sclerosis

Edward A Pankey et al. J Clin Rheumatol Musculoskelet Med. .

Abstract

Pulmonary arterial hypertension (PAH) is a devastating disease with limited therapeutic options. Moreover, when PAH occurs in patients diagnosed with systemic sclerosis, worse outcomes are observed. The purpose of this review is to discuss the etiologies of PAH found in the systemic sclerosis patient, limitations of current medical therapies, and, finally, potential therapies for patients with this combination.

Keywords: connective tissue disease; pulmonary arterial hypertension; pulmonary hypertension; systemic sclerosis.

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References

    1. Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010 Jun 15;69(10):1809–15. - PubMed
    1. Silman AJ. Scleroderma–demographics and survival. J Rheumatol Suppl. 1997 May;48:58–61. - PubMed
    1. Mayes MD, Lacey JV, Jr, Beebe-Dimmer J, Gillespie BW, Cooper B, Laing TJ, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003 Aug;48(8):2246–55. - PubMed
    1. McLaughlin V, Humbert M, Coghlan G, Nash P, Steen V. Pulmonary arterial hypertension: the most devastating vascular complication of systemic sclerosis. Rheumatology (Oxford) 2009 Jun;48(Suppl 3):iii25–31. - PubMed
    1. Hassoun PM. Therapies for scleroderma-related pulmonary arterial hypertension. Expert Rev Respir Med. 2009;3(2):187–96. - PMC - PubMed

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