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Complex Challenges of Pulmonary Hypertension

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Scleroderma

Abstract

It is now well appreciated that pulmonary hypertension is a frequent complication of scleroderma and that it occurs in both of the major disease subsets. However, despite a much greater understanding of the different forms of pulmonary hypertension and the availability of effective treatments for different forms of this disease, it remains one of the most feared complications of scleroderma. Compared with other forms of PAH that occur in scleroderma has a significantly worse outcome, and this is especially in comparison with idiopathic PAH. Thus, the strategies that are used for PAH may need to be modified in the context of scleroderma, and this is discussed below.

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Correspondence to John Gerard Coghlan MD, FRCP .

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Coghlan, J.G. (2012). Complex Challenges of Pulmonary Hypertension. In: Varga, J., Denton, C., Wigley, F. (eds) Scleroderma. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5774-0_38

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