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Review
. 2004 Sep;63(9):1009-14.
doi: 10.1136/ard.2003.017673.

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

E Hachulla  1 , J G Coghlan
Affiliations
Review

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

E Hachulla et al. Ann Rheum Dis. 2004 Sep.

Abstract

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.

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Figures

Figure 1
Figure 1
PAH: pathogenesis occurs before clinical symptoms.22 Reproduced, with permission of Elsevier Science from Rich.22
See this image and copyright information in PMC

References

    1. Nature. 1988 Mar 31;332(6163):411-5 - PubMed
    1. Prog Cardiovasc Dis. 1988 Nov-Dec;31(3):205-38 - PubMed
    1. N Engl J Med. 1992 Jul 9;327(2):76-81 - PubMed
    1. Q J Med. 1992 Feb;82(298):139-48 - PubMed
    1. J Rheumatol. 1992 Oct;19(10):1566-71 - PubMed

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