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Vascular receptor autoantibodies in pulmonary arterial hypertension associated with systemic sclerosis.
Mike O Becker
1 ,
Angela Kill ,
Marissa Kutsche ,
Jeannine Guenther ,
Angelika Rose ,
Christoph Tabeling ,
Martin Witzenrath ,
Anja A Kühl ,
Harald Heidecke ,
Hossein A Ghofrani ,
Henning Tiede ,
Ralph T Schermuly ,
Nils Nickel ,
Marius M Hoeper ,
Ivo Lukitsch ,
Maik Gollasch ,
Wolfgang M Kuebler ,
Sebastian Bock ,
Gerd R Burmester ,
Duska Dragun ,
Gabriela Riemekasten
Oct 1 2014
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Abstract
Systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) portends worse outcome than other forms of PAH. Vasoconstrictive and vascular remodeling actions of endothelin (ET) 1 and angiotensin (Ang) II via endothelin receptor type A (ETAR) and Ang receptor type-1 (AT1R) activation are implicated in PAH pathogenesis.