Pulmonary Arterial Hypertension in Connective Tissue Disorders: Pathophysiology and Treatment

Overview

Journal Exp Biol Med (Maywood)

Specialty Biology

Date 2019 Jan 24

PMID 30669861

Citations 31

Authors

Elisabetta Zanatta

Pamela Polito

Giulia Famoso

Maddalena Larosa

Elena De Zorzi

Elena Scarpieri

Franco Cozzi

Andrea Doria

Affiliations

Soon will be listed here.

Abstract

Our article focuses on the pathogenesis and treatment of CTD-PAH. In the latest ESC/ESR guidelines for PAH, the authors underline that although CTD-PAH should follow the same treatment protocol as idiopathic PAH, the therapeutic approach is more complex and difficult in the former. This review throws light on several peculiar aspects of CTD-PAH and the latest findings in the pathogenesis, namely, the role of inflammation in the maladaptive right ventricle remodeling in SSc-PAH where immunosuppressants are classically believed to be ineffective. Furthermore, we discuss the major critical points in the therapy of CTD-PAH which is one of the strengths of our article. To the best of our knowledge, there are no other reviews that exclusively focus on the pathogenesis and treatment of CTD-PAH patients, with an emphasis on the more critical issues. Thus, it is our contention that our work would be of interest to the readers.

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