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Exp Biol Med (Maywood). 2019 Jan 22:1535370218824101. doi: 10.1177/1535370218824101. [Epub ahead of print]

Pulmonary arterial hypertension in connective tissue disorders: Pathophysiology and treatment.

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1 Department of Medicine-DIMED, Division of Rheumatology, University of Padova, 35128 Padova, Italy.
2 Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, 35128 Padova, Italy.


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.


Pulmonary arterial hypertension; combination therapy; connective tissue disorders; immunosuppressants; vasodilators


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