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Pulmonary arterial hypertension in connective tissue disorders: Pathophysiology and treatment

Elisabetta Zanatta1, Pamela Polito1, Giulia Famoso2

  • 11 Department of Medicine-DIMED, Division of Rheumatology, University of Padova, 35128 Padova, Italy.

Experimental Biology and Medicine (Maywood, N.J.)
|January 24, 2019
PubMed

Insights

This review explores connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH), detailing its complex pathogenesis and treatment challenges. It highlights inflammation

Area of Science:

  • Cardiology and Rheumatology
  • Pulmonary Hypertension Pathogenesis

Background:

  • Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) presents unique therapeutic challenges compared to idiopathic PAH.
  • Current guidelines suggest similar treatment protocols but acknowledge increased complexity in CTD-PAH management.
  • The role of inflammation in right ventricle remodeling in systemic sclerosis-associated PAH (SSc-PAH) is a critical, often overlooked, aspect.

Purpose of the Study:

  • To provide an in-depth review focusing exclusively on the pathogenesis and treatment of CTD-PAH.
  • To elucidate the specific mechanisms driving CTD-PAH, including inflammation's role in right ventricle remodeling.
  • To critically analyze and discuss the major challenges and nuances in managing CTD-PAH.

Main Methods:

  • Comprehensive literature review of recent findings on CTD-PAH pathogenesis.
  • Analysis of current treatment strategies and guidelines for PAH.
  • Focus on specific aspects of CTD-PAH, particularly inflammation in SSc-PAH.

Main Results:

  • Inflammation plays a significant role in maladaptive right ventricle remodeling in SSc-PAH, challenging traditional views on immunosuppressant efficacy.
  • CTD-PAH requires a tailored therapeutic approach beyond standard PAH protocols.
  • Key critical points in CTD-PAH therapy are identified and discussed.

Conclusions:

  • CTD-PAH pathogenesis is complex, with inflammation being a key driver in specific subtypes like SSc-PAH.
  • Effective treatment of CTD-PAH necessitates addressing its unique pathological features and therapeutic complexities.
  • This review offers a focused perspective on CTD-PAH, addressing a gap in existing literature.
Abstract

Keywords:
Pulmonary arterial hypertensioncombination therapyconnective tissue disordersimmunosuppressantsvasodilators

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