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An Update on Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: a Review of the Current Literature

  • Sneha M. Sundaram
  • Lorinda Chung
Scleroderma (J Varga, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Scleroderma

Abstract

Purpose of Review

This review will summarize the most current literature on the clinical impact, epidemiology, risk factors, screening recommendations, predictors of outcomes, and treatment options in patients with pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc).

Recent Findings

PAH continues to be a major cause of morbidity and mortality in SSc. Many risk factors and predictors of outcomes have been identified in patients with SSc including clinical, hemodynamic, and laboratory parameters. Screening for PAH in SSc patients is important and screening algorithms have been developed. Despite many available treatment options for PAH, prognosis remains poor.

Summary

Awareness of risk factors, early detection, and up-front combination treatment are important considerations in SSc-PAH and may lead to improved outcomes. Further research to develop better biomarkers and therapies is needed to continue to improve survival and outcomes in patients with SSc-PAH.

Keywords

Systemic sclerosis Pulmonary hypertension SSc PAH Scleroderma Connective tissue disease 

Notes

Compliance with Ethical Standards

Conflict of Interest

Dr. Chung reports grants from United Therapeutics, personal fees from Third Rock Venture, Reata, Gilead, and Actelion, outside the submitted work.

Dr. Sundaram has no conflicts to disclose.

Human and Animal Rights and Informed Consent

Human and Animal Rights: All reported studies/experiments with human or animal subjects performed by the authors have been previously published and complied with all applicable ethical standards (including the Helsinki declaration and its amendments, institutional/national research committee standards, and international/national/institutional guidelines).

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply 2018

Authors and Affiliations

  1. 1.Department of Internal MedicineCalifornia Pacific Medical CenterSan FranciscoUSA
  2. 2.Division of Immunology and RheumatologyStanford UniversityPalo AltoUSA
  3. 3.VA Palo Alto Health Care SystemPalo AltoUSA

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