Systemic sclerosis-associated pulmonary arterial hypertension

Jérôme Le Pavec, Marc Humbert, Luc Mouthon, Paul M. Hassoun

Research output: Contribution to journalReview articlepeer-review

Abstract

Pulmonary arterial hypertension (PAH) is a devastating vascular complication of a number of connective tissue diseases, including systemic sclerosis (SSc), where it has a dramatic impact on the clinical course and overall survival and is the single most common cause of death in patients afflicted with this syndrome. Although remarkable advances have been achieved in elucidating the pathogenesis of PAH over the past 2 decades, leading to the development of disease-targeted therapies for the idiopathic form of this condition (IPAH), the response to therapy is suboptimal in SSc-related PAH (SSc-PAH), and survival remains very poor. Factors accounting for striking clinical and prognostic differences between these two syndromes are unclear but may include a more pronounced autoimmune, cellular, and inflammatory response, and a higher prevalence of comorbidities in SSc-PAH, including cardiac and pulmonary venous and parenchymal involvement. Furthermore, currently available markers of disease severity and clinical tools to assess response to therapy, which may be reliable in IPAH, are either limited or lacking in SSc-PAH. Thus, a more focused approach, including a better understanding of the pathogenesis and genetic factors underlying the development of SSc-PAH, a search for more specific and reliable tools to adequately assess functional impairment and monitor therapy, as well as the design of novel targeted therapies, are all urgently required to alter the dismal course of this syndrome.

Original languageEnglish (US)
Pages (from-to)1285-1293
Number of pages9
JournalAmerican journal of respiratory and critical care medicine
Volume181
Issue number12
DOIs
StatePublished - Jun 15 2010

Keywords

  • Prognostic factors
  • Pulmonary hypertension
  • Scleroderma

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine

Fingerprint Dive into the research topics of 'Systemic sclerosis-associated pulmonary arterial hypertension'. Together they form a unique fingerprint.

Cite this