The management of pulmonary arterial hypertension in the setting of systemic sclerosis

Stephen Mathai, N. Nazzareno Galié

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Pulmonary hypertension (PH) commonly complicates systemic sclerosis (SSc). PH related to SSc can develop in multiple ways and thus be classified in any of the five groups of pulmonary hypertension as defined by the World Health Organization. PH significantly impacts morbidity and mortality in SSc and, along with interstitial lung disease, is currently the leading cause of death in this population. While advances in therapeutics have led to improvements in symptoms, functional capacity, and outcomes in patients with pulmonary arterial hypertension (PAH), the response to therapy in PAH related to SSc (SSc-PAH) is, in general, less robust than other forms of PAH. Further, there are few randomized clinical trials of these therapies in other forms of PH, and thus, treatment in these populations is challenging. Newer therapeutic strategies, such as simultaneously targeting multiple pathways involved in the putative pathogenesis of the disease or utilizing inhaled therapies, may offer improved outcomes for SSc-PAH and lead to randomized trials in other forms of SSc-related PH. Focusing on supportive therapy, such as pulmonary rehabilitation, may also be important in the management of this complex disease.

Original languageEnglish (US)
Title of host publicationScleroderma: From Pathogenesis to Comprehensive Management
PublisherSpringer International Publishing
Pages411-419
Number of pages9
ISBN (Electronic)9783319314075
ISBN (Print)9783319314051
DOIs
StatePublished - Jan 1 2016

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Systemic Scleroderma
Pulmonary Hypertension
Therapeutics
Interstitial Lung Diseases
Disease Management
Population
Cause of Death
Rehabilitation
Randomized Controlled Trials
Morbidity

Keywords

  • Epidemiology
  • Outcomes
  • Pulmonary hypertension
  • Scleroderma
  • Treatment

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Mathai, S., & Nazzareno Galié, N. (2016). The management of pulmonary arterial hypertension in the setting of systemic sclerosis. In Scleroderma: From Pathogenesis to Comprehensive Management (pp. 411-419). Springer International Publishing. https://doi.org/10.1007/978-3-319-31407-5_28

The management of pulmonary arterial hypertension in the setting of systemic sclerosis. / Mathai, Stephen; Nazzareno Galié, N.

Scleroderma: From Pathogenesis to Comprehensive Management. Springer International Publishing, 2016. p. 411-419.

Research output: Chapter in Book/Report/Conference proceedingChapter

Mathai, S & Nazzareno Galié, N 2016, The management of pulmonary arterial hypertension in the setting of systemic sclerosis. in Scleroderma: From Pathogenesis to Comprehensive Management. Springer International Publishing, pp. 411-419. https://doi.org/10.1007/978-3-319-31407-5_28
Mathai S, Nazzareno Galié N. The management of pulmonary arterial hypertension in the setting of systemic sclerosis. In Scleroderma: From Pathogenesis to Comprehensive Management. Springer International Publishing. 2016. p. 411-419 https://doi.org/10.1007/978-3-319-31407-5_28
Mathai, Stephen ; Nazzareno Galié, N. / The management of pulmonary arterial hypertension in the setting of systemic sclerosis. Scleroderma: From Pathogenesis to Comprehensive Management. Springer International Publishing, 2016. pp. 411-419
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