Management of the scleroderma patient with pulmonary arterial hypertension failing initial therapy

Christopher J. Mullin, Stephen Mathai

Research output: Chapter in Book/Report/Conference proceedingChapter


Pulmonary arterial hypertension (PAH) commonly complicates systemic sclerosis (SSc) and is a leading cause of death in this patient population. While advances in our understanding of the pathobiology of PAH have led to novel therapies directed at PAH, response to these agents in patients with PAH related to SSc (SSc-PAH) has in general been less robust than in patients with other forms of PAH. The reasons for these differences in response to therapy between SSc-PAH and other PAH patients are complex, but likely include the outcome measure selected to assess response (e.g., the 6-min walk test and hospitalizations) and concomitant diseases that influence response (e.g., gastrointestinal dysmotility and renal dysfunction), among others. In this chapter, we present a patient vignette to highlight the challenges in management of SSc-PAH and to offer our practice pattern to address these challenges.

Original languageEnglish (US)
Title of host publicationScleroderma: From Pathogenesis to Comprehensive Management
PublisherSpringer International Publishing
Number of pages10
ISBN (Electronic)9783319314075
ISBN (Print)9783319314051
StatePublished - Jan 1 2016


  • Lung transplantation
  • Medical management
  • Pulmonary arterial hypertension
  • Scleroderma

ASJC Scopus subject areas

  • General Medicine


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