title: Improved transplant-free survival in patients with systemic sclerosis-associated pulmonary hypertension and interstitial lung disease.
abstract: OBJECTIVE
  Survival in patients with systemic sclerosis (SSc)-associated pulmonary hypertension (PH) and interstitial lung disease (ILD) is poor. Evidence supporting the efficacy of aggressive pulmonary arterial hypertension (PAH)-targeted therapy in this population is limited. The aim of this study was to investigate transplant-free survival in patients with isolated SSc-related PAH or SSc-related PH-ILD who were treated with aggressive PAH-targeted therapy.
  
  
  METHODS
  SSc patients with right-sided heart catheterization (RHC)-diagnosed precapillary PH (mean pulmonary artery pressure ≥25 mm Hg, pulmonary capillary wedge pressure ≤15 mm Hg, and pulmonary vascular resistance ≥240 dynes × second/cm(5) ) were included. Patients were classified as having ILD based on review of high-resolution computed tomography (CT) chest imaging and spirometry. The Kaplan-Meier method was applied and Cox proportional hazards models were constructed to analyze survival and identify predictive variables.
  
  
  RESULTS
  Of 99 patients with SSc-related precapillary PH, 28% had SSc-related PAH and 72% had SSc-related PH-ILD. The 1- and 2-year survival estimates were, respectively, 72% and 59% in the SSc-related PH-ILD group versus 82% and 66% in the SSc-related PAH group (P = 0.5). Within 6 months of the diagnostic RHC, 24% of all patients were started on prostanoid therapy; an additional 24% were started on prostanoid therapy after 6 months. In the multivariate model, male sex (hazard ratio [HR] 0.7, P = 0.01) and prostanoid therapy initiation within 6 months of the RHC (HR 1.4, P = 0.01) were the only factors significantly associated with transplant-free survival, after accounting for the presence of ILD and severity of PH.
  
  
  CONCLUSIONS
  In this study, survival of patients with SSc-related PH-ILD was modestly improved relative to historical series. While these findings may not be generalizable, improved survival may be due partly to aggressive PAH-targeted therapy.
authors.affiliation: University of California, Los Angeles.
authors.forename: Elizabeth R
authors.initials: ER
authors.lastname: Volkmann
authors.forename: Rajeev
authors.initials: R
authors.lastname: Saggar
authors.forename: Dinesh
authors.initials: D
authors.lastname: Khanna
authors.forename: Bryant
authors.initials: B
authors.lastname: Torres
authors.forename: Arjan
authors.initials: A
authors.lastname: Flora
authors.forename: Lynne
authors.initials: L
authors.lastname: Yoder
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Clements
authors.forename: Robert M
authors.initials: RM
authors.lastname: Elashoff
authors.forename: David J
authors.initials: DJ
authors.lastname: Ross
authors.forename: Harsh
authors.initials: H
authors.lastname: Agrawal
authors.forename: Nabeel
authors.initials: N
authors.lastname: Borazan
authors.forename: Daniel E
authors.initials: DE
authors.lastname: Furst
authors.forename: Rajan
authors.initials: R
authors.lastname: Saggar
mesh_terms: D000328:Adult; D000368:Aged; D065128:Endothelin Receptor Antagonists; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality*; D053208:Kaplan-Meier Estimate; D017563:Lung Diseases, Interstitial / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D011379:Prognosis; D016016:Proportional Hazards Models; D011669:Pulmonary Wedge Pressure / Q000187:drug effects; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000188:drug therapy* / Q000401:mortality*; D014655:Vascular Resistance / Q000187:drug effects
chemical_list: D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors