title: Survival in systemic sclerosis with pulmonary arterial hypertension has not improved in the modern era.
abstract: BACKGROUND
  The impact of modern therapy on survival in pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc) is not clear. We sought to determine associations among commonly used clinical and hemodynamic variables, treatment, and long-term survival in PAH associated with SSc compared with PAH defined as idiopathic, familial, or associated with anorexigens.
  
  
  METHODS
  The observation period (1996-2010) included the option for epoprostenol and the availability of oral agents in 2002 (modern era of endothelin antagonists and phosphodiesterase-5 inhibitors). Primary outcome was all-cause mortality.
  
  
  RESULTS
  Eighty-three patients had SSc (mean age, 59 years), and 120 had PAH (mean age, 51 years) (P &lt; .0001, &gt; 80% were functional class III or IV in both groups). Compared with PAH, SSc had a lower mean pulmonary artery pressure (48 mm Hg vs 58 mm Hg, P &lt; .0001) and pulmonary vascular resistance (10 resistance units vs 15 resistance units, P &lt; .0001), and a higher cardiac index (2.3 L/min/m2 vs 1.8 L/min/m2, P &lt; .0001). PAH was more often treated with prostacyclin (71% vs 44%, P &lt; .0001), but there were no differences in the use of monotherapy or combination oral therapy. SSc had a twofold-higher mortality over the 14 years. The 5-year survival in the modern era for PAH was 87%, compared with 51% for SSc (P &lt; .001).
  
  
  CONCLUSIONS
  Despite an improvement in clinical status, unlike in PAH, mortality in SSc has not improved since the introduction of epoprostenol.
authors.affiliation: Division of Cardiovascular Medicine, University of Michigan, Ann Arbor, MI. Electronic address: mrubenfi@med.umich.edu.
authors.forename: Melvyn
authors.initials: M
authors.lastname: Rubenfire
authors.affiliation: Preventive Medicine and Medicine-Cardiology, Northwestern University Feinberg School of Medicine, Chicago, IL.
authors.forename: Mark D
authors.initials: MD
authors.lastname: Huffman
authors.affiliation: Division of Cardiovascular Medicine, University of Michigan, Ann Arbor, MI.
authors.forename: Sangeetha
authors.initials: S
authors.lastname: Krishnan
authors.affiliation: Scleroderma Research Consultants, Avon, CT.
authors.forename: James R
authors.initials: JR
authors.lastname: Seibold
authors.affiliation: Division of Rheumatology, University of Michigan, Ann Arbor, MI.
authors.forename: Elena
authors.initials: E
authors.lastname: Schiopu
authors.affiliation: Division of Cardiovascular Medicine, University of Michigan, Ann Arbor, MI.
authors.forename: Vallerie V
authors.initials: VV
authors.lastname: McLaughlin
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D015331:Cohort Studies; D011464:Epoprostenol / Q000627:therapeutic use; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications* / Q000401:mortality*; D015996:Survival Rate
chemical_list: D000959:Antihypertensive Agents; D011464:Epoprostenol