title: Comparison of baseline characteristics and survival between patients with idiopathic and connective tissue disease-related pulmonary arterial hypertension.
abstract: BACKGROUND
  Both idiopathic pulmonary arterial hypertension (IPAH) and pulmonary arterial hypertension (PAH) related to connective tissue diseases (CPAH) are classified in the group of PAH disorders. However, CPAH has a particularly worse prognosis than IPAH. Few studies have compared the clinical, functional and hemodynamic profiles of IPAH and CPAH.
  
  
  METHODS
  We performed a retrospective cohort study of patients with IPAH or CPAH. Demographic characteristics, functional status (FE), pulmonary function test and hemodynamic values at the time of diagnosis were compared between the two etiologies. Global cumulative survival rates free from transplantation (SFT) and survival according to date of diagnosis were analyzed.
  
  
  RESULTS
  Despite similar PAH severity, patients with CPAH showed a more severe baseline impairment of 6-minute walking test (6MWT) (307 +/- 116 m vs 378 +/- 101 m) and diffusion capacity of the lung for carbon monoxide (DLCO) (57 +/- 25% vs 75 +/- 30% of predicted) than IPAH (p < 0.01). Survival rates at 1, 3 and 5 years of follow-up were 87%, 71% and 63% for IPAH, and 70%, 53% and 42% for CPAH, respectively (p < 0.05). IPAH showed better survival when treatment was started after Year 2000 (p = 0.01). However, CPAH showed a poorer prognosis than IPAH in the more recent era (p < 0.05). CPAH (hazard ratio [HR] = 2.03), DLCO <80% (HR = 1.98) and treatment before Year 2000 (HR = 2.27) were associated with an independent increased risk of death or transplantation.
  
  
  CONCLUSIONS
  Despite similar functional and hemodynamic severity, patients with CPAH showed a more severe baseline impairment of 6MWT and DLCO and worse overall prognosis than IPAH. Both IPAH and CPAH survival improved in the current era. Nevertheless, CPAH still showed a poorer prognosis than IPAH.
authors.affiliation: Pulmonary Hypertension, Heart Failure and Heart Transplantation Unit, Cardiology Department, 12 de Octubre University Hospital, Avenida Andalucia Km 5.4, Madrid, Spain. majorcardio@yahoo.es
authors.forename: Maria J
authors.initials: MJ
authors.lastname: Ruiz-Cano
authors.forename: Pilar
authors.initials: P
authors.lastname: Escribano
authors.forename: Rafael
authors.initials: R
authors.lastname: Alonso
authors.forename: Juan
authors.initials: J
authors.lastname: Delgado
authors.forename: Patricia
authors.initials: P
authors.lastname: Carreira
authors.forename: Teresa
authors.initials: T
authors.lastname: Velazquez
authors.forename: Miguel A Gomez
authors.initials: MA
authors.lastname: Sanchez
authors.forename: Carlos
authors.initials: C
authors.lastname: Sáenz de la Calzada
mesh_terms: D000328:Adult; D001794:Blood Pressure / Q000502:physiology; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D015331:Cohort Studies; D003240:Connective Tissue Diseases / Q000188:drug therapy / Q000401:mortality* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000401:mortality* / Q000503:physiopathology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008168:Lung / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate; D016138:Walking / Q000502:physiology
chemical_list: D002121:Calcium Channel Blockers; D007166:Immunosuppressive Agents