title: Sex disparities in systemic sclerosis-associated pulmonary arterial hypertension: a cohort study.
abstract: BACKGROUND
  The impact of male sex as a determinant of health outcomes in systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is controversial. The primary objective of this study was to evaluate the effect of sex on survival in patients with SSc-PAH. The secondary objectives were to evaluate the effect of sex on age of PAH diagnosis, time from SSc diagnosis to PAH diagnosis, and SSc disease manifestations.
  
  
  METHODS
  Sex-based disparities were evaluated in a cohort of SSc-PAH patients with a primary outcome of time from PAH diagnosis to all-cause mortality. Secondary outcomes were differences in age of diagnosis, disease duration, and SSc manifestations. Survival differences were evaluated using Kaplan-Meier and Cox proportional hazard models.
  
  
  RESULTS
  We identified 378 SSc-PAH (58 males, 320 females) patients, with a female:male ratio of 5.5:1. Males had a shorter mean ± standard deviation time from SSc diagnosis to PAH diagnosis (1.7 ± 14 versus 5.5 ± 14.2 years); shorter PAH duration (3.5 ± 3.1 versus 4.7 ± 4.2 years), increased frequency of renal crisis (19 % versus 8 %, relative risk (RR) 2.33, 95 %CI 1.22, 4.46), interstitial lung disease (67 % versus 48 %, RR 1.41, 95 %CI 1.14, 1.74), and diffuse subtype (40 % versus 22 %, RR 1.84, 95 %CI 1.26, 2.69). Males appeared to have decreased 1-, 2-, 3-, and 5-year survival (83.2 %, 68.7 %, 53.2 %, 45.6 %) compared to females (85.7 %, 75.7 %, 66.4 %, 57.4 %). However, there was no difference in mortality between sexes (HR 1.43 (95 %CI 0.97, 2.13).
  
  
  CONCLUSIONS
  Sex disparities appear to exist in the frequency of PAH, time to PAH diagnosis, PAH disease duration and SSc disease burden. However, male sex does not independently impact SSc-PAH survival.
authors.affiliation: Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, Faculty of Medicine, University of Toronto, Ground Floor, East Wing, Toronto Western Hospital, 399 Bathurst Street, Toronto, ON, M5T 2S8, Canada. cpasarikovski2015@meds.uwo.ca.
authors.forename: Christopher R
authors.initials: CR
authors.lastname: Pasarikovski
authors.affiliation: University Health Network Pulmonary Hypertension Programme, Toronto General Hospital, Divisions of Respirology and Critical Care Medicine, Department of Medicine, Faculty of Medicine, University of Toronto, Toronto, ON, Canada. John.Granton@uhn.ca.
authors.forename: John T
authors.initials: JT
authors.lastname: Granton
authors.affiliation: Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, Faculty of Medicine, University of Toronto, Ground Floor, East Wing, Toronto Western Hospital, 399 Bathurst Street, Toronto, ON, M5T 2S8, Canada. adrienne.roos@gmail.com.
authors.forename: Adrienne M
authors.initials: AM
authors.lastname: Roos
authors.affiliation: Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, Faculty of Medicine, University of Toronto, Ground Floor, East Wing, Toronto Western Hospital, 399 Bathurst Street, Toronto, ON, M5T 2S8, Canada. aaylary@yahoo.ca.
authors.forename: Saghar
authors.initials: S
authors.lastname: Sadeghi
authors.affiliation: Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, Faculty of Medicine, University of Toronto, Ground Floor, East Wing, Toronto Western Hospital, 399 Bathurst Street, Toronto, ON, M5T 2S8, Canada. amiekron@gmail.com.
authors.forename: Amie T
authors.initials: AT
authors.lastname: Kron
authors.affiliation: University Health Network Pulmonary Hypertension Programme, Toronto General Hospital, Division of Respirology, Department of Medicine, Faculty of Medicine, University of Toronto, Toronto, ON, Canada. John.Thenganatt@uhn.ca.
authors.forename: John
authors.initials: J
authors.lastname: Thenganatt
authors.affiliation: University Health Network Pulmonary Hypertension Programme, Toronto General Hospital, Division of Respirology, Women's College Hospital, Department of Medicine, Faculty of Medicine, University of Toronto, Toronto, ON, Canada. Jakov.Moric@uhn.ca.
authors.forename: Jakov
authors.initials: J
authors.lastname: Moric
authors.affiliation: Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, Faculty of Medicine, University of Toronto, Ground Floor, East Wing, Toronto Western Hospital, 399 Bathurst Street, Toronto, ON, M5T 2S8, Canada. Cathy.Chau@uhn.ca.
authors.forename: Cathy
authors.initials: C
authors.lastname: Chau
authors.affiliation: Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, Faculty of Medicine, University of Toronto, Ground Floor, East Wing, Toronto Western Hospital, 399 Bathurst Street, Toronto, ON, M5T 2S8, Canada. sindhu.Johnson@uhn.ca.|University Health Network Pulmonary Hypertension Programme, Toronto General Hospital, Division of Respirology, Department of Medicine, Faculty of Medicine, University of Toronto, Toronto, ON, Canada. sindhu.Johnson@uhn.ca.|Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, ON, Canada. sindhu.Johnson@uhn.ca.
authors.forename: Sindhu R
authors.initials: SR
authors.lastname: Johnson
mesh_terms: D000328:Adult; D000368:Aged; D015331:Cohort Studies; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000401:mortality*; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000401:mortality*; D012727:Sex Characteristics; D015996:Survival Rate / Q000639:trends
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