title: Epidemiology and disease characteristics of systemic sclerosis-related pulmonary arterial hypertension: results from a real-life screening programme.
abstract: Pulmonary arterial hypertension (PAH) is the leading cause of death in systemic sclerosis (SSc). Annual screening with echocardiogram (ECHO) is recommended. We present the methodological aspects of a PAH screening programme in a large Australian SSc cohort, the epidemiology of SSc-PAH in this cohort, and an evaluation of factors influencing physician adherence to PAH screening guidelines.
  
  
  
  Patient characteristics and results of PAH screening were determined in all patients enrolled in a SSc longitudinal cohort study. Adherence to PAH screening guidelines was assessed by a survey of Australian rheumatologists. Summary statistics, chi-square tests, univariate and multivariable logistic regression were used to determine the associations of risk factors with PAH.
  
  
  
  Among 1636 patients with SSc, 194 (11.9%) had PAH proven by right-heart catheter. Of these, 160 were detected by screening. The annual incidence of PAH was 1.4%. Patients with PAH diagnosed on subsequent screens, compared with patients in whom PAH was diagnosed on first screen, were more likely to have diffuse SSc (p = 0.03), be in a better World Health Organisation (WHO) Functional Class at PAH diagnosis (p = 0.01) and have less advanced PAH evidenced by higher mean six-minute walk distance (p = 0.03), lower mean pulmonary arterial pressure (p = 0.009), lower mean pulmonary vascular resistance (p = 0.006) and fewer non-trivial pericardial effusions (p = 0.03). Adherence to annual PAH screening using an ECHO-based algorithm was poor among Australian rheumatologists, with less than half screening their patients with SSc of more than ten years disease duration.
  
  
  
  PAH is a common complication of SSc. Physician adherence to PAH screening recommendations remains poor. Identifying modifiable barriers to screening may improve adherence and ultimately patient outcomes.
authors.affiliation: Department of Medicine, The University of Melbourne at St Vincent's Hospital (Melbourne), 41 Victoria Parade, Fitzroy, 3065, Victoria, Australia.|Departments of Rheumatology and Medicine, The University of Melbourne at St Vincent's Hospital (Melbourne), 41 Victoria Parade, Fitzroy, 3065, Victoria, Australia.
authors.forename: Kathleen
authors.initials: K
authors.lastname: Morrisroe
authors.affiliation: Departments of Rheumatology and Medicine, The University of Melbourne at St Vincent's Hospital (Melbourne), 41 Victoria Parade, Fitzroy, 3065, Victoria, Australia.
authors.forename: Wendy
authors.initials: W
authors.lastname: Stevens
authors.affiliation: Monash University and Monash Health, 246 Clayton Road, Clayton, 3168, Victoria, Australia.
authors.forename: Joanne
authors.initials: J
authors.lastname: Sahhar
authors.affiliation: Departments of Rheumatology and Medicine, The University of Melbourne at St Vincent's Hospital (Melbourne), 41 Victoria Parade, Fitzroy, 3065, Victoria, Australia.
authors.forename: Candice
authors.initials: C
authors.lastname: Rabusa
authors.affiliation: Department of Medicine, The University of Melbourne at St Vincent's Hospital (Melbourne), 41 Victoria Parade, Fitzroy, 3065, Victoria, Australia. m.nikpour@unimelb.edu.au.|Departments of Rheumatology and Medicine, The University of Melbourne at St Vincent's Hospital (Melbourne), 41 Victoria Parade, Fitzroy, 3065, Victoria, Australia. m.nikpour@unimelb.edu.au.
authors.forename: Mandana
authors.initials: M
authors.lastname: Nikpour
authors.affiliation: Rheumatology Unit, Royal Adelaide Hospital, North Terrace, SA, 5000, Australia.|Discipline of Medicine, University of Adelaide, Adelaide, SA, 5000, Australia.
authors.forename: Susanna
authors.initials: S
authors.lastname: Proudman
mesh_terms: D000328:Adult; D000368:Aged; D001315:Australia; D015331:Cohort Studies; D005260:Female; D019983:Guideline Adherence / Q000706:statistics & numerical data*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology*; D008137:Longitudinal Studies; D008297:Male; D008403:Mass Screening / Q000706:statistics & numerical data*; D008875:Middle Aged; D012219:Rheumatology / Q000706:statistics & numerical data; D012595:Scleroderma, Systemic / Q000150:complications*
keywords: Pulmonary arterial hypertension; Scleroderma; Screening algorithm; Systemic sclerosis