title: A comparison of the predictive accuracy of three screening models for pulmonary arterial hypertension in systemic sclerosis.
abstract: BACKGROUND
  There is evidence that early screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) improves outcomes. We compared the predictive accuracy of two recently published screening algorithms (DETECT 2013 and Australian Scleroderma Interest Group (ASIG) 2012) for SSc-associated PAH (SSc-PAH) with the commonly used European Society of Cardiology/European Respiratory Society (ESC/ERS 2009) guidelines.
  
  
  METHODS
  We included 73 consecutive SSc patients with suspected PAH undergoing right heart catheterization (RHC). The three screening models were applied to each patient. For each model, contingency table analysis was used to determine sensitivity, specificity, and positive (PPV) and negative (NPV) predictive values for PAH. These properties were also evaluated in an 'alternate scenario analysis' in which the prevalence of PAH was set at 10%.
  
  
  RESULTS
  RHC revealed PAH in 27 (36.9%) patients. DETECT and ASIG algorithms performed equally in predicting PAH with sensitivity and NPV of 100%. The ESC/ERS guidelines had sensitivity of 96.3% and NPV of only 91%, missing one case of PAH; these guidelines could not be applied to three patients who had absent tricuspid regurgitant (TR) jet. The ASIG algorithm had the highest specificity (54.5%). With PAH prevalence set at 10%, the NPV of the models was unchanged, but the PPV dropped to less than 20%.
  
  
  CONCLUSIONS
  In this cohort, the DETECT and ASIG algorithms out-perform the ESC/ERS guidelines, detecting all patients with PAH. The ESC/ERS guidelines have limitations in the absence of a TR jet. Ultimately, the choice of SSc-PAH screening algorithm will also depend on cost and ease of application.
authors.affiliation: Department of Rheumatology, St Vincent's Hospital Melbourne, 41 Victoria Parade, Fitzroy, VIC, 3065, Australia. yanjie.hao@svhm.org.au.|Department of Rheumatology and Clinical Immunology, Peking University First Hospital, 8 Xishiku Street, Beijing, China. yanjie.hao@svhm.org.au.
authors.forename: Yanjie
authors.initials: Y
authors.lastname: Hao
authors.affiliation: Department of Rheumatology, St Vincent's Hospital Melbourne, 41 Victoria Parade, Fitzroy, VIC, 3065, Australia. vivek.thakkar@sswahs.nsw.gov.au.|Department of Rheumatology, Liverpool Hospital, Elizabeth Street, Liverpool, NSW 2170, Australia. vivek.thakkar@sswahs.nsw.gov.au.|School of Medicine, University of Western Sydney, Locked bag 1797, Penrith, NSW 2751, Australia. vivek.thakkar@sswahs.nsw.gov.au.
authors.forename: Vivek
authors.initials: V
authors.lastname: Thakkar
authors.affiliation: Department of Rheumatology, St Vincent's Hospital Melbourne, 41 Victoria Parade, Fitzroy, VIC, 3065, Australia. wendy.stevens@svhm.org.au.
authors.forename: Wendy
authors.initials: W
authors.lastname: Stevens
authors.affiliation: Department of Rheumatology, St Vincent's Hospital Melbourne, 41 Victoria Parade, Fitzroy, VIC, 3065, Australia. kathleen.morrisroe2@svhm.org.au.
authors.forename: Kathleen
authors.initials: K
authors.lastname: Morrisroe
authors.affiliation: Department of Cardiology, St Vincent's Hospital Melbourne, 41 Victoria Parade, Fitzroy, VIC, 3065, Australia. david.prior@svhm.org.au.
authors.forename: David
authors.initials: D
authors.lastname: Prior
authors.affiliation: Department of Rheumatology, St Vincent's Hospital Melbourne, 41 Victoria Parade, Fitzroy, VIC, 3065, Australia. candice.rabusa@svhm.org.au.
authors.forename: Candice
authors.initials: C
authors.lastname: Rabusa
authors.affiliation: Institute of Rheumatology and Orthopaedics, Royal Prince Alfred Hospital, Queen Elizabeth II building, Missendon Road, Camperdown, NSW 2050, Australia. petery@bigpond.net.au.
authors.forename: Peter
authors.initials: P
authors.lastname: Youssef
authors.affiliation: The University of Notre Dame, 19 Mouat Street, Fremantle, WA, 6959, Australia. egabbay@respiratorywest.com.au.
authors.forename: Eli
authors.initials: E
authors.lastname: Gabbay
authors.affiliation: Department of Rheumatology, Royal Perth Hospital, Wellington Street, GPO Box X2213, Perth, WA, 6001, Australia. janet.roddy@health.wa.gov.au.
authors.forename: Janet
authors.initials: J
authors.lastname: Roddy
authors.affiliation: Department of Rheumatology, Flinders Medical Centre, Flinders Drive, Bedford Park, SA, 5042, Australia. jennywalk@gmail.com.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Walker
authors.affiliation: Department of Rheumatology, The Menzies Institute Tasmania, Private Bag 23, Hobart, TAS 7001, Australia. jane.zochling@utas.edu.au.
authors.forename: Jane
authors.initials: J
authors.lastname: Zochling
authors.affiliation: Department of Rheumatology, Monash Medical Centre, 246 Clayton Road, Clayton, VIC, 3168, Australia. josahhar@bigpond.com.
authors.forename: Joanne
authors.initials: J
authors.lastname: Sahhar
authors.affiliation: Rheumatology Research Unit, Department of Medicine, University of Queensland, PO Box 368, Maroochydore, QLD 4558, Australia. pnash@tpg.com.au.
authors.forename: Peter
authors.initials: P
authors.lastname: Nash
authors.affiliation: Rheumatology Department, The Queen Elizabeth Hospital, 28 Woodville Road, Woodville South, SA, 5011, Australia. susan.lester@health.sa.gov.au.
authors.forename: Susan
authors.initials: S
authors.lastname: Lester
authors.affiliation: Rheumatology Department, The Queen Elizabeth Hospital, 28 Woodville Road, Woodville South, SA, 5011, Australia. maureen.rischmueller@health.sa.gov.au.|Discipline of Medicine, University of Adelaide, North Terrace, SA, 5000, Australia. maureen.rischmueller@health.sa.gov.au.
authors.forename: Maureen
authors.initials: M
authors.lastname: Rischmueller
authors.affiliation: Department of Rheumatology, Royal Adelaide Hospital, North Terrace, SA, 5000, Australia. sproudman@internode.on.net.|Discipline of Medicine, University of Adelaide, North Terrace, SA, 5000, Australia. sproudman@internode.on.net.
authors.forename: Susanna M
authors.initials: SM
authors.lastname: Proudman
authors.affiliation: Department of Rheumatology, St Vincent's Hospital Melbourne, 41 Victoria Parade, Fitzroy, VIC, 3065, Australia. m.nikpour@unimelb.edu.au.|Department of Medicine at St Vincent's Hospital Melbourne, The University of Melbourne, 41 Victoria Parade, Fitzroy, VIC, 3065, Australia. m.nikpour@unimelb.edu.au.
authors.forename: Mandana
authors.initials: M
authors.lastname: Nikpour
mesh_terms: D000368:Aged; D000465:Algorithms; D015331:Cohort Studies; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000453:epidemiology*; D008297:Male; D008403:Mass Screening / Q000379:methods / Q000592:standards*; D008875:Middle Aged; D011237:Predictive Value of Tests; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000453:epidemiology*