title: Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study.
abstract: OBJECTIVE
  Earlier detection of pulmonary arterial hypertension (PAH), a leading cause of death in systemic sclerosis (SSc), facilitates earlier treatment. The objective of this study was to develop the first evidence-based detection algorithm for PAH in SSc.
  
  
  METHODS
  In this cross-sectional, international study conducted in 62 experienced centres from North America, Europe and Asia, adults with SSc at increased risk of PAH (SSc for >3 years and predicted pulmonary diffusing capacity for carbon monoxide <60%) underwent a broad panel of non-invasive assessments followed by diagnostic right heart catheterisation (RHC). Univariable and multivariable analyses selected the best discriminatory variables for identifying PAH. After assessment for clinical plausibility and feasibility, these were incorporated into a two-step, internally validated detection algorithm. Nomograms for clinical practice use were developed.
  
  
  RESULTS
  Of 466 SSc patients at increased risk of PAH, 87 (19%) had RHC-confirmed PAH. PAH was mild (64% in WHO functional class I/II). Six simple assessments in Step 1 of the algorithm determined referral to echocardiography. In Step 2, the Step 1 prediction score and two echocardiographic variables determined referral to RHC. The DETECT algorithm recommended RHC in 62% of patients (referral rate) and missed 4% of PAH patients (false negatives). By comparison, applying European Society of Cardiology/European Respiratory Society guidelines to these patients, 29% of diagnoses were missed while requiring an RHC referral rate of 40%.
  
  
  CONCLUSIONS
  The novel, evidence-based DETECT algorithm for PAH detection in SSc is a sensitive, non-invasive tool which minimises missed diagnoses, identifies milder disease and addresses resource usage.
authors.affiliation: Cardiology Department, Royal Free Hospital, London, UK.
authors.forename: J Gerry
authors.initials: JG
authors.lastname: Coghlan
authors.affiliation: Centre for Rheumatology, Royal Free Hospital, London, UK.
authors.forename: Christopher P
authors.initials: CP
authors.lastname: Denton
authors.affiliation: Centre for Pulmonary Hypertension, University Hospital, Heidelberg, Germany.
authors.forename: Ekkehard
authors.initials: E
authors.lastname: Grünig
authors.affiliation: Medical University of Vienna, Department of Internal Medicine II, Division of Cardiology, Vienna, Austria.
authors.forename: Diana
authors.initials: D
authors.lastname: Bonderman
authors.affiliation: Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Oliver
authors.initials: O
authors.lastname: Distler
authors.affiliation: Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA.
authors.forename: Dinesh
authors.initials: D
authors.lastname: Khanna
authors.affiliation: Department of Rheumatology and Clinical Immunology, Justus-Liebig-University Giessen, Kerckhoff Clinic Bad Nauheim, Germany.
authors.forename: Ulf
authors.initials: U
authors.lastname: Müller-Ladner
authors.affiliation: Department of Medicine, Division of Rheumatology, Western University of Canada, London, Ontario, Canada.
authors.forename: Janet E
authors.initials: JE
authors.lastname: Pope
authors.affiliation: Department of Rheumatology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
authors.forename: Madelon C
authors.initials: MC
authors.lastname: Vonk
authors.affiliation: Global Medical Affairs, Actelion Pharmaceuticals Ltd, Allschwil, Switzerland.
authors.forename: Martin
authors.initials: M
authors.lastname: Doelberg
authors.affiliation: Clinical Development, Actelion Pharmaceuticals Ltd, Allschwil, Switzerland.
authors.forename: Harbajan
authors.initials: H
authors.lastname: Chadha-Boreham
authors.affiliation: Center for Medical Statistics, Informatics, and Intelligent Systems, Medical University of Vienna, Vienna, Austria.
authors.forename: Harald
authors.initials: H
authors.lastname: Heinzl
authors.affiliation: Clinical Development, Actelion Pharmaceuticals Ltd, Allschwil, Switzerland.
authors.forename: Daniel M
authors.initials: DM
authors.lastname: Rosenberg
authors.affiliation: Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA.
authors.forename: Vallerie V
authors.initials: VV
authors.lastname: McLaughlin
authors.affiliation: Scleroderma Research Consultants LLC, Avon, Connecticut, USA.
authors.forename: James R
authors.initials: JR
authors.lastname: Seibold
mesh_terms: D000328:Adult; D000368:Aged; D000465:Algorithms; D001944:Breath Tests; D006328:Cardiac Catheterization / Q000379:methods*; D003430:Cross-Sectional Studies; D042241:Early Diagnosis; D004452:Echocardiography / Q000379:methods*; D019317:Evidence-Based Medicine; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D049451:Nomograms; D011653:Pulmonary Diffusing Capacity; D011669:Pulmonary Wedge Pressure; D018570:Risk Assessment; D012595:Scleroderma, Systemic / Q000150:complications*; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index
keywords: Arterial Hypertension; Epidemiology; Systemic Sclerosis