title: Recognition of pulmonary hypertension in the rheumatology community: lessons from a Quality Enhancement Research Initiative.
abstract: OBJECTIVE
  The aim of this study was to utilise the Quality Enhancement Research Initiative in Systemic Sclerosis (QuERI-SSc) to measure and reduce a perceived gap in the diagnosis of pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc).
  
  
  METHODS
  Rheumatologists enrolled patients with SSc (aged ≥ 18 years) and provided data on a panel of diagnostic tests over 3 years. Pulmonary function testing, echocardiography, 6-minute walk distance, N-terminal pro-brain natriuretic peptide assays, high-resolution computed tomography of the lungs, and ventilation/perfusion scan plus right heart catheterisation (RHC; when appropriate) were emphasised. Exclusion criteria included previously documented PAH, interstitial lung disease, and SSc overlapping with other connective tissue disease.
  
  
  RESULTS
  Participating rheumatologists enrolled 207 patients with SSc (90% female; 80% white), with a median age of 57 years and median disease duration of 5 years. A total of 82% of patients were classified as New York Heart Association functional class I and II; of these patients, 177 had an echocardiogram at enrolment and 191 at any time during the study. Of those who met study-specified criteria for RHC at enrolment, only 3 of 7 patients underwent RHC.
  
  
  CONCLUSIONS
  The screening algorithm was successful in identifying patients with mild impairment. Although specific tools were recommended for screening PAH in patients with SSc, results indicate that significant diagnostic care gaps still exist in the general rheumatology community. Better understanding and adherence to guidelines could improve the care and, ideally, outcomes of these high-risk patients.
authors.affiliation: Department of Internal Medicine, University of Michigan Scleroderma Program, Ann Arbor, MI, USA. khannad@med.umich.edu.
authors.forename: D
authors.initials: D
authors.lastname: Khanna
authors.forename: Ma
authors.initials: M
authors.lastname: Tan
authors.forename: D E
authors.initials: DE
authors.lastname: Furst
authors.forename: N S
authors.initials: NS
authors.lastname: Hill
authors.forename: V V
authors.initials: VV
authors.lastname: McLaughlin
authors.forename: R M
authors.initials: RM
authors.lastname: Silver
authors.forename: V D
authors.initials: VD
authors.lastname: Steen
authors.forename: A
authors.initials: A
authors.lastname: Langer
authors.forename: J R
authors.initials: JR
authors.lastname: Seibold
mesh_terms: D000368:Aged; D006328:Cardiac Catheterization; D019468:Disease Management; D015150:Echocardiography, Doppler; D005260:Female; D019983:Guideline Adherence; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology; D008168:Lung / Q000000981:diagnostic imaging*; D008297:Male; D008403:Mass Screening; D008875:Middle Aged; D017410:Practice Guidelines as Topic; D058996:Quality Improvement; D011787:Quality of Health Care; D013902:Radiography, Thoracic; D012129:Respiratory Function Tests; D012219:Rheumatology / Q000592:standards*; D012595:Scleroderma, Systemic / Q000150:complications / Q000628:therapy*; D014057:Tomography, X-Ray Computed