title: Prevalence of pulmonary hypertension in an unselected, mixed connective tissue disease cohort: results of a nationwide, Norwegian cross-sectional multicentre study and review of current literature.
abstract: OBJECTIVE
  The aim of this study was to assess the overall prevalence of pulmonary hypertension (PH) in an unselected MCTD cohort and review the current knowledge with a systematic database search.
  
  
  METHODS
  A nationwide multicentre cohort of 147 adult MCTD patients were initially screened for PH by echocardiography, high-resolution computed tomography (HRCT), pulmonary function tests and N-terminal pro-brain natriuretic peptide (NT-proBNP) and then followed up for a mean of 5.6 years. Right-sided heart catheterization was performed when estimated pulmonary artery systolic pressure was >40 mmHg on echocardiography. PH was diagnosed according to the 2009 European Society of Cardiology and European Respiratory Society guidelines.
  
  
  RESULTS
  At inclusion, 2.0% (3/147) had established PH. Two additional PH patients were identified during follow-up, giving a total PH frequency in the cohort of 3.4% (5/147). All five had elevated serum NT-proBNP. Two had isolated pulmonary arterial hypertension (PAH) and three PH associated with interstitial lung disease (PH-ILD). Three PH patients died during follow-up. Nine other patients in the cohort also died, but none of them had echocardiographic signs of PH prior to death.
  
  
  CONCLUSIONS
  The data from the current unselected MCTD cohort suggest that the prevalence of PH is much lower than expected from previous studies but confirm the seriousness of the disease complication.
authors: Rheumatology Unit, Oslo University Hospital Rikshospitalet, PO Box 4950 Nydalen, 0424 Oslo, Norway. ragunnar@gmail.com
authors: Ragnar
authors: R
authors: Gunnarsson
authors: Arne K
authors: AK
authors: Andreassen
authors: Øyvind
authors: Ø
authors: Molberg
authors: Åse Stavland
authors: ÅS
authors: Lexberg
authors: Kari
authors: K
authors: Time
authors: Alvilde Sofie Strand
authors: AS
authors: Dhainaut
authors: Liv-Turid
authors: LT
authors: Bertelsen
authors: Øyvind
authors: Ø
authors: Palm
authors: Karen
authors: K
authors: Irgens
authors: Andrea
authors: A
authors: Becker-Merok
authors: Jan Leidulf
authors: JL
authors: Nordeide
authors: Villy
authors: V
authors: Johnsen
authors: Sonja
authors: S
authors: Pedersen
authors: Anne
authors: A
authors: Prøven
authors: Lamya Samir Noori
authors: LS
authors: Garabet
authors: Torhild
authors: T
authors: Garen
authors: Trond Mogens
authors: TM
authors: Aaløkken
authors: Inge-Margrethe
authors: IM
authors: Gilboe
authors: Jan Tore
authors: JT
authors: Gran
mesh_terms: D000328:Adult; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D004452:Echocardiography; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology; D008297:Male; D008947:Mixed Connective Tissue Disease / Q000453:epidemiology*; D009664:Norway / Q000453:epidemiology; D015995:Prevalence; D014057:Tomography, X-Ray Computed / Q000379:methods
keywords: connective tissue diseases; echocardiography; heart catheterization; mixed connective tissue disease; prevalence; pulmonary hypertension; review