title: Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies.
abstract: OBJECTIVE
  To measure the prevalence of different types of pulmonary hypertension (PH) and to identify patients with systemic sclerosis (SSc) at highest risk in a multicenter European sample, with a metaanalysis of relevant studies.
  
  
  METHODS
  Consecutive patients with SSc recruited at 11 French and Italian centers underwent detailed evaluations, including Doppler echocardiography, chest computed tomography, pulmonary function tests, and right-heart catheterization (RHC), to detect the presence and causes of PH. A metaanalysis was performed, including data from 4 other studies.
  
  
  RESULTS
  Among 206 patients in whom it was suspected, PH was confirmed by RHC in 83 patients (7%). Precapillary PH was found in 64 patients (5%), of whom 42 had pulmonary arterial hypertension (PAH) and 22 had PH secondary to interstitial lung disease (ILD). RHC identified 17 patients (1%) with postcapillary PH secondary to left-heart disease. Patients with DLCO/alveolar volume < 70% were more likely to have precapillary PH (87.5% vs 42%; p < 0.0001). Precapillary and postcapillary PH were associated with advanced age (68 ± 14 vs 59 ± 12 yrs, p < 0.0001, and 74 ± 16 vs 61.5 ± 10 yrs, p < 0.0001, respectively). The metaanalysis of 3818 patients showed a prevalence of precapillary PH of 9% (95% CI 6%-12%) and identified advanced age, longer disease duration, and limited cutaneous disease subset as risk factors for this condition.
  
  
  CONCLUSIONS
  The prevalence of precapillary PH in our multicenter study of SSc was 5%, and in the metaanalysis 9%. Our observations support use of RHC to confirm the presence of precapillary PH suspected by noninvasive testing. We also identified patients at high risk who should be carefully monitored.
authors.affiliation: Department of Rheumatology A, Paris Descartes University, Cochin Hospital, APHP, Paris, France.
authors.forename: Jérôme
authors.initials: J
authors.lastname: Avouac
authors.forename: Paolo
authors.initials: P
authors.lastname: Airò
authors.forename: Christophe
authors.initials: C
authors.lastname: Meune
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Beretta
authors.forename: Philippe
authors.initials: P
authors.lastname: Dieude
authors.forename: Paola
authors.initials: P
authors.lastname: Caramaschi
authors.forename: Kiet
authors.initials: K
authors.lastname: Tiev
authors.forename: Susanna
authors.initials: S
authors.lastname: Cappelli
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Diot
authors.forename: Alessandra
authors.initials: A
authors.lastname: Vacca
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Cracowski
authors.forename: Jean
authors.initials: J
authors.lastname: Sibilia
authors.forename: André
authors.initials: A
authors.lastname: Kahan
authors.forename: Marco
authors.initials: M
authors.lastname: Matucci-Cerinic
authors.forename: Yannick
authors.initials: Y
authors.lastname: Allanore
mesh_terms: D000367:Age Factors; D000368:Aged; D006328:Cardiac Catheterization; D015897:Comorbidity; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology*; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012595:Scleroderma, Systemic / Q000453:epidemiology*; D012720:Severity of Illness Index; D044465:White People