title: Prediction of pulmonary hypertension related to systemic sclerosis by an index based on simple clinical observations.
abstract: OBJECTIVE
  To develop a score to estimate the risk of developing pulmonary hypertension (PH) in patients with systemic sclerosis (SSc).
  
  
  METHODS
  We first examined the prevalence and characteristics of precapillary PH confirmed by right-heart catheterization in a cross-sectional (derivation) sample of 1,165 SSc patients, and we developed a risk prediction score (RPS) based on simple clinical observations associated with PH. We next prospectively tested the 3-year predictive power of the "Cochin RPS" in a separate (validation) sample of 443 patients presenting with PH-free SSc at baseline.
  
  
  RESULTS
  In the derivation sample, age, forced vital capacity, and diffusing capacity for carbon monoxide/alveolar volume were independently associated with the presence of PH and were used to create the Cochin RPS. PH developed during followup in 20 patients in the validation sample. The area under the receiver operating characteristic curve of the Cochin RPS was 0.87 (95% confidence interval 0.79-0.95). With a cutoff value of 2.73, patients at risk of PH during followup could be identified with 89.5% sensitivity and 74.1% specificity. PH occurred in 0.6% of patients in the lowest 2 quintiles of the Cochin RPS, in 1.7% of patients in the third and fourth quintiles, and in 17.1% of patients in the highest quintile (P<0.0001 by log rank test). Patients in the highest quintile incurred a >35-fold higher risk of developing PH compared with patients in the 2 lowest quintiles (P=0.001).
  
  
  CONCLUSIONS
  Using routine clinical observations, we developed a simple score that accurately predicted the risk of PH in SSc.
authors.affiliation: Paris Descartes University and Department of Rheumatology A, Cochin Hospital, AP-HP, Paris, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Meune
authors.forename: Jérôme
authors.initials: J
authors.lastname: Avouac
authors.forename: Paolo
authors.initials: P
authors.lastname: Airò
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Beretta
authors.forename: Philippe
authors.initials: P
authors.lastname: Dieudé
authors.forename: Karim
authors.initials: K
authors.lastname: Wahbi
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: D000328:Adult; D000368:Aged; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D015995:Prevalence; D011446:Prospective Studies; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology