title: Prediction of new onset of resting pulmonary arterial hypertension in systemic sclerosis.
abstract: BACKGROUND
  Early detection of pulmonary arterial hypertension (PH) is crucial in systemic scleroderma. However, predictors of new onset of resting PH during follow-up (FUPH) have been poorly explored.
  
  
  OBJECTIVE
  To determine whether nailfold videocapillaroscopy (NVC) grade and exercise echocardiographic variables are predictors of FUPH.
  
  
  METHODS
  We prospectively enrolled 40 patients with systemic sclerosis (age 54±13 years; 68% women). All patients underwent graded semisupine exercise echocardiography and NVC. Baseline resting PH and FUPH were defined as systolic pulmonary arterial pressure (sPAP)>35 mmHg, and exercise-induced PH (EIPH) as exercise sPAP>50 mmHg.
  
  
  RESULTS
  Seventeen patients developed EIPH (43%). During follow-up (FU) (25±15 months), 11 patients without baseline PH developed FUPH (28%), all from the EIPH group. Patients with FUPH were significantly older (60±14 vs 50±12 years; P=0.04), had higher resting and exercise sPAP (30±4 vs 22±5 and 60±12 vs 40±11 mmHg, respectively; P<0.0001) and a higher exercise E/e' ratio (9.4±0.7 vs 5.8±0.4; P=0.0003) and presented more frequently NVC grade>2 (90% vs 35%; P=0.0009). After adjustment for age, resting sPAP, exercise sPAP and NVC grade>2 were associated with maximal resting sPAP during follow-up and FUPH (P<0.05). Patients with both EIPH and NVC grade>2 had a very high incidence of FUPH (82%), and both variables remained strongly associated with FUPH after adjustment for age (hazard ratio 11.6, 95% confidence interval 2.4-55.3; P=0.002).
  
  
  CONCLUSIONS
  Exercise echocardiography and NVC can identify a subgroup of patients with systemic sclerosis who are at risk of developing FUPH.
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Damien
authors.initials: D
authors.lastname: Voilliot
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Julien
authors.initials: J
authors.lastname: Magne
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Raluca
authors.initials: R
authors.lastname: Dulgheru
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Seisyou
authors.initials: S
authors.lastname: Kou
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Christine
authors.initials: C
authors.lastname: Henri
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Luis
authors.initials: L
authors.lastname: Caballero
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Carla
authors.initials: C
authors.lastname: De Sousa
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Muriel
authors.initials: M
authors.lastname: Sprynger
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium.
authors.forename: Béatrice
authors.initials: B
authors.lastname: Andre
authors.affiliation: University of Liège Hospital, GIGA Cardiovascular Sciences, Department of Cardiology, Heart Valve Clinic, University Hospital Sart Tilman, Liège, Belgium. Electronic address: lpierard@chu.ulg.ac.be.
authors.forename: Luc A
authors.initials: LA
authors.lastname: Pierard
authors.affiliation: Gruppo Villa Maria Care and Research, Anthea Hospital, Bari, Italy. Electronic address: plancellotti@chu.ulg.ac.be.
authors.forename: Patrizio
authors.initials: P
authors.lastname: Lancellotti
mesh_terms: D004452:Echocardiography; D005080:Exercise Test; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology*; D008297:Male; D020678:Microscopic Angioscopy; D008875:Middle Aged; D011237:Predictive Value of Tests; D011379:Prognosis; D011446:Prospective Studies; D012595:Scleroderma, Systemic / Q000150:complications*
keywords: Capillaroscopie; Exercise echocardiography; Hypertension artérielle pulmonaire; Nailfold videocapillaroscopy; Pulmonary arterial hypertension; Scleroderma; Sclérodermie; Échocardiographie d’effort