title: Pulmonary hypertension in systemic sclerosis: prevalence, incidence and predictive factors in a large multicentric Italian cohort.
abstract: OBJECTIVE
  This paper aims to investigate the prevalence, the incidence of pulmonary hypertension (PH) and its subtypes in Italian patients with systemic sclerosis (SSc) and to characterise features associated with and predictive of development of PH.
  
  
  METHODS
  Eight-hundred and sixty-seven consecutive SSc patients recruited at 4 Italian centres were enrolled. At admission, all patients underwent a careful history, physical examination, EKG, lung high resolution computed tomography (HRCT), pulmonary function tests, B-mode echocardiography and right heart catheterisation (RHC), if indicated. Patients were then visited every 6-12 months. A RHC was performed in those patients in whom PH was suspected for the presence of pre-specified criteria.
  
  
  RESULTS
  Among the 212 patients in whom it was suspected, PH was confirmed by RHC in 69 patients. On 31st December 2010, the point prevalence of P-arterial-H(PAH) and PH associated with interstitial lung disease (PH-ILD) was 3.7% and 1.4%, respectively; that of postcapillary PH was 1.3%. The estimated incidence rates of PH and PAH were respectively 1.85/100 patient-years and 1.02/100 patient-years. Multivariate analysis indicated that diffusing lung capacity for CO (DLCO) ≤55% (HR 4.45, 95%CI 2.24-8.83; p<0.001) and sPAP >40 mmHg (HR 18.03, 95%CI 9.01-36.06; p<0.001) were associated with an increased risk to develop PAH. SystolicPAP >40 mmHg resulted the only predictor of PH-ILD (HR 5.17, 95%CI 1.37-19.5; p=0.018) and post-capillary PH (HR 7.91, 95%CI 1.88-33.1; p=0.005) development.
  
  
  CONCLUSIONS
  Our study confirms a lower prevalence of PH in Italy compared to Anglo-Saxon cohorts. We also identified patients at high risk, who should be carefully monitored.
authors.affiliation: Rheumatology Unit, Second University of Naples, Naples, Italy. michele_iudici@hotmail.com
authors.forename: Michele
authors.initials: M
authors.lastname: Iudici
authors.forename: Veronica
authors.initials: V
authors.lastname: Codullo
authors.forename: Dilia
authors.initials: D
authors.lastname: Giuggioli
authors.forename: Valeria
authors.initials: V
authors.lastname: Riccieri
authors.forename: Giovanna
authors.initials: G
authors.lastname: Cuomo
authors.forename: Silvia
authors.initials: S
authors.lastname: Breda
authors.forename: Andreina
authors.initials: A
authors.lastname: Manfredi
authors.forename: Nicoletta
authors.initials: N
authors.lastname: Iannace
authors.forename: Michele
authors.initials: M
authors.lastname: D'Alto
authors.forename: Stefano
authors.initials: S
authors.lastname: Ghio
authors.forename: Rosario
authors.initials: R
authors.lastname: Rossi
authors.forename: Carmine Dario
authors.initials: CD
authors.lastname: Vizza
authors.forename: Roberto
authors.initials: R
authors.lastname: Caporali
authors.forename: Guido
authors.initials: G
authors.lastname: Valesini
authors.forename: Clodoveo
authors.initials: C
authors.lastname: Ferri
authors.forename: Gabriele
authors.initials: G
authors.lastname: Valentini
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D006328:Cardiac Catheterization; D002648:Child; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000453:epidemiology*; D015994:Incidence; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D015995:Prevalence; D012129:Respiratory Function Tests; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000453:epidemiology*; D055815:Young Adult