TY - JOUR
T1 - Pulmonary hypertension in systemic sclerosis
T2 - Prevalence, incidence and predictive factors in a large multicentric italian cohort
AU - Iudici, Michele
AU - Codullo, Veronica
AU - Giuggioli, Dilia
AU - Riccieri, Valeria
AU - Cuomo, Giovanna
AU - Breda, Silvia
AU - Manfredi, Andreina
AU - Iannace, Nicoletta
AU - D'Alto, Michele
AU - Ghio, Stefano
AU - Rossi, Rosario
AU - Vizza, Carmine Dario
AU - Caporali, Roberto
AU - Valesini, Guido
AU - Ferri, Clodoveo
AU - Valentini, Gabriele
PY - 2013
Y1 - 2013
N2 - Objectives. 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; p40 mmHg (HR 18.03, 95%CI 9.01-36.06; p40 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. Conclusion. 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.
AB - Objectives. 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; p40 mmHg (HR 18.03, 95%CI 9.01-36.06; p40 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. Conclusion. 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.
KW - Epidemiology
KW - Pulmonary hypertension
KW - Systemic sclerosis
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M3 - Article
C2 - 23910607
AN - SCOPUS:84883747206
VL - 31
JO - Clinical and Experimental Rheumatology
JF - Clinical and Experimental Rheumatology
SN - 0392-856X
IS - SUPPL.76
ER -