PT  - JOURNAL ARTICLE
AU  - JÉRÔME AVOUAC
AU  - PAOLO AIRÒ
AU  - CHRISTOPHE MEUNE
AU  - LORENZO BERETTA
AU  - PHILIPPE DIEUDE
AU  - PAOLA CARAMASCHI
AU  - KIET TIEV
AU  - SUSANNA CAPPELLI
AU  - ELISABETH DIOT
AU  - ALESSANDRA VACCA
AU  - JEAN-LUC CRACOWSKI
AU  - JEAN SIBILIA
AU  - ANDRÉ KAHAN
AU  - MARCO MATUCCI-CERINIC
AU  - YANNICK ALLANORE
TI  - Prevalence of Pulmonary Hypertension in Systemic Sclerosis in European Caucasians and Metaanalysis of 5 Studies
AID  - 10.3899/jrheum.100245
DP  - 2010 Nov 01
TA  - The Journal of Rheumatology
PG  - 2290--2298
VI  - 37
IP  - 11
4099  - http://www.jrheum.org/content/37/11/2290.short
4100  - http://www.jrheum.org/content/37/11/2290.full
SO  - J Rheumatol2010 Nov 01; 37
AB  - 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. Conclusion. 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.