Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies

Jérôme Avouac, Paolo Airò, Christophe Meune, Lorenzo Beretta, Philippe Dieude, Paola Caramaschi, Kiet Tiev, Susanna Cappelli, Elisabeth Diot, Alessandra Vacca, Jean Luc Cracowski, Jean Sibilia, André Kahan, Marco Matucci-Cerinic, Yannick Allanore

Research output: Contribution to journalArticlepeer-review


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. The Journal of Rheumatology

Original languageEnglish
Pages (from-to)2290-2298
Number of pages9
JournalJournal of Rheumatology
Issue number11
Publication statusPublished - Nov 2010


  • Catheterization
  • Metaanalysis
  • Prevalence
  • Pulmonary hypertension
  • Systemic sclerosis

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Immunology and Allergy


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