The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France

Eric Hachulla, Pascal De Groote, Virginie Gressin, Jean Sibilia, Elisabeth Diot, Patrick Carpentier, Luc Mouthon, Pierre Yves Hatron, Patrick Jego, Yannick Allanore, Kiet Phong Tiev, Christian Agard, Anne Cosnes, Daniela Cirstea, Joël Constans, Dominique Farge, Jean François Viallard, Jean Robert Harle, Frédéric Patat, Bernard ImbertAndré Kahan, Jean Cabane, Pierre Clerson, Loïc Guillevin, Marc Humbert, Marie Sylvie Doutre, Thierry Schaeverbeke, Marc Alain Billès, Patricia Réant, Claire Dromer, Philippe Gosse, Philippe Lemetayer, Antoine-Béclère, Olivier Sitbon, Xavier Jaïs, Abdul Monem Hamid, Vincent Ioos, Gérald Simonneau, Cécile Roiron, Jean Luc Cracowski, Carole Saurier, Muriel Salvant, Carole Schwebel, Christophe Pison, David Launay, Viviane Queyrel, Marc Lambert, Sandrine Morell-Dubois, Hilaire Charlanne, Nicolas Lamblin, Jacques Ninet, Fadi Jamal, Geneviève Dérumeaux, Jean Yves Bayle, Fréderique Retornaz, Gilbert Habib, Sébastien Renard, Martine Reynaud-Gaubert, Jean Dominique De Korwin, Christine Suty-Selton, François Chabot, Mohamed Hamidou, Jean Pierre Gueffet, Patrice Guérin, Erwan Bressolette, Alain Haloun, Thomas Papo, Olivier Lidove, Catherine Picard-Dahan, David Messika-Zeitoun, Laure Cabanes, Christian Spaulding, Alice Berezné, Camille Francès, Sellim Trad, Emmanuel Molinari, Anne Claude Koeger, Dominique De Zuttere, Stéphanie Ederhy, Nadjib Hammoudi, Mohamed Ziani, Isabelle Lazareth, Ulrique Michon-Pasturel, Yara Antakly-Hanon, Jacques Serfati, Suzanne Ménasché, Jacqueline Chevrant-Breton, Marcel Laurent, Hélène Petit, Ari Chaouat, Daniel Adoue, Nathalie Blot-Souletie, Bruno Degano, Laurent Machet, Christian Marchal, Cédric Giraudeau

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Objective. An algorithm for the detection of pulmonary arterial hypertension (PAH), based on the presence of dyspnea and the findings of Doppler echocardiographic evaluation of the velocity of tricuspid regurgitation (VTR) and right-sided heart catheterization (RHC), which was applied in a large multicenter systemic sclerosis (SSc) population, estimated the prevalence of PAH to be 7.85%. The aim of this observational study was to investigate the incidence of PAH and pulmonary hypertension (PH) during a 3-year followup of patients from the same cohort (the ItinérAIR-Sclérodermie Study). Methods. Patients with SSc and without evidence of PAH underwent evaluation for dyspnea and VTR at study entry and during subsequent visits. Patients in whom PAH was suspected because of a VTR of 2.8-3.0 meters/second and unexplained dyspnea or a VTR of >3.0 meters/second underwent RHC to confirm the diagnosis. Results. A total of 384 patients were followed up for a mean ± SD of 41.03 ± 5.66 months (median 40.92 months). At baseline, 86.7% of the patients were women, and the mean ± SD age of the patients was 53.1 ± 12.0 years. The mean ± SD duration of SSc at study entry was 8.7 ± 7.6 years. After RHC, PAH was diagnosed in 8 patients, postcapillary PH in 8 patients, and PH associated with severe pulmonary fibrosis in 2 patients. The incidence of PAH was estimated to be 0.61 cases per.

Original languageEnglish
Pages (from-to)1831-1839
Number of pages9
JournalArthritis and Rheumatism
Issue number6
StatePublished - Jun 2009


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