OBJECTIVE: Most of the data about the prevalence of pulmonary arterial hypertension (PAH) are from tertiary centers that are biased toward seeing more severe cases; therefore, the true prevalence of PAH among patients with connective tissue disease is unknown. We sought to determine the point prevalence of undiagnosed PAH in community-based rheumatology practices. METHODS: The study design was a multicenter, prospective and retrospective survey and analysis of clinical cases in 50 community rheumatology practices. We evaluated a total of 909 patients with either scleroderma (systemic sclerosis [SSc]) or mixed connective tissue disease (MCTD). If a subject had not been diagnosed as having PAH, then a new Doppler echocardiogram was obtained to measure cardiac parameters, including estimated right ventricular systolic pressure (ERVSP), and a full review of medical records was done. RESULTS: Of 909 screened patients, 791 were evaluable and completed the study; 669 had not previously been studied for PAH. Of these 669 patients, 89 (13.3%) were found by Doppler echocardiography to have an ERVSP of > or = 40 mm Hg. Of these 89 patients, 82 (92.1%) had SSc and 7 (7.9%) had MCTD. The total prevalence of PAH in the survey was 26.7% (211 of 791 patients, including 122 with known PAH and 89 newly diagnosed as having PAH). Doppler echocardiographic data showed 20 of 89 patients (22.5%) with ERVSP of > or = 50 mm Hg, 20 of 89 patients (22.5%) with increased RV dimension, and 25 of 89 patients (28.1%) with right atrial enlargement. Patients with ERVSP > or = 40 mm Hg had decreased exercise tolerance compared with those with ERVSP <40 mm Hg (27% compared with 9.5%, respectively, were severely symptomatic). CONCLUSION: A significant number of patients with SSc or MCTD (13.3%) followed up in a community rheumatology practice setting have undiagnosed elevated ERVSP consistent with PAH.
OBJECTIVE: Most of the data about the prevalence of pulmonary arterial hypertension (PAH) are from tertiary centers that are biased toward seeing more severe cases; therefore, the true prevalence of PAH among patients with connective tissue disease is unknown. We sought to determine the point prevalence of undiagnosed PAH in community-based rheumatology practices. METHODS: The study design was a multicenter, prospective and retrospective survey and analysis of clinical cases in 50 community rheumatology practices. We evaluated a total of 909 patients with either scleroderma (systemic sclerosis [SSc]) or mixed connective tissue disease (MCTD). If a subject had not been diagnosed as having PAH, then a new Doppler echocardiogram was obtained to measure cardiac parameters, including estimated right ventricular systolic pressure (ERVSP), and a full review of medical records was done. RESULTS: Of 909 screened patients, 791 were evaluable and completed the study; 669 had not previously been studied for PAH. Of these 669 patients, 89 (13.3%) were found by Doppler echocardiography to have an ERVSP of > or = 40 mm Hg. Of these 89 patients, 82 (92.1%) had SSc and 7 (7.9%) had MCTD. The total prevalence of PAH in the survey was 26.7% (211 of 791 patients, including 122 with known PAH and 89 newly diagnosed as having PAH). Doppler echocardiographic data showed 20 of 89 patients (22.5%) with ERVSP of > or = 50 mm Hg, 20 of 89 patients (22.5%) with increased RV dimension, and 25 of 89 patients (28.1%) with right atrial enlargement. Patients with ERVSP > or = 40 mm Hg had decreased exercise tolerance compared with those with ERVSP <40 mm Hg (27% compared with 9.5%, respectively, were severely symptomatic). CONCLUSION: A significant number of patients with SSc or MCTD (13.3%) followed up in a community rheumatology practice setting have undiagnosed elevated ERVSP consistent with PAH.
Authors: Francisco J García-Hernández; María J Castillo-Palma; Carles Tolosa-Vilella; Alfredo Guillén-Del Castillo; Manuel Rubio-Rivas; Mayka Freire; José A Vargas-Hitos; José A Todolí-Parra; Mónica Rodríguez-Carballeira; Gerard Espinosa-Garriga; Dolores Colunga-Argüelles; Norberto Ortego-Centeno; Luis Trapiella-Martínez; María M Rodero-Roldán; Xavier Pla-Salas; Isabel Perales-Fraile; Isaac Pons-Martín Del Campo; Antonio J Chamorro; Rafael A Fernández-de la Puebla Giménez; Ana B Madroñero-Vuelta; Manuel Ruíz-Muñoz; Vicent Fonollosa-Pla; Carmen P Simeón-Aznar Journal: Clin Rheumatol Date: 2018-12-07 Impact factor: 2.980
Authors: Dinesh Khanna; Otylia Kowal-Bielecka; Puja P Khanna; Anna Lapinska; Steven M Asch; Neil Wenger; Kevin K Brown; Philip J Clements; Terry Getzug; Maureen D Mayes; Thomas A Medsger; Ronald Oudiz; Robert Simms; Virginia Steen; Paul Maranian; Daniel E Furst Journal: Clin Exp Rheumatol Date: 2011-05-16 Impact factor: 4.473
Authors: Reda E Girgis; Adaani E Frost; Nicholas S Hill; Evelyn M Horn; David Langleben; Vallerie V McLaughlin; Ronald J Oudiz; Ivan M Robbins; James R Seibold; Shelley Shapiro; Victor F Tapson; Robyn J Barst Journal: Ann Rheum Dis Date: 2007-05-01 Impact factor: 19.103