Francisco J García-Hernández1, María J Castillo-Palma2, Carles Tolosa-Vilella3, Alfredo Guillén-Del Castillo4, Manuel Rubio-Rivas5, Mayka Freire6, José A Vargas-Hitos7, José A Todolí-Parra8, Mónica Rodríguez-Carballeira9, Gerard Espinosa-Garriga10, Dolores Colunga-Argüelles11, Norberto Ortego-Centeno12, Luis Trapiella-Martínez13, María M Rodero-Roldán14, Xavier Pla-Salas15, Isabel Perales-Fraile16, Isaac Pons-Martín Del Campo17, Antonio J Chamorro18, Rafael A Fernández-de la Puebla Giménez19, Ana B Madroñero-Vuelta20, Manuel Ruíz-Muñoz21, Vicent Fonollosa-Pla4, Carmen P Simeón-Aznar4. 1. Department of Internal Medicine, Hospital Universitario Virgen del Rocío, Seville, Spain. pacolageno@gmail.com. 2. Department of Internal Medicine, Hospital Universitario Virgen del Rocío, Seville, Spain. 3. Department of Internal Medicine, Corporación Sanitaria Universitaria Parc Taulí, Sabadell, Spain. 4. Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain. 5. Department of Internal Medicine, Hospital Universitario de Bellvitge, L'Hospitalet de Llobregat, Spain. 6. Systemic Autoimmune Diseases and Thrombosis Unit, Department of Internal Medicine, Complejo Hospitalario Universitario de Vigo, Pontevedra, Galicia, Spain. 7. Department of Internal Medicine, Hospital Universitario Virgen de las Nieves, Granada, Spain. 8. Department of Internal Medicine, Hospital Universitario y Politécnico La Fe, Valencia, Spain. 9. Department of Internal Medicine, Hospital Universitario Mútua Terrassa, Barcelona, Spain. 10. Systemic Autoimmune Diseases Unit, Instituto Clínic de Medicina y Dermatología, Hospital Universitario Clínic, Barcelona, Spain. 11. Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain. 12. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Campus de la Salud, Complejo Universitario de Granada, Granada, Spain. 13. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain. 14. Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain. 15. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Consorci Hospitalari de Vic, Barcelona, Spain. 16. Department of Internal Medicine, Hospital Universitario Rey Juan Carlos, Móstoles, Spain. 17. Autoimmune Diseases Unit, Department of Internal Medicine, Hospital de Igualada, Barcelona, Spain. 18. Department of Internal Medicine, Complejo Asistencial Universitario de Salamanca, Salamanca, Spain. 19. Department of Internal Medicine, Hospital Universitario Reina Sofía, Córdoba, Spain. 20. Department of Internal Medicine, Hospital General San Jorge, Huesca, Spain. 21. Department of Internal Medicine, Hospital Universitario Fundación Alcorcón, Madrid, Spain.
Abstract
INTRODUCTION: Our objective was to evaluate the pulmonary hypertension (PH) data for Spanish patients with systemic sclerosis (SSc), define the PH types and determine the associated factors. METHOD: Descriptive study of PH-related data from the multicentre RESCLE registry. Estimated systolic pulmonary artery pressure (esPAP), measured via echocardiogram was considered elevated if ≥ 35 mmHg. Left heart disease (LHD) and interstitial lung disease (ILD) were identified. When performed, data from right heart catheterisation (RHC) were collected. RESULTS: esPAP was elevated in 350 of 808 patients (43.3%). One hundred and forty-four patients (17.8%) were considered to have PH (88 via RHC and the rest due to elevated esPAP along with evidence of significant LHD or ILD): PAH 3.7%, secondary to ILD 8.3%, secondary to LHD 2.8% and unclassified 3%. Prevalence of elevated esPAP was greater in diffuse SSc (dSSc) than in limited scleroderma (lSSc) (50.5 vs. 42.2%, p 0.046). In the group with elevated esPAP, a lower prevalence of anti-centromere antibodies (41.9% vs. 52.3%, p 0.006) and a greater prevalence of anti-topoisomerase-1 antibodies (ATA) (25.1% vs. 18.6%, p 0.04) were observed compared to the group with normal esPAP. Patients with elevated esPAP had a lower rate of digital ulcers (50.6% vs. 60.2%, p 0.007) and esophageal involvement (83.6% vs. 88.7%, p 0.07) and higher rate of renal crisis (4.6% vs. 1.8%, p 0.066). CONCLUSIONS: Prevalence of PAH was lower than expected (3.7%). Probability of having elevated esPAP was higher among patients with dSSc and among those with ATA.
INTRODUCTION: Our objective was to evaluate the pulmonary hypertension (PH) data for Spanish patients with systemic sclerosis (SSc), define the PH types and determine the associated factors. METHOD: Descriptive study of PH-related data from the multicentre RESCLE registry. Estimated systolic pulmonary artery pressure (esPAP), measured via echocardiogram was considered elevated if ≥ 35 mmHg. Left heart disease (LHD) and interstitial lung disease (ILD) were identified. When performed, data from right heart catheterisation (RHC) were collected. RESULTS: esPAP was elevated in 350 of 808 patients (43.3%). One hundred and forty-four patients (17.8%) were considered to have PH (88 via RHC and the rest due to elevated esPAP along with evidence of significant LHD or ILD): PAH 3.7%, secondary to ILD 8.3%, secondary to LHD 2.8% and unclassified 3%. Prevalence of elevated esPAP was greater in diffuse SSc (dSSc) than in limited scleroderma (lSSc) (50.5 vs. 42.2%, p 0.046). In the group with elevated esPAP, a lower prevalence of anti-centromere antibodies (41.9% vs. 52.3%, p 0.006) and a greater prevalence of anti-topoisomerase-1 antibodies (ATA) (25.1% vs. 18.6%, p 0.04) were observed compared to the group with normal esPAP. Patients with elevated esPAP had a lower rate of digital ulcers (50.6% vs. 60.2%, p 0.007) and esophageal involvement (83.6% vs. 88.7%, p 0.07) and higher rate of renal crisis (4.6% vs. 1.8%, p 0.066). CONCLUSIONS: Prevalence of PAH was lower than expected (3.7%). Probability of having elevated esPAP was higher among patients with dSSc and among those with ATA.
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Authors: C P Simeón-Aznar; V Fonollosa-Plá; Carles Tolosa-Vilella; G Espinosa-Garriga; M Campillo-Grau; M Ramos-Casals; F J García-Hernández; M J Castillo-Palma; J Sánchez-Román; J L Callejas-Rubio; N Ortego-Centeno; M V Egurbide-Arberas; L Trapiellla-Martínez; L Caminal-Montero; L Sáez-Comet; J Velilla-Marco; M T Camps-García; E de Ramón-Garrido; E M Esteban-Marcos; L Pallarés-Ferreres; N Navarrete-Navarrete; J A Vargas-Hitos; R Gómez de la Torre; G Salvador-Cervello; J J Rios-Blanco; M Vilardell-Tarrés Journal: Medicine (Baltimore) Date: 2015-10 Impact factor: 1.817