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2. Left ventricular diastolic dysfunction in systemic sclerosis: Clinical, immunological and survival differences in the Spanish RESCLE registry

Author

González García, Andrés, Fabregate, Martin, Manzano, Luis, Guillén del Castillo, Alfredo, Rubio Rivas, Manuel, Argibay, Ana, Marín Ballvé, Adela, Rodríguez Pintó, Ignasi, Pla Salas, Xavier, Marí-Alfonso, Begoña, Callejas Moraga, Eduardo, Colunga Argüelles, Dolores, Sáez Comet, Luis, González-Echávarri, Cristina, Ortego-Centeno, Norberto, Vargas Hitos, José Antonio, Todolí Parra, José Antonio, Trapiella Martínez, Luis, Herranz Marín, María Teresa, Freire, Mayka, Chamorro, Antonio-J, Perales Fraile, Isabel, Madroñero Vuelta, Ana Belén, Sánchez Trigo, Sabela, Tolosa Vilella, Carles, Fonollosa Pla, Vicent, and Simeón Aznar, Carmen Pilar

Published
2022
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3. Spanish scleroderma risk score (RESCLESCORE) to predict 15-year all-cause mortality in scleroderma patients at the time of diagnosis based on the RESCLE cohort: Derivation and internal validation

Author

Rubio-Rivas, Manuel, Corbella, Xavier, Guillén-del-Castillo, Alfredo, Tolosa Vilella, Carles, Colunga Argüelles, Dolores, Argibay, Ana, Vargas Hitos, José Antonio, Todolí Parra, José Antonio, González-Echávarri, Cristina, Ortego-Centeno, Norberto, Trapiella Martínez, Luis, Rodríguez Carballeira, Mónica, Marín Ballvé, Adela, Pla Salas, Xavier, Perales Fraile, Isabel, Chamorro, Antonio-J, Madroñero Vuelta, Ana Belén, Freire, Mayka, Ruiz Muñoz, Manuel, González García, Andrés, Pons Martín del Campo, Isaac, Sánchez García, María Esther, Bernal Bello, David, Espinosa, Gerard, García Hernández, Francisco José, Sáez Comet, Luis, Ríos Blanco, Juan José, Fernández de la Puebla Giménez, Rafael Ángel, Sánchez Trigo, Sabela, Fonollosa Pla, Vicent, and Simeón Aznar, Carmen Pilar

Published
2020
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4. Hepatobiliary involvement in systemic sclerosis and the cutaneous subsets: Characteristics and survival of patients from the Spanish RESCLE Registry

Author

Marí-Alfonso, Begoña, Simeón-Aznar, Carmen Pilar, Guillén-Del Castillo, Alfredo, Rubio-Rivas, Manuel, Trapiella-Martínez, Luis, Todolí-Parra, José Antonio, Rodríguez Carballeira, Mónica, Marín-Ballvé, Adela, Iniesta-Arandia, Nerea, Colunga-Argüelles, Dolores, Castillo-Palma, María Jesús, Sáez-Comet, Luis, Egurbide-Arberas, María Victoria, Ortego-Centeno, Norberto, Freire, Mayka, Vargas Hitos, José Antonio, Chamorro, Antonio-J, Madroñero-Vuelta, Ana Belen, Perales-Fraile, Isabel, Pla-Salas, Xavier, Fernández-De-La-Puebla, Rafael A., Fonollosa-Pla, Vicent, and Tolosa-Vilella, Carles

Published
2018
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5. Very early and early systemic sclerosis in the Spanish scleroderma Registry (RESCLE) cohort

Author

Trapiella-Martínez, Luis, Díaz-López, José Bernardino, Caminal-Montero, Luis, Tolosa-Vilella, Carles, Guillén-Del Castillo, Alfredo, Colunga-Argüelles, Dolores, Rubio-Rivas, Manuel, Iniesta-Arandia, Nerea, Castillo-Palma, María Jesús, Sáez-Comet, Luis, Egurbide-Arberas, María Victoria, Ortego-Centeno, Norberto, Freire, Mayka, Vargas-Hitos, Jose Antonio, Ríos-Blanco, Juan José, Todolí-Parra, Jose Antonio, Rodríguez-Carballeira, Mónica, Marín-Ballvé, Adela, Chamorro-Fernández, Antonio Javier, Pla-Salas, Xavier, Madroñero-Vuelta, Ana Belén, Ruiz-Muñóz, Manuel, Fonollosa-Pla, Vicent, and Simeón-Aznar, Carmen Pilar

Published
2017
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6. First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study

Author

Rubio-Rivas, Manuel, Corbella, Xavier, Pestaña-Fernández, Melany, Tolosa-Vilella, Carles, Guillen-del Castillo, Alfredo, Colunga-Argüelles, Dolores, Trapiella-Martínez, Luis, Iniesta-Arandia, Nerea, Castillo-Palma, María Jesús, Sáez-Comet, Luis, Egurbide-Arberas, María Victoria, Ortego-Centeno, Norberto, Freire, Mayka, Vargas-Hitos, Jose Antonio, Ríos-Blanco, Juan José, Todolí-Parra, Jose Antonio, Rodríguez-Carballeira, Mónica, Marín-Ballvé, Adela, Segovia-Alonso, Pablo, Pla-Salas, Xavier, Madroñero-Vuelta, Ana Belén, Ruiz-Muñoz, Manuel, Fonollosa-Pla, Vicent, Simeón-Aznar, Carmen Pilar, on behalf of RESCLE investigators, Autoimmune Diseases Study Group (GEAS), Callejas Moraga, E, Calvo, E., Carbonell, C., Castillo, M. J., Chamorro, A. J., Colunga, D., Corbella, X., Egurbide, M. V., Espinosa, G., Fonollosa, V., Freire, M., García Hernández, F. J., González León, R., Guillén del Castillo, A., Iniesta, N., Lorenzo, R., Madroñero, A. B., Marí, B., Marín, A., Ortego-Centeno, N., Pérez Conesa, M., Pestaña, M., Pla, X., Ríos Blanco, J. J., Rodríguez Carballeira, M., Rubio Rivas, M., Ruiz Muñoz, M., Sáez Comet, L., Segovia, P., Simeón, C. P., Soto, A., Tarí, E., Todolí, J. A., Tolosa, C., Trapiella, L., Vargas Hitos, J. A., and Verdejo, G.

Published
2018
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7. Nailfold videocapillaroscopy patterns in systemic sclerosis: implications for cutaneous subsets, disease features and prognostic value for survival

Author

Tolosa-Vilella, Carles, primary, del Mar Rodero-Roldán, Maria, additional, Guillen-del-Castillo, Alfredo, additional, Marín-Ballvé, Adela, additional, Boldova-Aguar, Rafael, additional, Marí-Alfonso, Begoña, additional, Feijoo-Massó, Carlos, additional, Colunga-Argüelles, Dolores, additional, Rubio-Rivas, Manuel, additional, Trapiella-Martínez, Luis, additional, Iniesta-Arandia, Nerea, additional, Callejas-Moraga, Eduardo, additional, García-Hernández, Francisco J., additional, Sáez-Comet, Luis, additional, González-Echávarri, Cristina, additional, Ortego-Centeno, Norberto, additional, Freire, Mayka, additional, Vargas-Hitos, Jose Antonio, additional, Ríos-Blanco, Juan J., additional, Todolí-Parra, Jose Antonio, additional, Rodríguez-Pintó, Ignasi, additional, Chamorro, Antonio-J., additional, Pla-Salas, Xavier, additional, Madroñero-Vuelta, Ana Belén, additional, Ruiz-Muñoz, Manuel, additional, Fonollosa-Pla, Vicent, additional, and Simeón-Aznar, Carmen Pilar, additional

Published
2023
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8. Digital ulcers and cutaneous subsets of systemic sclerosis: Clinical, immunological, nailfold capillaroscopy, and survival differences in the Spanish RESCLE Registry

Author

Tolosa-Vilella, Carles, Morera-Morales, Maria Lluisa, Simeón-Aznar, Carmen Pilar, Marí-Alfonso, Begoña, Colunga-Arguelles, Dolores, Callejas_Rubio, José Luis, Rubio-Rivas, Manuel, Freire-Dapena, Maika, Guillén-del Castillo, Alfredo, Iniesta-Arandia, Nerea, Castillo-Palma, Maria Jesús, Egurbide-Arberas, Marivi, Trapiellla-Martínez, Luis, Vargas-Hitos, José A, Todolí-Parra, José Antonio, Rodriguez-Carballeira, Mónica, Marin-Ballvé, Adela, Pla-Salas, Xavier, Rios-Blanco, Juan José, and Fonollosa-Pla, Vicent

Published
2016
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9. Correction to: First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study

Author

Rubio-Rivas, Manuel, Corbella, Xavier, Pestaña-Fernández, Melany, Tolosa-Vilella, Carles, Castillo, Alfredo Guillen-del, Colunga-Argüelles, Dolores, Trapiella-Martínez, Luis, Iniesta-Arandia, Nerea, Castillo-Palma, María Jesús, Sáez-Comet, Luis, Egurbide-Arberas, María Victoria, Ortego-Centeno, Norberto, Freire, Mayka, Vargas-Hitos, Jose Antonio, Ríos-Blanco, Juan José, Todolí-Parra, Jose Antonio, Rodríguez-Carballeira, Mónica, Marín-Ballvé, Adela, Segovia-Alonso, Pablo, Pla-Salas, Xavier, Madroñero-Vuelta, Ana Belén, Ruiz-Muñoz, Manuel, Fonollosa-Pla, Vicent, Simeón-Aznar, Carmen Pilar, on behalf of RESCLE investigators, and Autoimmune Diseases Study Group (GEAS)

Published
2018
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10. Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension

Author

Guillén-del Castillo, Alfredo, López-Meseguer, Manuel, Fonollosa-Pla, Vicent, Sáez, Berta, Colunga-Argüelles, Dolores, Revilla-López, Eva, Rubio-Rivas, Manuel, Cristo Ropero, María José, Argibay, Ana, Barberà, Joan Albert, Pla-Salas, Xavier, Martínez-Meñaca, Amaya, Madroñero-Vuelta, Ana Belén, Lara Padrón, Antonio, Sáez-Comet, Luis, Domingo Morera, Juan Antonio, González-Echávarri, Cristina, Mombiela, Teresa, Ortego-Centeno, Norberto, Marín González, Manuela, Tolosa-Vilella, Carles, Blanco, Isabel, Escribano-Subias, Pilar, Simeón-Aznar, Carmen Pilar, RESCLE Consortium, REHAP Consortium, Guillén-del Castillo, Alfredo, López-Meseguer, Manuel, Fonollosa-Pla, Vicent, Sáez, Berta, Colunga-Argüelles, Dolores, Revilla-López, Eva, Rubio-Rivas, Manuel, Cristo Ropero, María José, Argibay, Ana, Barberà, Joan Albert, Pla-Salas, Xavier, Martínez-Meñaca, Amaya, Madroñero-Vuelta, Ana Belén, Lara Padrón, Antonio, Sáez-Comet, Luis, Domingo Morera, Juan Antonio, González-Echávarri, Cristina, Mombiela, Teresa, Ortego-Centeno, Norberto, Marín González, Manuela, Tolosa-Vilella, Carles, Blanco, Isabel, Escribano-Subias, Pilar, Simeón-Aznar, Carmen Pilar, RESCLE Consortium, and REHAP Consortium

Abstract

To assess severity markers and outcomes of patients with systemic sclerosis (SSc) with or without pulmonary arterial hypertension (PAH-SSc/non-PAH-SSc), and the impact of interstitial lung disease (ILD) on PAH-SSc. Non-PAH-SSc patients from the Spanish SSc registry and PAH-SSc patients from the Spanish PAH registry were included. A total of 364 PAH-SSc and 1589 non-PAH-SSc patients were included. PAH-SSc patients had worse NYHA-functional class (NYHA-FC), worse forced vital capacity (FVC) (81.2 ± 20.6% vs 93.6 ± 20.6%, P < 0.001), worse tricuspid annular plane systolic excursion (TAPSE) (17.4 ± 5.2 mm vs 19.9 ± 6.7 mm, P < 0.001), higher incidence of pericardial effusion (30% vs 5.2%, P < 0.001) and similar prevalence of ILD (41.8% vs. 44.9%). In individuals with PAH-SSc, ILD was associated with worse hemodynamics and pulmonary function tests (PFT). Up-front combination therapy was used in 59.8% and 61.7% of patients with and without ILD, respectively. Five-year transplant-free survival rate was 41.1% in PAH-SSc patients and 93.9% in non-PAH-SSc patients (P < 0.001). Global survival of PAH-SSc patients was not affected by ILD regardless its severity. The multivariate survival analysis in PAH-SSc patients confirmed age at diagnosis, worse NYHA-FC, increased PVR, reduced DLCO, and lower management with up-front combination therapy as major risk factors. In conclusion, in PAH-SSc cohort risk of death was greatly increased by clinical, PFT, and hemodynamic factors, whereas it was decreased by up-front combination therapy. Concomitant ILD worsened hemodynamics and PFT in PAH-SSc but not survival regardless of FVC impairment.

Published
2022

11. Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension

Author

Guillén-Del-Castillo, Alfredo, Meseguer, Manuel López, Fonollosa-Pla, Vicent, Sáez Giménez, Berta, Colunga-Argüelles, Dolores, Revilla-López, Eva, Rubio-Rivas, Manuel, Ropero, Maria José Cristo, Argibay, Ana, Barberá, Joan Albert, Pla Salas, Xavier, Martínez Meñaca, Amaya, Madroñero Vuelta, Ana Belén, Lara Padrón, Antonio, Sáez Comet, Luis, Domingo Morera, Juan Antonio, González-Echávarri, Cristina, Mombiela, Teresa, Ortego-Centeno, Norberto, Marín González, Manuela, Tolosa-Vilella, Carles, Blanco, Isabel, Escribano Subías, Pilar, Simeón-Aznar, Carmen Pilar, Aurtenetxe Pérez, Águeda, Barrios Garrido-Lestache, Elvira, Bedate Díaz, Pedro, Cifrián, José Manuel, Cristo Ropero, Maria Jose, Dos Subirá, Laura, Elías Hernández, Teresa, García Hernández, Francisco José, Carbonell, Juan Gil, Segovia, Ariadna González, Valverde, Tamara Hermida, Baldomero, Idaira Fámara Hernández, Hernández-González, Ignacio, Huertas, Julia Herrero, Palomares, Luis Jara, Arjona, Josefa Jiménez, Padrón, Antonio Lara, Lázaro-Salvador, María, López-Ramón, Marta, López-Reyes, Raquel, González, Manuela Marín, Meñaca, Amaya Martínez, Etxaniz, Francisco Javier Mazo, Velasco, Virginia Naranjo, Candelera, Remedios Otero, González, Isabel Otero, Lozano, Beatriz Rodríguez, Nieto, María Jesús Rodríguez, Soriano, Joaquín Rueda, Giménez, Berta Sáez, Safont, Belén, Llinas, Ernest Sala, Sebastián, Laura, Cubero, Javier Segovia, Domenech, María Teresa Subirana, Masmiquel, Maria Baldà, Moraga, Eduardo Callejas, Chamorro, Antonio-J., Freire, Mayka, Guillén-del-Castillo, Alfredo, Marín, Maria Teresa Herranz, Vuelta, Ana Belén Madroñero, Ballvé, Adela Marín, Fernández, Melany Pestaña, Salas, Xavier Pla, Pintó, Ignasi Rodríguez, Comet, Luis Sáez, Cervelló, Gonzalo Salvador, Parra, José Antonio Todolí, Trapiella, Luis, Hitos, José Antonio Vargas, Marín, Adela (REHAP Consortium), Institut Català de la Salut, [Guillén-Del-Castillo A, Fonollosa-Pla V, Simeón-Aznar CP] Unitat de Malalties Autoimmunes, Servei de Medicina Interna, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Meseguer ML, Revilla-López E] Servei de Pneumologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Sáez Giménez B] Servei de Pneumologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Departament de Fisiologia, Universitat Autònoma de Barcelona, Bellaterra, Spain. [Colunga-Argüelles D] Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain, and Vall d'Hebron Barcelona Hospital Campus

Subjects
Respiratory Tract Diseases::Lung Diseases::Lung Diseases, Interstitial [DISEASES], enfermedades respiratorias::enfermedades pulmonares::hipertensión pulmonar [ENFERMEDADES], Hypertension, Pulmonary, Impacte, enfermedades de la piel y tejido conjuntivo::enfermedades del tejido conjuntivo::esclerodermia sistémica [ENFERMEDADES], Pulmonary hypertension, Respiratory Tract Diseases::Lung Diseases::Hypertension, Pulmonary [DISEASES], Anàlisi de supervivència (Biometria), Pulmonary diseases, polycyclic compounds, Survival analysis (Biometry), Humans, Familial Primary Pulmonary Hypertension, skin and connective tissue diseases, Hipertensió pulmonar, Pulmonary Arterial Hypertension, Respiratory tract diseases, Hipotensió arterial, Multidisciplinary, Scleroderma, Systemic, integumentary system, respiratory system, respiratory tract diseases, Malalties dels pulmons, Pulmons - Malalties, Esclerosi sistemàtica progressiva - Tractament, Impact, Scleroderma (Disease), enfermedades respiratorias::enfermedades pulmonares::enfermedades pulmonares intersticiales [ENFERMEDADES], Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic [DISEASES], Càncer de pulmó, Systemic sclerosis, Lung cancer, Esclerodèrmia, Lung Diseases, Interstitial
Abstract

To assess severity markers and outcomes of patients with systemic sclerosis (SSc) with or without pulmonary arterial hypertension (PAH-SSc/non-PAH-SSc), and the impact of interstitial lung disease (ILD) on PAH-SSc. Non-PAH-SSc patients from the Spanish SSc registry and PAH-SSc patients from the Spanish PAH registry were included. A total of 364 PAH-SSc and 1589 non-PAH-SSc patients were included. PAH-SSc patients had worse NYHA-functional class (NYHA-FC), worse forced vital capacity (FVC) (81.2 ± 20.6% vs 93.6 ± 20.6%, P

Published
2022

12. Corrigendum to ‘Spanish scleroderma risk score (RESCLESCORE) to predict 15-year all-cause mortality in scleroderma patients at the time of diagnosis based on the RESCLE cohort: Derivation and internal validation’ [AUTREV 19-5 (2020) 102507]

Author

Rubio-Rivas, Manuel, Corbella, Xavier, Guillén-del-Castillo, Alfredo, Tolosa Vilella, Carles, Colunga Argüelles, Dolores, Argibay, Ana, Vargas Hitos, José Antonio, Todolí Parra, José Antonio, González-Echávarri, Cristina, Ortego-Centeno, Norberto, Trapiella Martínez, Luis, Rodríguez Carballeira, Mónica, Marín Ballvé, Adela, Pla Salas, Xavier, Perales Fraile, Isabel, Chamorro, Antonio-J, Madroñero Vuelta, Ana Belén, Freire, Mayka, Ruiz Muñoz, Manuel, González García, Andrés, Pons Martín del Campo, Isaac, Sánchez García, María Esther, Bernal Bello, David, Espinosa, Gerard, García Hernández, Francisco José, Sáez Comet, Luis, Ríos Blanco, Juan José, Fernández de la Puebla Giménez, Rafael Ángel, Sánchez Trigo, Sabela, Fonollosa Pla, Vicent, and Simeón Aznar, Carmen Pilar

Published
2021
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16. Pulmonary hypertension in Spanish patients with systemic sclerosis. Data from the RESCLE registry

Author

García-Hernández, Francisco José, Castillo-Palma, María J., Tolosa-Vilella, Carles, Guillén-del Castillo, Alfredo, Rubio-Rivas, Manuel, Freire, Mayka, Vargas-Hitos, José A., Todolí-Parra, José A., Rodríguez-Carballeira, Mónica, Espinosa, Gerard, Colunga-Argüelles, Dolores, Ortego-Centeno, Norberto, Trapiella-Martínez, Luis, Rodero-Roldán, María M., Pla-Salas, Xavier, Perales-Fraile, Isabel, Pons-Martín del Campo, Isaac, Chamorro, Antonio J., Fernández-de la Puebla Giménez, Rafael A., Madroñero-Vuelta, Ana Belén, Ruíz-Muñoz, Manuel, Fonollosa-Pla, Vicent, Simeón-Aznar, Carmen Pilar, García-Hernández, Francisco José, Castillo-Palma, María J., Tolosa-Vilella, Carles, Guillén-del Castillo, Alfredo, Rubio-Rivas, Manuel, Freire, Mayka, Vargas-Hitos, José A., Todolí-Parra, José A., Rodríguez-Carballeira, Mónica, Espinosa, Gerard, Colunga-Argüelles, Dolores, Ortego-Centeno, Norberto, Trapiella-Martínez, Luis, Rodero-Roldán, María M., Pla-Salas, Xavier, Perales-Fraile, Isabel, Pons-Martín del Campo, Isaac, Chamorro, Antonio J., Fernández-de la Puebla Giménez, Rafael A., Madroñero-Vuelta, Ana Belén, Ruíz-Muñoz, Manuel, Fonollosa-Pla, Vicent, and Simeón-Aznar, Carmen Pilar

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.

Published
2019

17. Longterm Efficacy and Safety of Monotherapy versus Combination Therapy in Systemic Sclerosis–associated Pulmonary Arterial Hypertension: A Retrospective RESCLE Registry Study

Author

Pestaña-Fernández, Melani, primary, Rubio-Rivas, Manuel, additional, Tolosa-Vilella, Carles, additional, Guillén-Del-Castillo, Alfredo, additional, Freire, Mayka, additional, Vargas-Hitos, Jose Antonio, additional, Todolí-Parra, Jose Antonio, additional, Rodríguez-Carballeira, Mónica, additional, Marín-Ballvé, Adela, additional, Espinosa, Gerard, additional, Colunga-Argüelles, Dolores, additional, Ortego-Centeno, Norberto, additional, Trapiella-Martínez, Luis, additional, Carbonell-Muñoz, Cristina, additional, Pla-Salas, Xavier, additional, Perales-Fraile, Isabel, additional, Corbella, Xavier, additional, Fonollosa-Pla, Vicent, additional, and Simeón-Aznar, Carmen Pilar, additional

Published
2019
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18. The incidence rate of pulmonary arterial hypertension and scleroderma renal crisis in systemic sclerosis patients with digital ulcers on endothelin antagonist receptors (ERAs) and phosphodiesterase-5 inhibitors (PDE5i).

Author

Pestaña-Fernández, Melani, Rubio-Rivas, Manuel, Tolosa-Vilella, Carles, Guillén-Del-Castillo, Alfredo, Colunga-Argüelles, Dolores, Argibay, Ana, Marí-Alfonso, Begoña, Marín-Ballvé, Adela, Pla-Salas, Xavier, Chamorro, Antonio-J, Castro-Salomó, Antoni, Madroñero-Vuelta, Ana Belén, Sánchez-García, María Esther, Sáez-Comet, Luis, González-Echávarri, Cristina, Ortego-Centeno, Norberto, Vargas-Hitos, José Antonio, Todolí-Parra, José Antonio, Trapiella-Martínez, Luis, and Lledó, Gema María

Subjects
KIDNEY disease diagnosis, CELL receptors, CONFIDENCE intervals, REPORTING of diseases, ENDOTHELINS, FINGERS, KIDNEY diseases, LONGITUDINAL method, MEDICAL records, PULMONARY hypertension, SYSTEMIC scleroderma, TIME, DISEASE incidence, DISEASE prevalence, RETROSPECTIVE studies, PHOSPHODIESTERASE inhibitors, DESCRIPTIVE statistics, ACQUISITION of data methodology, SKIN ulcers, CHEMICAL inhibitors, DISEASE complications
Abstract

Introduction Endothelin antagonist receptors (ERAs) and phosphodiesterase-5 inhibitors (PDE5i) are beneficial in pulmonary arterial hypertension (PAH) and digital ulcers (DU) and prevent from DU recurrences. Our study aimed to determine the difference in the incidence rate of PAH and scleroderma renal crisis (SRC) in patients with SSc and DU (SSc-DU) under ERAs/PDE5i or without treatment. Methods We conducted a retrospective cohort study including SSc-DU patients from the Spanish Scleroderma Registry (RESCLE). The primary outcome was the incidence rate of PAH and SRC in patients under ERAs/PDE5i or not. Results Some 544 patients out of 1817 (29.9%) in the RESCLE database had DU, 221 (40.6%) under ERAs/PDE5i and 323 (59.4%) not. The incidence rate (95% CI) difference between patients under treatment or not under did not reach statistical significance in PAH [−0.1 (−4.8, 4.69), P  = 0.988] or in SRC [0.7 (−2.2, 3.7), P  = 0.620]. However, the time from the first DU to the diagnosis of SRC was delayed in treated patients [mean (s. d.) 7.6 (5.8) years vs 2.9  (5.3); P  = 0.021]. The dcSSc subset was more prevalent in the treatment group (36 vs 26%; P  = 0.018), along with anti-topoisomerase I antibodies (34 vs 18%; P  < 0.001) and tendon friction rubs (12 vs 6%; P  = 0.038), whereas the lcSSc subset was more prevalent in the no-treatment group (57 vs 66%; P  = 0.031) along with ACA (37 vs 46%; P  = 0.031). Conclusion There was no difference in the incidence rate of PAH and SRC between groups. However, treatment with ERAs and/or PDE5i appeared to delay the occurrence of SRC. [ABSTRACT FROM AUTHOR]

Published
2021
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19. Influence of antibody profile in clinical features and prognosis in a cohort of Spanish patients with systemic sclerosis

Author

Iniesta Arandia, Nerea, Simeon-Aznar, Carmen Pilar, Guillen Del Castillo, Alfredo, Colunga Arguelles, Dolores, Rubio-Rivas, Manuel, Trapiella Martinez, Luis, Garcia Hernandez, Francisco Jose, Saez Comet, Luis, Egurbide Arberas, Maria Victoria, Ortego-Centeno, Norberto, Freire, Mayka, Mari Alfonso, Begona, Vargas Hitos, Jose Antonio, Juan Jose Rios Blanco, Todoli Parra, Jose Antonio, Rodriguez-Carballeira, Monica, Marin Ballve, Adela, Chamorro Fernandez, Antonio Javier, Pla Salas, Xavier, Madronero Vuelta, Ana Belen, Ruiz Munoz, Manuel, Fonollosa Pla, Vicent, Espinosa, Gerard, and Rescle Investigators, Autoimmune Diseases Study Group

Subjects
antibody profile, integumentary system, anti-centromere antibody, systemic sclerosis, anti-topoisomerase antibody, anti-nuclear antibodies, skin and connective tissue diseases, anti-RNA-polymerase III antibody
Abstract

Objective. To assess the clinical manifestations and prognosis of Spanish patients with systemic sclerosis (SSc) according to their immunological profile. Methods. From the Spanish Scleroderma Study Group or RESCLE (Registro de ESCLErodermia as Spanish nomenclature) Registry we selected those patients in which anti-centromere (ACA), anti-topoisomerase I (ATA), and anti-RNA polymerase III (ARA) antibodies had been determined, and a single positivity for each SSc specific antibody was detected. Demographic, clinical, laboratory, and survival data were compared according to the serologic status of these antibodies. Results. Overall, 209 SSc patients were included. In 128 (61%) patients ACA was the only positive antibody, 46 (22%) were only positive for ATA, and 35 (17%) for ARA. Of note, the three groups were mutually exclusive. In univariate analysis, patients with ACA presented more frequently limited cutaneous SSc (lcSSc) (p

Published
2017

20. Longterm Efficacy and Safety of Monotherapy versus Combination Therapy in Systemic Sclerosis-associated Pulmonary Arterial Hypertension: A Retrospective RESCLE Registry Study.

Author

Pestaña-Fernández, Melani, Rubio-Rivas, Manuel, Tolosa-Vilella, Carles, Guillén-Del-Castillo, Alfredo, Freire, Mayka, Vargas-Hitos, Jose Antonio, Todolí-Parra, Jose Antonio, Rodríguez-Carballeira, Mónica, Marín-Ballvé, Adela, Espinosa, Gerard, Colunga-Argüelles, Dolores, Ortego-Centeno, Norberto, Trapiella-Martínez, Luis, Carbonell-Muñoz, Cristina, Pla-Salas, Xavier, Perales-Fraile, Isabel, Corbella, Xavier, Fonollosa-Pla, Vicent, Simeón-Azna, Carmen Pilar, and Simeón-Aznar, Carmen Pilar

Published
2020
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21. Pulmonary hypertension in Spanish patients with systemic sclerosis. Data from the RESCLE registry.

Author

García-Hernández, Francisco J., Castillo-Palma, María J., Tolosa-Vilella, Carles, Guillén-del Castillo, Alfredo, Rubio-Rivas, Manuel, Freire, Mayka, Vargas-Hitos, José A., Todolí-Parra, José A., Rodríguez-Carballeira, Mónica, Espinosa-Garriga, Gerard, Colunga-Argüelles, Dolores, Ortego-Centeno, Norberto, Trapiella-Martínez, Luis, Rodero-Roldán, María M., Pla-Salas, Xavier, Perales-Fraile, Isabel, Pons-Martín del Campo, Isaac, Chamorro, Antonio J., Fernández-de la Puebla Giménez, Rafael A., and Madroñero-Vuelta, Ana B.

Subjects
SYSTEMIC scleroderma, PULMONARY hypertension, INTERSTITIAL lung diseases, PULMONARY artery, HEART diseases
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. [ABSTRACT FROM AUTHOR]

Published
2019
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22. IMAGEN DE LA SEMANA

Author

Pla Salas, Xavier, primary, Tolosa Vilella, Carles, additional, Soler, Alfons, additional, and Oristrell Salvà, Joaquim, additional

Published
2008
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25. Evaluating the functioning of a rapid diagnostic unit of patients with Constitutional syndrome: a retrospective study

Author

Depoorter Ruelle, Isabel, Universitat de Girona. Facultat de Medicina, Pla Salas, Xavier, and Castells Cervelló, Xavier

Subjects
Aprimament, Weight loss, Diagnòstic, Astènia, Asthenia, Symptoms, Diagnosis, Símptomes, Hospital Universitari de Vic
Abstract

BACKGROUND: Constitutional syndrome (CS) is a group of non-specific symptoms (asthenia, anorexia and involuntary weight loss) frequently found in the setting of a wide range of diseases, amongst which cancer is the most important. Those in charge of carrying out the diagnostic approach of patients with CS are the Rapid diagnostic units. This unit’s main objectives are to reduce diagnostic delays at the same time of preventing unnecessary admission, since they are ambulatory care units. PURPOSE: To evaluate the functioning of the Rapid diagnostic unit of Hospital Universitari de Vic in terms of agility and rapidity of the CS evaluation and detect alterations in order to improve them. And to know the frequency of the diseases responsible for the constitutional syndrome. METHODS: We analysed the medical history of all the patients with constitutional syndrome attended at the Rapid Diagnostic Unit in two years (from May 2015 to April 2017). RESULTS: a total of 88 patients were referred to the unit from different health facilities (specially from primary health care and emergency department). The unit took a median of 3 days to visit patients with CS since they were referred and a median of 3 more days to achieve the diagnosis. The median time to get complementary tests performed and the medical report obtained was 5,5 days. The most frequent cause of CS was cancer (37,5% of all the diagnosis), followed by psychiatric diseases (22,7% of all the diagnosis). The most frequent type of cancer were the digestive neoplasms, and amongst all the cancer, 60,6% presented metastasis at the moment of the diagnosis. CONCLUSION: The rapid diagnostic unit of Hospital Universitari de Vic is efficient in speeding up the diagnostic process of patients with CS. In the case of potentially serious diseases, as cancer, the unit has the agility to accelerate the diagnostic procedures as much as possible, so the best therapeutic approach can be conducted in these patients. The most common diseases responsible for constitutional syndrome were malignant neoplasms, which most of them had already advanced stages. Cancer was followed, in decreasing frequency, by psychiatric disorders and organic but non-neoplastic diseases as digestive diseases

Published
2018

26. Effectiveness of a medical polyvalent Day Care Hospital: evaluation of the reduction of hospitalizations and days of hospitable stay: a before-and-after study

Author

Baldà Masmiquel, Maria, Universitat de Girona. Facultat de Medicina, Pla Salas, Xavier, and Lucchetti, Gianni Enrico

Subjects
Hospitals -- Pacients, Day hospitals, Hospitals de dia, Hospital patients
Abstract

Day Care Hospitals (DCH) are medical healthcare units where are attended patients that need diagnostic methods or treatments that last a few hours of attention, but that do not need to be hospitalized. “Hospital Universitari de Vic” has recently initiated a new project to improve the medical attention of the patients and to clear up the contact between professionals. One of the pillars of the project is the opening of a medic DCH. There are few studies that evaluate the medical care and the profitability of DCH in Spain, but they have generally concluded that DCHs provide a reduction of pressure on the Emergency Room and on the Hospitalization Floor Justification: This study is realized to evaluate the effectiveness of the opening of the DCH in Vic with the reduction of hospitalizations and the reduction of the time that patients are hospitalized. A lower number of hospitalizations implies a lower number of complications related to the hospitalizations, a lower sanitary expense and an improvement on the optimizations of the resources. This study will also provide data that could be interesting for the sanitary centers that want to open a DCH AIMS: To describe the characteristics of the chronic patients (HF, chronic pneumopathies and LC) that are attended at the DCH. To determine the effect on the number of hospitalizations and hospitable stays of the patients with chronic advanced diseases (HF, CP, LC) with the functioning of the Day Care Hospital. To determine the real impact in the general number of medical hospitalizations in the HUV Methods: This study has been realized with a before-and-after study, with the opening of the DCH in Vic as the main intervention. It has been realized in the medical DCH of the HUV with a sample of 78 subjects, which have been collected with a consecutive no-probabilistic sampling of the patients that have been attended in the DCH of the HUV Demographic, clinical, diagnostic and treatment data of the patients have been collected. The number of hospitalizations, hospitable stays and attentions to the Emergency Room has also been collected during a period of time that has been equal between the after and the before period of the DCH opening. The hospitalizations and the hospitable stays during the months of DCH functioning have been compared with the same period of the 2 previous years (2013 and 2014). The statistical analysis has been realized with the SPSS statistical package v21.0 Results: The most frequent attended disease was heart failure, with the 50% of prevalence in the DCH. It has been detected a borderline statistical significance of the reduction of medical hospitalizations compared to the previous two years, when the DCH wasn’t working Conclusion: The medical Day Care Hospital in Vic has reduced the number of hospitalizations and hospitable stays of the attended patients during the six months that has been working

Published
2015

27. Serodiscordant patients with systemic sclerosis: when antibody does not correspond to skin involvement.

Author

Iniesta Arandia N, Espinosa G, Tolosa Vilella C, Guillén Del Castillo A, Rubio Rivas M, Freire M, Vargas Hitos JA, Todolí Parra JA, Rodríguez Carballeira M, Marín Ballvé A, Colunga Argüelles D, González de Echávarri Pérez de Heredia C, Ortego-Centeno N, Trapiella Martínez L, Pla Salas X, Chamorro AJ, Perales Fraile I, Ruiz Muñoz M, Fernández de la Puebla Giménez RÁ, Madroñero Vuelta AB, Pons Martín Del Campo I, Jiménez Pérez de Heredia I, González García A, Fonollosa Pla V, and Simeón Aznar CP

Subjects
Autoantibodies, Humans, Hypertension, Pulmonary, Lung Diseases, Interstitial, Scleroderma, Diffuse, Scleroderma, Systemic
Abstract

Objectives: Diffuse cutaneous systemic sclerosis (dcSSc) is associated with anti-topoisomerase (ATA) whereas limited cutaneous (lcSSc) and sine scleroderma (ssSSc) are mainly associated with anti-centromere antibody (ACA). Serodiscordant patients were defined as lcSSc subjects with ATA, dcSSc with ACA, and ssSSc with ATA. The aim of the present study was to compare the clinical manifestations and prognosis between serodiscordant patients and their counterparts (those with lcSSc with ACA, dcSSc with ATA and ssSSc with ACA, respectively)., Methods: From the Spanish Scleroderma Registry we selected those patients for whom skin involvement (dcSSc, lcSSc or ssSSc) was detailed at baseline and last visit and ACA and ATA had been determined. Demographic, clinical characteristics, and survival data were compared according to the antibody status., Results: The whole cohort comprised 901 patients and six mutually exclusive groups were defined: lcSScACA in 511 (57%) patients, lcSScATA group in 87 (10%), dcSScATA group in 172 (19%), dcSScACA group in 21 (2%), ssSScACA group in 92 (10%), and ssSScATA group in 18 (2%) patients, respectively. Interstitial lung disease (ILD) and severe ILD were more frequent in patients with dcSScATA than in those with dcSScACA. Conversely, the prevalence of isolated pulmonary hypertension (without ILD) was higher in those with dcSScACA (15% vs. 2%; p=0.018). No differences were found regarding survival when comparing serodiscordant patients with the seroconcordants patients., Conclusions: In our cohort, the prevalence of serodiscordant SSc patients was low. They differed from their counterparts in some clinical manifestations. The management of patients with SSc should be guided by both serology and cutaneous subtype.

Published
2020

28. Clinical and epidemiological differences between men and women with systemic sclerosis: a study in a Spanish systemic sclerosis cohort and literature review.

Author

Freire M, Rivera A, Sopeña B, Tolosa Vilella C, Guillén-Del Castillo A, Colunga Argüelles D, Callejas Rubio JL, Rubio Rivas M, Trapiella Martínez L, Todolí Parra JA, Rodríguez Carballeira M, Iniesta Arandia N, García Hernández FJ, Egurbide Arberas MV, Sáez Comet L, Vargas Hitos JA, Ríos Blanco JJ, Marín Ballvé A, Pla Salas X, Madroñero Vuelta AB, Ruiz Muñoz M, Fonollosa Pla V, and Simeón Aznar CP

Subjects
Cause of Death, Cohort Studies, Female, Humans, Male, Prognosis, Prospective Studies, Scleroderma, Systemic complications, Scleroderma, Systemic epidemiology, Sex Characteristics, Scleroderma, Systemic mortality
Abstract

Objectives: The low overall prevalence of systemic sclerosis (SSc) and the low proportion of male patients have resulted in a scarcity of studies assessing sex differences in Ssc patients, and contradictory results have often been show among those studies that have been performed., Methods: A prospective study was conducted with the Spanish RESCLE register to analyse the influence of gender on survival of SSc patients., Results: In total, 1506 SSc patients (1341 women, 165 men) were recruited from 21 centres. Older age at onset (OR 1.02), shorter time from onset to diagnosis (OR 0.96), smoking (OR 2.57), interstitial lung disease (ILD) (OR 1.58), less predisposition to sicca syndrome and to antinuclear antibody positivity (OR 0.29 and 0.43, respectively), and higher compliance with the ACR 1980 criteria (OR 1.79) were independently associated with the male sex. During follow-up, 30.4% of men versus 14.6% of women died (p<0.001). Survival at 10 years from the onset of symptoms was 75.3% for men and 92.9% for women (p<0.001), and the difference remained after selecting only SSc-related deaths (85.6% vs. 96.1%, p<0.001). The mortality predictive factors were diffuse SSc (OR 2.26), ILD (OR 1.82), digital ulcers (OR 1.38), tendon friction rubs (OR 1.74), male sex (OR 1.53), increased age at onset (OR 1.13) and isolated PH (considering only deaths from diagnosis), both in the overall (OR 3.63) and female cohorts (OR 3.97). The same risk factors were observed in the male cohort, except for isolated PH and ILD., Conclusions: The present study confirms the existence of epidemiological, clinical, laboratory and prognostic gender differences in systemic sclerosis patients.

Published
2017

29. Influence of antibody profile in clinical features and prognosis in a cohort of Spanish patients with systemic sclerosis.

Author

Iniesta Arandia N, Simeón-Aznar CP, Guillén Del Castillo A, Colunga Argüelles D, Rubio-Rivas M, Trapiella Martínez L, García Hernández FJ, Sáez Comet L, Egurbide Arberas MV, Ortego-Centeno N, Freire M, Marí Alfonso B, Vargas Hitos JA, Ríos Blanco JJ, Todolí Parra JA, Rodríguez-Carballeira M, Marín Ballvé A, Chamorro Fernández AJ, Pla Salas X, Madroñero Vuelta AB, Ruiz Muñoz M, Fonollosa Pla V, and Espinosa G

Subjects
Adult, Aged, Centromere immunology, Cohort Studies, DNA Topoisomerases, Type I immunology, Female, Humans, Lung Diseases, Interstitial epidemiology, Male, Middle Aged, Prognosis, RNA Polymerase III immunology, Scleroderma, Systemic complications, Scleroderma, Systemic mortality, Autoantibodies analysis, Scleroderma, Systemic immunology
Abstract

Objectives: To assess the clinical manifestations and prognosis of Spanish patients with systemic sclerosis (SSc) according to their immunological profile., Methods: From the Spanish Scleroderma Study Group or RESCLE (Registro de ESCLErodermia as Spanish nomenclature) Registry we selected those patients in which anti-centromere (ACA), anti-topoisomerase I (ATA), and anti-RNA polymerase III (ARA) antibodies had been determined, and a single positivity for each SSc specific antibody was detected. Demographic, clinical, laboratory, and survival data were compared according to the serologic status of these antibodies., Results: Overall, 209 SSc patients were included. In 128 (61%) patients ACA was the only positive antibody, 46 (22%) were only positive for ATA, and 35 (17%) for ARA. Of note, the three groups were mutually exclusive. In univariate analysis, patients with ACA presented more frequently limited cutaneous SSc (lcSSc) (p<0.001), whereas diffuse cutaneous SSc (dcSSc) was the most frequent subtype in patients with ATA (54%) and ARA (62%) (both p<0.001). Positive patients for ARA showed the highest prevalence of joint involvement (p<0.001) and those from ATA group had a higher prevalence of interstitial lung disease (ILD) (p<0.001). Scleroderma renal crisis was more frequent in the ARA group (p<0.001). In multivariate analysis, ACA were associated with female gender and were protective for dcSSc and ILD. ATA were found to be protective for lcSSc and they were independently associated with interstitial reticular pattern. ARA positivity was independently associated with dcSSc. We did not find differences in mortality between the three groups., Conclusions: In Spanish SSc patients, the presence of SSc specific antibodies conferred a distinctive clinical profile.

Published
2017

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