116 results on '"Vilella, Carles"'
Search Results
2. Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension
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Guillén-Del-Castillo, Alfredo, Meseguer, Manuel López, Fonollosa-Pla, Vicent, Giménez, Berta Sáez, Colunga-Argüelles, Dolores, Revilla-López, Eva, Rubio-Rivas, Manuel, Ropero, Maria Jose Cristo, Argibay, Ana, Barberá, Joan Albert, Salas, Xavier Pla, Meñaca, Amaya Martínez, Vuelta, Ana Belén Madroñero, Padrón, Antonio Lara, Comet, Luis Sáez, Morera, Juan Antonio Domingo, González-Echávarri, Cristina, Mombiela, Teresa, Ortego-Centeno, Norberto, González, Manuela Marín, Tolosa-Vilella, Carles, Blanco, Isabel, Subías, Pilar Escribano, and Simeón-Aznar, Carmen Pilar
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- 2022
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3. Author Correction: Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension
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Guillén-Del-Castillo, Alfredo, Meseguer, Manuel López, Fonollosa-Pla, Vicent, Giménez, Berta Sáez, Colunga-Argüelles, Dolores, Revilla-López, Eva, Rubio-Rivas, Manuel, Ropero, Maria Jose Cristo, Argibay, Ana, Barberá, Joan Albert, Salas, Xavier Pla, Meñaca, Amaya Martínez, Vuelta, Ana Belén Madroñero, Padrón, Antonio Lara, Comet, Luis Sáez, Morera, Juan Antonio Domingo, González-Echávarri, Cristina, Mombiela, Teresa, Ortego-Centeno, Norberto, González, Manuela Marín, Tolosa-Vilella, Carles, Blanco, Isabel, Subías, Pilar Escribano, and Simeón-Aznar, Carmen Pilar
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- 2022
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4. Anti–Polymyositis/Scl Antibodies in Systemic Sclerosis: Clinical Associations in a Multicentric Spanish Cohort and Review of the Literature
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Iniesta Arandia, Nerea, Espinosa, Gerard, Guillén del Castillo, Alfredo, Tolosa-Vilella, Carles, Colunga-Argüelles, Dolores, González de Echávarri Pérez de Heredia, Cristina, Lledó, Gema M., Comet, Luis Sáez, Ortego-Centeno, Norberto, Vargas Hito, José Antonio, Rubio-Rivas, Manuel, Freire, Mayka, Ríos-Blanco, Juan José, Rodríguez-Carballeira, Mónica, Trapiella-Martínez, Luis, Fonollosa-Pla, Vicent, and Simeón-Aznar, Carmen Pilar
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- 2022
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5. Nailfold videocapillaroscopy patterns in systemic sclerosis: implications for cutaneous subsets, disease features and prognostic value for survival
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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
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- 2023
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6. First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study
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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.
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- 2018
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7. Prognostic implications of extra-hepatic clinical manifestations, autoimmunity and microscopic nail capillaroscopy in patients with primary biliary cirrhosis
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Marí-Alfonso, Begoña, Amengual-Guedan, María José, Vergara-Gómez, Mercè, Simeón-Aznar, Carmen Pilar, Fonollosa-Plà, Vicente, Jove-Buxeda, Esther, Oliva-Morera, Juan, and Tolosa-Vilella, Carles
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- 2016
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8. Quantitative videocapillaroscopy correlates with functional respiratory parameters: a clue for vasculopathy as a pathogenic mechanism for lung injury in systemic sclerosis
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Guillén-Del-Castillo, Alfredo, Simeón-Aznar, Carmen Pilar, Callejas-Moraga, Eduardo L., Tolosa-Vilella, Carles, Alonso-Vila, Serafín, Fonollosa-Pla, Vicente, and Selva-O’Callaghan, Albert
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- 2018
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9. Left ventricular diastolic dysfunction in systemic sclerosis: Clinical, immunological and survival differences in the Spanish RESCLE registry
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González García, Andrés, primary, Fabregate, Martin, additional, Manzano, Luis, additional, Guillén del Castillo, Alfredo, additional, Rubio Rivas, Manuel, additional, Argibay, Ana, additional, Marín Ballvé, Adela, additional, Rodríguez Pintó, Ignasi, additional, Pla Salas, Xavier, additional, Marí-Alfonso, Begoña, additional, Callejas Moraga, Eduardo, additional, Colunga Argüelles, Dolores, additional, Sáez Comet, Luis, additional, González-Echávarri, Cristina, additional, Ortego-Centeno, Norberto, additional, Vargas Hitos, José Antonio, additional, Todolí Parra, José Antonio, additional, Trapiella Martínez, Luis, additional, Herranz Marín, María Teresa, additional, Freire, Mayka, additional, Chamorro, Antonio-J, additional, Perales Fraile, Isabel, additional, Madroñero Vuelta, Ana Belén, additional, Sánchez Trigo, Sabela, additional, Tolosa Vilella, Carles, additional, Fonollosa Pla, Vicent, additional, and Simeón Aznar, Carmen Pilar, additional
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- 2022
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10. Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension
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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.
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- 2022
11. Correction to: First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study
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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)
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- 2018
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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]
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Rubio-Rivas, Manuel, primary, Corbella, Xavier, additional, Guillén-del-Castillo, Alfredo, additional, Tolosa Vilella, Carles, additional, Colunga Argüelles, Dolores, additional, Argibay, Ana, additional, Vargas Hitos, José Antonio, additional, Todolí Parra, José Antonio, additional, González-Echávarri, Cristina, additional, Ortego-Centeno, Norberto, additional, Trapiella Martínez, Luis, additional, Rodríguez Carballeira, Mónica, additional, Marín Ballvé, Adela, additional, Pla Salas, Xavier, additional, Perales Fraile, Isabel, additional, Chamorro, Antonio-J, additional, Madroñero Vuelta, Ana Belén, additional, Freire, Mayka, additional, Ruiz Muñoz, Manuel, additional, González García, Andrés, additional, Pons Martín del Campo, Isaac, additional, Sánchez García, María Esther, additional, Bernal Bello, David, additional, Espinosa, Gerard, additional, García Hernández, Francisco José, additional, Sáez Comet, Luis, additional, Ríos Blanco, Juan José, additional, Fernández de la Puebla Giménez, Rafael Ángel, additional, Sánchez Trigo, Sabela, additional, Fonollosa Pla, Vicent, additional, and Simeón Aznar, Carmen Pilar, additional
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- 2021
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13. Effect of mycophenolate sodium in scleroderma-related interstitial lung disease
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Simeón-Aznar, Carmen Pilar, Fonollosa-Plá, Vicent, Tolosa-Vilella, Carles, Selva-O’Callaghan, Albert, Solans-Laqué, Roser, and Vilardell-Tarrés, Miquel
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- 2011
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14. Anti–Polymyositis/Scl Antibodies in Systemic Sclerosis
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Iniesta Arandia, Nerea, primary, Espinosa, Gerard, additional, Guillén del Castillo, Alfredo, additional, Tolosa-Vilella, Carles, additional, Colunga-Argüelles, Dolores, additional, González de Echávarri Pérez de Heredia, Cristina, additional, Lledó, Gema M., additional, Comet, Luis Sáez, additional, Ortego-Centeno, Norberto, additional, Vargas Hito, José Antonio, additional, Rubio-Rivas, Manuel, additional, Freire, Mayka, additional, Ríos-Blanco, Juan José, additional, Rodríguez-Carballeira, Mónica, additional, Trapiella-Martínez, Luis, additional, Fonollosa-Pla, Vicent, additional, and Simeón-Aznar, Carmen Pilar, additional
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- 2021
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15. Low Rate High Frequency Data Transmission from Very Remote Sensors
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Bergada, Pau, primary, Alsina-Pages, RosaMa, additional, Vilella, Carles, additional, and Ramon, Joan, additional
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- 2012
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16. 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
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Rubio-Rivas, Manuel, primary, Corbella, Xavier, additional, Guillén-del-Castillo, Alfredo, additional, Tolosa Vilella, Carles, additional, Colunga Argüelles, Dolores, additional, Argibay, Ana, additional, Vargas Hitos, José Antonio, additional, Todolí Parra, José Antonio, additional, González-Echávarri, Cristina, additional, Ortego-Centeno, Norberto, additional, Trapiella Martínez, Luis, additional, Rodríguez Carballeira, Mónica, additional, Marín Ballvé, Adela, additional, Pla Salas, Xavier, additional, Perales Fraile, Isabel, additional, Chamorro, Antonio-J, additional, Madroñero Vuelta, Ana Belén, additional, Freire, Mayka, additional, Ruiz Muñoz, Manuel, additional, González García, Andrés, additional, Pons Martín del Campo, Isaac, additional, Sánchez García, María Esther, additional, Bernal Bello, David, additional, Espinosa, Gerard, additional, García Hernández, Francisco José, additional, Sáez Comet, Luis, additional, Ríos Blanco, Juan José, additional, Fernández de la Puebla Giménez, Rafael Ángel, additional, Sánchez Trigo, Sabela, additional, Fonollosa Pla, Vicent, additional, and Simeón Aznar, Carmen Pilar, additional
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- 2020
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17. Esclerosis sistémica y enfermedad hepato-biliar : estudio bidireccional de la relación entre la esclerosis sistémica y la enfermedad hepato-biliar
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Tolosa Vilella, Carles, Fonollosa Pla, Vicent, Marí Alfonso, Begoña, Universitat Autònoma de Barcelona. Departament de Medicina, Tolosa Vilella, Carles, Fonollosa Pla, Vicent, Marí Alfonso, Begoña, and Universitat Autònoma de Barcelona. Departament de Medicina
- Abstract
La esclerosis sistémica (ES) es una enfermedad autoinmune sistémica (EAS) que puede afectar a la mayoría de órganos internos mediante la acumulación de colágeno en los tejidos y/o desarrollo de una vasculopatía obliterante, en un entorno de disfunción autoinmune. Aunque la disfunción hepato-biliar (DHB) no es un hallazgo infrecuente en la ES, su presencia no se considera característica de la enfermedad y se han publicado pocos estudios que valoren la relación bidireccional entre la ES y la DHB, en particular autoinmune órgano-específica. El primer artículo de esta Tesis doctoral es un estudio observacional multicéntrico Español de 1572 pacientes con ES y como objetivos establece la prevalencia de DHB en pacientes con ES y enumera las causas que son predominantemente autoinmunes órgano-específicas, como la colangitis biliar primaria (CBP) en 4.3% y la hepatitis autoinmune en 1.2% y CBP sin anticuerpos antimitocondria en 0.4%.. La presencia de DHB en pacientes con ES se asocia independientemente a un menor riesgo de padecer una ES cutánea difusa y a una mayor prevalencia de calcinosis cutánea, disfunción diastólica de ventrículo izquierdo, síndrome seco y positividad de los anticuerpos anticentrómero (ACA). La presencia de DHB no influyó en la supervivencia. Según los subtipos cutáneos, la DHB se asocia a: 1) en pacientes con ES cutánea limitada, a un mayor tiempo desde el inicio de la ES hasta su diagnóstico y a una mayor prevalencia de síndrome seco y presencia de ACA; 2) en pacientes con ES sine escleroderma, a una mayor prevalencia de síndrome seco, y 3) en pacientes con ES cutánea difusa, no se identificó ningún rasgo diferencial con respecto a los pacientes sin DHB. El segundo artículo es un estudio observacional que reclutó una cohorte de 62 pacientes con CBP a los que se aplicó un protocolo de estudio dirigido a determinar la prevalencia de EAS asociada a esta entidad. Los resultados muestran que la prevalencia de EAS en pacientes con CBP es del 35.4%. La ES e, Systemic sclerosis (SSc) is a chronic autoimmune systemic disease (ASD) that may affect most internal organs caused by an accumulation of collagen in tissues and/or the development of obliterative vasculopathy in the context of autoimmune dysfunction. Although liver disturbance is not a rare finding in SSc, its presence is not considered characteristic of this disease and few studies have assessed the bidirectional relationship between SSc and hepatobiliary dysfunction (HBD), especially addressed to investigating organ-specific diseases. The first article of this doctoral thesis is a Spanish multicenter observational study including 1572 patients with SSc that establishes the prevalence of HBD in patients with SSc at 7.5% and the main causes as organ-specific autoimmune diseases, such as primary biliary cholangitis (PBC) at 4.3%, autoimmune hepatitis at 1.2% and PBC without antimitochondrial antibodies at 0.4%. The presence of HBD in patients with SSc is independently associated with a lower risk of developing diffuse cutaneous SSc and a greater prevalence of cutaneous calcinosis, diastolic dysfunction of left ventricle, Sicca syndrome and positive anticentromere antibodies. The presence of HBD did not influence survival. According to cutaneous subtypes, HBD is associated with: 1) In patients with limited cutaneous SS, a longer time-to-diagnosis since SSc onset to its diagnosis and a greater prevalence of sicca syndrome and presence of anticentromere antibodies; 2) In patients with SSc sine scleroderma, a greater prevalence of sicca syndrome, and 3) In patients with diffuse cutaneous SSc, no distinguishing feature was identified compared to patients without HBD. The second article is an observational study recruiting a cohort of 62 patients with PBC included in a study protocol to determine the prevalence of ASD associated to this entity. The results obtained show that the prevalence of ASD in patients with PBC is 35.4%. SSc is the most prevalent condition (21%), es
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- 2019
18. Pulmonary hypertension in Spanish patients with systemic sclerosis. Data from the RESCLE registry
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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
19. Longterm Efficacy and Safety of Monotherapy versus Combination Therapy in Systemic Sclerosis–associated Pulmonary Arterial Hypertension: A Retrospective RESCLE Registry Study
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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
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- 2019
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20. Olanzapine-induced agranulocytosis: A case report and review of the literature
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Tolosa-Vilella, Carles, Ruiz-Ripoll, Ada, Mari-Alfonso, Begoña, and Naval-Sendra, Elsa
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- 2002
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21. 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).
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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]
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- 2021
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22. Association of a rare variant of the TNFSF13B gene with susceptibility to Rheumatoid Arthritis and Systemic Lupus Erythematosus
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Junta de Andalucía, Redes Temáticas de Investigación Cooperativa en Salud (España), Instituto de Salud Carlos III, Ministerio de Economía y Competitividad (España), Carreira, Patricia E. [0000-0001-8279-3806], González-Serna, David, Ortiz-Fernández, Lourdes, Vargas, Sofía, García, Antonio, Raya, Enrique, Fernández-Gutiérrez, Benjamín, López-Longo, Francisco Javier, Balsa, Alejandro, González-Álvaro, Isidoro, Narváez, Javier, Gómez-Vaquero, C., Sabio, José Mario, García-Portales, Rosa, González-Escribano, María Francisca, Tolosa-Vilella, Carles, Carreira, Patricia E., Kiemeney, Lambertus A., Coenen, Marieke J. H., Witte, Torsten, Schneider, Matthias, González-Gay, M. A., Martín, Javier, Junta de Andalucía, Redes Temáticas de Investigación Cooperativa en Salud (España), Instituto de Salud Carlos III, Ministerio de Economía y Competitividad (España), Carreira, Patricia E. [0000-0001-8279-3806], González-Serna, David, Ortiz-Fernández, Lourdes, Vargas, Sofía, García, Antonio, Raya, Enrique, Fernández-Gutiérrez, Benjamín, López-Longo, Francisco Javier, Balsa, Alejandro, González-Álvaro, Isidoro, Narváez, Javier, Gómez-Vaquero, C., Sabio, José Mario, García-Portales, Rosa, González-Escribano, María Francisca, Tolosa-Vilella, Carles, Carreira, Patricia E., Kiemeney, Lambertus A., Coenen, Marieke J. H., Witte, Torsten, Schneider, Matthias, González-Gay, M. A., and Martín, Javier
- Abstract
A rare variant (BAFF-var) of the tumor necrosis factor superfamily 13b (TNFSF13B) gene has been recently associated with multiple sclerosis (MS) and systemic lupus erythematosus (SLE). The aim of this study was to investigate the association between TNFSF13B BAFF-var and susceptibility to rheumatoid arthritis (RA) and replicate that association in SLE. 6,218 RA patients, 2,575 SLE patients and 4,403 healthy controls from three different countries were included in the study. TNFSF13B BAFF-var was genotyped using TaqMan allelic discrimination assay. PLINK software was used for statistical analyses. TNFSF13B BAFF-var was significantly associated with RA (p = 0.015, OR = 1.21, 95% CI = 1.03–1.41) in the Spanish cohort. A trend of association was observed in the Dutch (p = 0.115) and German (p = 0.228) RA cohorts. A meta-analysis of the three RA cohorts included in this study revealed a statistically significant association (p = 0.002, OR = 1.24, 95% CI = 1.10–1.38). In addition, TNFSF13B BAFF-var was significantly associated with SLE in the Spanish (p = 0.001, OR = 1.41, 95% CI = 1.14–1.74) and the German cohorts (p = 0.030, OR = 1.86, 95% CI = 1.05–3.28), with a statistically significant p-value obtained in the meta-analysis (p = 0.0002, OR = 1.46, 95% CI = 1.09–2.32). The results obtained confirm the known association of TNFSF13B BAFF-var with SLE and, for the first time, demonstrate that this variant contributes to susceptibility to RA.
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- 2018
23. Longterm Efficacy and Safety of Monotherapy versus Combination Therapy in Systemic Sclerosis-associated Pulmonary Arterial Hypertension: A Retrospective RESCLE Registry Study.
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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
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- 2020
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24. Hepatobiliary involvement in systemic sclerosis and the cutaneous subsets: Characteristics and survival of patients from the Spanish RESCLE Registry
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Marí-Alfonso, Begoña, primary, Simeón-Aznar, Carmen Pilar, additional, Guillén-Del Castillo, Alfredo, additional, Rubio-Rivas, Manuel, additional, Trapiella-Martínez, Luis, additional, Todolí-Parra, José Antonio, additional, Rodríguez Carballeira, Mónica, additional, Marín-Ballvé, Adela, additional, Iniesta-Arandia, Nerea, additional, Colunga-Argüelles, Dolores, additional, Castillo-Palma, María Jesús, additional, Sáez-Comet, Luis, additional, Egurbide-Arberas, María Victoria, additional, Ortego-Centeno, Norberto, additional, Freire, Mayka, additional, Vargas Hitos, José Antonio, additional, Chamorro, Antonio-J, additional, Madroñero-Vuelta, Ana Belen, additional, Perales-Fraile, Isabel, additional, Pla-Salas, Xavier, additional, Fernández-De-La-Puebla, Rafael A., additional, Fonollosa-Pla, Vicent, additional, and Tolosa-Vilella, Carles, additional
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- 2018
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25. Crisis paratiroidea: hipercalcemia como urgencia médica
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Medina Ortega, Laura, primary, Feijoo Massó, Carlos, additional, and Tolosa Vilella, Carles, additional
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- 2018
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26. Parathyroid crisis: Hypercalcemia as a medical emergency
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Medina Ortega, Laura, primary, Feijoo Massó, Carlos, additional, and Tolosa Vilella, Carles, additional
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- 2018
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27. Very early and early systemic sclerosis in the Spanish scleroderma Registry (RESCLE) cohort
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Trapiella-Martínez, Luis, primary, Díaz-López, José Bernardino, additional, Caminal-Montero, Luis, additional, Tolosa-Vilella, Carles, additional, Guillén-Del Castillo, Alfredo, additional, Colunga-Argüelles, Dolores, additional, Rubio-Rivas, Manuel, additional, Iniesta-Arandia, Nerea, additional, Castillo-Palma, María Jesús, additional, Sáez-Comet, Luis, additional, Egurbide-Arberas, María Victoria, additional, Ortego-Centeno, Norberto, additional, Freire, Mayka, additional, Vargas-Hitos, Jose Antonio, additional, Ríos-Blanco, Juan José, additional, Todolí-Parra, Jose Antonio, additional, Rodríguez-Carballeira, Mónica, additional, Marín-Ballvé, Adela, additional, Chamorro-Fernández, Antonio Javier, additional, Pla-Salas, Xavier, additional, Madroñero-Vuelta, Ana Belén, additional, Ruiz-Muñóz, Manuel, additional, Fonollosa-Pla, Vicent, additional, and Simeón-Aznar, Carmen Pilar, additional
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- 2017
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28. Registry of the Spanish Network for Systemic Sclerosis
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Simeón-Aznar, C.P., Fonollosa-Plá, V., Tolosa-Vilella, Carles, Espinosa-Garriga, G., Campillo-Grau, M., Ramos-Casals, M., García-Hernández, F.J., Castillo-Palma, M.J., Sánchez-Román, J., Callejas-Rubio, J.L., Ortego-Centeno, N., Egurbide-Arberas, M.V., Trapiellla-Martínez, L., Caminal-Montero, L., Sáez-Comet, L., Velilla-Marco, J., Camps-García, M.T., de Ramón-Garrido, E., Esteban-Marcos, E.M., Pallarés-Ferreres, L., Navarrete-Navarrete, N., Vargas-Hitos, J.A., de la Torre, R. Gómez, Salvador-Cervello, G., Rios-Blanco, J.J., and Vilardell-Tarrés, M.
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Adult ,Male ,Scleroderma, Systemic ,Observational Study ,Middle Aged ,Risk Factors ,Spain ,Cause of Death ,Humans ,Female ,Registries ,Research Article ,Aged ,Retrospective Studies - Abstract
Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan–Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, P
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- 2015
29. Registry of the Spanish network for systemic sclerosis: Survival, prognostic factors, and causes of death
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Simeón Aznar, Carmen Pilar, Fonollosa Pla, Vicent, Tolosa Vilella, Carles, Espinosa Garriga, Gerard, Campillo Grau, M., Ramos Casals, Manuel, García Hernández, F.J., Castillo Palma, María Jesús, Sánchez Román, J., Callejas Rubio, José Luis, Ortego Centeno, Norberto, Egurbide Arberas, María Victoria, Trapiella Martínez, Luis, Caminal Montero, L., Sáez Comet, Luis, Velilla Marco, J., Camps García, María Teresa, Ramón Garrido, E . de, Esteban Marcos, E.M., Pallarés Ferreres, Lucio, Navarrete Navarrete, N., Vargas Hitos, José Antonio, Gómez de la Torre, Ricardo, Salvador Cervelló, Gonzalo, Ríos Blanco, Juan José, Vilardell Tarrés, M., Spanish Scleroderma Study Group (SSSG), Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI), Systemic Autoimmune Diseases Group (GEAS), Spanish Scleroderma Study Group (SSSG), Spanish Society of Internal Medicine, Spain, [Simeón-Aznar,CP, Fonollosa-Plá,V, Vilardell-Tarrés,M] Department of Internal Medicine, Hospital Valld’Hebron. [Tolosa-Vilella,C] Department of Internal Medicine, Hospital Parc Taulí, Sabadell. [Espinosa-Garriga,G, Campillo-Grau,M] Department of Autoimmune Diseases, Hospital Clinic. [Campillo-Grau,M] Laboratori of Computacional Medicine, Bioestatistics Unit, Universitat Autònoma de Barcelona, Bellaterra, Barcelona. [García-Hernández,FJ, Castillo-Palma,MJ, Sánchez-Román,J] Unit of Connective Tissue Diseases, Department of Internal Medicine, Hospital Virgen del Rocio, Sevilla. [Callejas-Rubio,JL, Ortego-Centeno,N] Unit of Autoimmune Systemic Diseases, Department of Internal Medicine, Hospital Clínico San Cecilio, Granada. [Egurbide-Arberas,MV] Department of Internal Medicine, Hospital de Cruces, Galdakano, Bilbao. [Trapiellla-Martínez,L] Department of Internal Medicine, Hospital de Cabueñes, Gijón. [Caminal-Montero,L] Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo. [Sáez-Comet,L, Velilla-Marco,J] Department of Internal Medicine, Hospital Miguel Servet, Zaragoza. [Camps-García,MT, de Ramón-Garrido,E] Department of Internal Medicine, Hospital Carlos Haya, Málaga. [Esteban-Marcos,EM, Pallarés-Ferreres,L]Department of Internal Medicine, Hospital Son Espases, Palma de Mallorca. [Navarrete-Navarrete,N, Vargas-Hitos,JA] Department of Internal Medicine, Hospital Virgen de las Nieves, Granada. [Gómez de la Torre,R] Department of Internal Medicine, Hospital San Agustín, Avilés. [Salvador-Cervello,G] Department of Internal Medicine, Hospital La Fe, Valencia. [Rios-Blanco,JJ] Department of Internal Medicine, Hospital La Paz, Madrid, and Universitat de Barcelona
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Male ,Multivariate analysis ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Models, Statistical::Proportional Hazards Models [Medical Subject Headings] ,sistema de registros ,Diseases::Respiratory Tract Diseases::Lung Diseases::Hypertension, Pulmonary [Medical Subject Headings] ,Autoimmune diseases ,humanos ,Scleroderma Renal Crisis ,España ,Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::Ulcer [Medical Subject Headings] ,Scleroderma ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Probability::Risk::Risk Factors [Medical Subject Headings] ,Diseases::Respiratory Tract Diseases::Lung Diseases::Lung Diseases, Interstitial [Medical Subject Headings] ,Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic [Medical Subject Headings] ,Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans [Medical Subject Headings] ,Úlcera ,Risk Factors ,Cause of Death ,Registries ,Masculino ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Morbidity::Prevalence [Medical Subject Headings] ,Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic::Scleroderma, Limited [Medical Subject Headings] ,mediana edad ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Mortality::Cause of Death [Medical Subject Headings] ,Cause of death ,Esclerodermia limitada ,anciano ,Esclerodermia difusa ,Malalties autoimmunitàries ,Interstitial lung disease ,Pronóstico ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Questionnaires [Medical Subject Headings] ,General Medicine ,adulto ,Middle Aged ,Modelos de riesgos proporcionales ,Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic::Scleroderma, Limited::CREST Syndrome [Medical Subject Headings] ,Humanos ,Encuestas y cuestionarios ,Female ,Factores de riesgo ,Adult ,medicine.medical_specialty ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Analysis of Variance::Multivariate Analysis [Medical Subject Headings] ,Check Tags::Male [Medical Subject Headings] ,causas de muerte ,Internal medicine ,Progresión de la enfermedad ,Análisis multivariante ,medicine ,factores de riesgo ,Tasa de supervivencia ,Humans ,Espanya ,Survival rate ,Aged ,Retrospective Studies ,Geographical Locations::Geographic Locations::Europe::Spain [Medical Subject Headings] ,Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic::Scleroderma, Diffuse [Medical Subject Headings] ,Scleroderma, Systemic ,Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::Disease Attributes::Disease Progression [Medical Subject Headings] ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Mortality::Survival Rate [Medical Subject Headings] ,business.industry ,estudios retrospectivos ,Retrospective cohort study ,medicine.disease ,Pulmonary hypertension ,Surgery ,Causas de muerte ,Scleroderma (Disease) ,Enfermedades pulmonares Intersticiales ,Spain ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Prognosis [Medical Subject Headings] ,Esclerodèrmia ,business ,Síndrome CREST ,Prevalencia ,Hipertensión pulmonar - Abstract
Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan-Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, P
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- 2015
30. Pulmonary hypertension in Spanish patients with systemic sclerosis. Data from the RESCLE registry.
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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.
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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]
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- 2019
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31. Digital ulcers and cutaneous subsets of systemic sclerosis: Clinical, immunological, nailfold capillaroscopy, and survival differences in the Spanish RESCLE Registry
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Tolosa-Vilella, Carles, primary, Morera-Morales, Maria Lluisa, additional, Simeón-Aznar, Carmen Pilar, additional, Marí-Alfonso, Begoña, additional, Colunga-Arguelles, Dolores, additional, Callejas_Rubio, José Luis, additional, Rubio-Rivas, Manuel, additional, Freire-Dapena, Maika, additional, Guillén-del Castillo, Alfredo, additional, Iniesta-Arandia, Nerea, additional, Castillo-Palma, Maria Jesús, additional, Egurbide-Arberas, Marivi, additional, Trapiellla-Martínez, Luis, additional, Vargas-Hitos, José A, additional, Todolí-Parra, José Antonio, additional, Rodriguez-Carballeira, Mónica, additional, Marin-Ballvé, Adela, additional, Pla-Salas, Xavier, additional, Rios-Blanco, Juan José, additional, and Fonollosa-Pla, Vicent, additional
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- 2016
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32. Implicación pronóstica de las manifestaciones clínicas extrahepáticas, autoinmunidad y capilaroscopia ungueal microscópica en pacientes con cirrosis biliar primaria
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Marí-Alfonso, Begoña, primary, Amengual-Guedan, María José, additional, Vergara-Gómez, Mercè, additional, Simeón-Aznar, Carmen Pilar, additional, Fonollosa-Plà, Vicente, additional, Jove-Buxeda, Esther, additional, Oliva-Morera, Juan, additional, and Tolosa-Vilella, Carles, additional
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- 2016
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33. Intravenous Cyclophosphamide Pulse Therapy in the Treatment of Systemic Sclerosis-Related Interstitial Lung Disease : A Long Term Study
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Simeón-Aznar, Carmen Pilar, Fonollosa Pla, Vicent, Tolosa-Vilella, Carles, Selva-O'Callaghan, A., Solans-Laqué, Roser, Palliza, E., Muñoz Gall, Xavier, Vilardell-Tarrés, M., and Universitat Autònoma de Barcelona. Departament de Medicina
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Vital capacity ,Cyclophosphamide ,Interstitial lung disease ,cyclophosphamide ,Article ,FEV1/FVC ratio ,DLCO ,Interquartile range ,Internal medicine ,medicine ,Lung volumes ,interstitial lung disease ,business.industry ,respiratory system ,medicine.disease ,Surgery ,respiratory tract diseases ,Regimen ,Cardiology ,Systemic sclerosis ,business ,medicine.drug - Abstract
Objective: Interstitial lung disease (ILD) frequently complicates systemic sclerosis (SSc). Cyclophosphamide (CYC) is a promising immunosuppressive therapy for SSc-related ILD. Our objective was to investigate the effectiveness of an intravenous CYC (iv CYC) pulse regime in SSc-related ILD during treatment and thereafter. Methods: In a prospective observational study ten consecutive patients with SSc-related ILD were treated with iv CYC in a pulse regime lasting from 6 to 24 months. Clinical status, pulmonary functional testing (PFT) and high resolution com- puted tomography (HRCT) of the chest were evaluated at enrolment and 6, 12 and 24 months thereafter. After treatment withdrawal, patients were followed up every 6 months with PFT and chest HRCT to monitor lung disease. Results: Clinical improvement was apparent in 8 out of 10 patients. The median values of forced vital capacity (FVC), forced expiratory volume in the first second (FEV1) and diffusion lung capacity for carbon monoxide (DLCO) as well as ground-glass pattern on HRCT did not change significantly after 6, 12 and 24 months of therapy. The follow-up continued in 8 out of 10 patients after treatment withdrawal for a median of 26.5 months (range: 12-48 months). The final median FVC was 54.5% of predicted value (interquartile range, IQR= 31.6%-94%). Only one patient suffered a FVC deterioration greater than 10%, even though less than 160 ml. The final median DLCO was 68% of predicted value (IQR=38.3-83.6%). Only 2 patients who developed pulmonary arterial hypertension deteriorated their DLCO values of more than 15%. Conclusions: An iv CYC pulse regimen over 24 months may stabilize pulmonary activity in patients with SSc-related ILD during the course of treatment and for a median of 26.5 months thereafter.
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- 2008
34. Applying the ACR/EULAR Systemic Sclerosis Classification Criteria to the Spanish Scleroderma Registry Cohort.
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Sáez-Comet, Luis, Pilar Simeón-Aznar, Carmen, Pérez-Conesa, Mercedes, Vallejo-Rodríguez, Carmen, Tolosa-Vilella, Carles, Iniesta-Arandia, Nerea, Colunga-Argüelles, Dolores, Victoria Egurbide-Arberas, Maria, Ortego-Centeno, Norberto, Antonio Vargas-Hitos, José, Freire-Dapena, Mayka, Rubio-Rivas, Manuel, José Ríos-Blanco, Juan, Trapiella-Martínez, Luis, Fonollosa-Pla, Vicent, Simeón-Aznar, Carmen Pilar, Egurbide-Arberas, Maria Victoria, Vargas-Hitos, José Antonio, Ríos-Blanco, Juan José, and RESCLE Investigators
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- 2015
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35. Registry of the Spanish Network for Systemic Sclerosis: Clinical Pattern According to Cutaneous Subsets and Immunological Status
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Simeón-Aznar, Carmen Pilar, primary, Fonollosa-Plá, Vicent, additional, Tolosa-Vilella, Carles, additional, Espinosa-Garriga, Gerard, additional, Ramos-Casals, Manel, additional, Campillo-Grau, Mercedes, additional, García-Hernández, Francisco José, additional, Castillo-Palma, María Jesús, additional, Sánchez-Román, Julio, additional, Callejas-Rubio, José Luis, additional, Ortego-Centeno, Norberto, additional, Egurbide-Arberas, Maria Victoria, additional, Trapiellla-Martínez, Luis, additional, Gallego-Villalobos, María, additional, Sáez-Comet, Luis, additional, Velilla-Marco, José, additional, Camps-García, María Teresa, additional, de Ramón-Garrido, Enrique, additional, Esteban Marcos, Eva María, additional, Pallarés-Ferreres, Lucio, additional, Hidalgo-Tenorio, Carmen, additional, Sabio-Sánchez, José Mario, additional, Gómez-de la Torre, Ricardo, additional, Salvador-Cervello, Gonzalo, additional, Rios-Blanco, Juan José, additional, Gil-Aguado, Antonio, additional, and Vilardell-Tarrés, Miquel, additional
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- 2012
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36. Hierarchical CRF with product label spaces for parts-based models
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Roig, Gemma, primary, Boix, Xavier, additional, De la Torre, Fernando, additional, Serrat, Joan, additional, and Vilella, Carles, additional
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- 2011
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37. Respuesta de los autores
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Tolosa Vilella, Carles, primary and Simeón Aznar, Carmen Pilar, additional
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- 2010
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38. Remote Sensing and Skywave Digital Communication from Antarctica
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Bergadà, Pau, primary, Deumal, Marc, additional, Vilella, Carles, additional, Regué, Joan, additional, Altadill, David, additional, and Marsal, Santi, additional
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- 2009
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39. El fenómeno de Raynaud
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Tolosa Vilella, Carles, primary, Simeón Aznar, Carmen Pilar, additional, and Gabarró Julià, Lourdes, additional
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- 2009
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40. IMAGEN DE LA SEMANA
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Pla Salas, Xavier, primary, Tolosa Vilella, Carles, additional, Soler, Alfons, additional, and Oristrell Salvà, Joaquim, additional
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- 2008
- Full Text
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41. Leucemia linfática crónica con transformación a linfoma difuso de célula grande B o síndrome de Richter con afectación ósea.
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Pla Salas, Xavier, additional, Tolosa Vilella, Carles, additional, Soler, Alfons, additional, and Oristrell Salvà, Joaquim, additional
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- 2008
- Full Text
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42. Olanzapine-induced agranulocytosis
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Tolosa-Vilella, Carles, primary, Ruiz-Ripoll, Ada, additional, Mari-Alfonso, Begoña, additional, and Naval-Sendra, Elsa, additional
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- 2002
- Full Text
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43. Antidepresivos tricíclicos: efectos adversos y ventajas de la monitorización terapéutica
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Tolosa Vilella, Carles, primary, Duñó Ambròs, Rosó, additional, and Escoté Llobet, Santiago, additional
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- 2002
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44. Raynaud's phenomenon and positive antinuclear antibodies in a malignancy
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Tolosa-Vilella, Carles, Ordi-Ros, Josep, Vilardell-Tarres, Miquel, Selva-O'Callaghan, Albert, and Jordana-Comajuncosa, Rosa
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Antinuclear antibodies -- Measurement ,Raynaud's disease -- Case studies ,Cancer -- Diagnosis ,Health - Abstract
Raynaud's phenomenon is characterized by vasospasm, constriction of blood vessels, in response to cold or emotional stress. Usually, this results in an initial whitening of the skin, followed by a bluish color and then reddening, but occasionally the response is incomplete and only part of the color changes occur. Raynaud's phenomenon may occur by itself as a primary disorder, or may be a consequence of disorders such as autoimmune diseases, in which the body makes antibodies against its own tissues. A case is reported of a 78-year-old woman with cancer who developed Raynaud's phenomenon and a high blood level of antinuclear antibodies (antibodies that react against components from the nucleus of cells). High levels of antinuclear antibodies are typically associated with autoimmune disorders. The woman had a two-month history of poor sensation and color changes in the hands, and weight loss. Laboratory tests showed elevated antinuclear antibodies, and ultrasound studies showed abnormalities in the liver. Biopsy confirmed cancerous cells in the liver. The tissue origin of the cancer could not be identified, and the patient died four months later. Raynaud's phenomenon has been previously associated with cancer chiefly in relation to chemotherapy. Measurable antinuclear antibody levels have also been found in association with cancer, but rarely in combination with Raynaud's phenomenon. The study suggests that sudden development of this disorder and measurable levels of these antibodies in patients without other evidence of autoimmune or connective tissue disorders may indicate the presence of cancer. (Consumer Summary produced by Reliance Medical Information, Inc.)
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- 1990
45. Mesangioproliferative Glomerulonephritis and Antibodies to Phospholipids in a Patient with Acute Q Fever: Case Report.
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Tolosa-Vilella, Carles, Rodríguez-Jornet, Angel, Font-Rocabanyera, Juli, and Andreu-Navarro, Xavier
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Glomerulonephritis is a rare complication of Q fever and is usually associated with the chronic form of disease, i.e., infectious endocarditis. We describe a 31-year-old man with a self-limited acute febrile illness due to Coxiella burnetii infection who had acute renal failure secondary to mesangioproliferative glomerulonephritis. Antibodies to phospholipids were detected and suggested the diagnosis of Q fever in this case. [ABSTRACT FROM PUBLISHER]
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- 1995
46. Estudi epidemiològic i dels factors predictius de desenvolupament de la crisi renal esclerodèrmica en la cohort del registre espanyol d'esclerodèrmia
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Pla Salas, Xavi, Tolosa Vilella, Carles, and Fonollosa Pla, Vicent
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Crisis renal esclerodérmica ,Esclerosis sistémica ,Scleroderma renal crisis ,Crisi renal esclerodèrmica ,Epidemiology ,616.4 ,Systemic sclerosis ,Epidemiología ,Epidemiologia ,Ciències de la Salut ,Esclerosi sistèmica - Abstract
La crisi renal esclerodèrmica (CRE) és una complicació poc freqüent però greu de l'esclerosi sistèmica (ES). Tanmateix, a la literatura existeix discrepància respecte l'existència de variacions en la seva prevalença al llarg del temps. A més, no es disposa d'estudis epidemiològics ni dels factors predictius de la CRE en el nostre entorn. Els objectius han estat: 1) caracteritzar la cohort de CRE en el registre espanyol d'esclerodèrmia (RESCLE), determinar la seva epidemiologia i els seus factors predictius; i 2) investigar si s'ha produït algun canvi en l'epidemiologia de la CRE al llarg del temps i identificar-ne les seves possibles causes. En el primer estudi, es van identificar 44 casos de CRE dins la cohort global de 1939 amb ES, constatant que la cohort espanyola de CRE té una prevalença molt baixa, del 2.3%. Es van identificar els següents factors predictors de CRE: pertànyer al subtipus d'esclerosi cutània difusa (EScd), període de temps més curt entre el debut i el diagnòstic d'ES, positivitat d'anticossos RNA polimerasa III (ARA) i major ús de glucocorticoides (GC) i d'anàlegs de la prostaciclina. Els resultats també van mostrar un alta morbiditat i mortalitat en aquesta sèrie. En el segon estudi, es van comparar la prevalença i incidència de CRE en 2 cohorts temporals diferents, observant un descens significatiu en la prevalença (3.7% vs. 1.3%) i incidència (3.5% vs. 1.08%) de la CRE al llarg del temps. Posteriorment, s'ha comparat les dues cohorts temporals (CR vs. CA) per tal d'identificar les causes d'aquesta variació epidemiològica. S'han identificat els següents factors associats a la CR: major prevalença de subtipus d'ES inicial i pre-ES, menor compliment de criteris ACR/EULAR 2013, major edat al diagnòstic d'ES, major exposició al fum del tabac, menor antecedent d'hipertensió arterial (HTA), menor prevalença d'afectació orgànica (úlceres digitals (UD), telangièctasis, calcinosi, malaltia pulmonar intersticial (MPI), neuropatia perifèrica i síndrome seca), menor presència de patrons esclerodèrmics a la capil·laroscòpia periungueal (CP), menor mortalitat global i un increment d'ús de tractament farmacològic per l'ES (major ús d'antagonistes del calci (ACa), vasodilatadors (VD) específics, antagonistes del receptor de l'endotelina-1 (ARE), inhibidors de l'enzim convertidor de l'angiotensina (IECA), antagonistes del receptor de l'angiotensina-II (ARA-II) i immunosupressors). La mortalitat global va ser del 18%, però amb una millora significativa al llarg del temps (29% vs. 11%). Podem concloure que: 1) la prevalença de CRE en la cohort de pacients amb ES és baixa (2.3%), tot i que s'ha associat a una supervivència reduïda respecte els pacients amb ES sense CRE; 2) els factors predictius de CRE identificats en aquest estudi han estat: el subtipus EScd, un temps més curt entre el debut i diagnòstic d'ES, major presència d'ARA i un major ús de GC i d'anàlegs de la prostaciclina; 3) la prevalença de la CRE ha disminuït significativament al llarg del temps en la cohort global. Es destacable que hi ha hagut una reducció de la prevalença en el subtipus EScl, però no en el subtipus EScd; 4) Els factors associats a una baixa prevalença de CRE en la CR són: major presència de subtipus precoces (ES inicial i pre-ES), major antecedent de tabaquisme i major edat al diagnòstic d'ES, però menor compliment de criteris classificatoris, menor antecedent d'HTA, UD, telangièctasis, calcinosi, MPI, neuropatia perifèrica i síndrome sec, així com menor presència de patrons esclerodèrmics a la CP. Així mateix, la CR s'ha associat també amb major ús de tractaments farmacològics, com ara els ACa, VD específics, IECA, ARA-II, i immunosupressors, i a una menor taxa de mortalitat. La crisis renal esclerodérmica (CRE) es una complicación poco frecuente pero grave de la esclerosis sistémica (ES). Sin embargo, en la literatura existe discrepancia respecto a la existencia de variaciones en su prevalencia a lo largo del tiempo. Además, no se dispone de estudios epidemiológicos ni de los factores predictivos de CRE en nuestro entorno. Los objetivos han sido: 1) caracterizar la cohorte de CRE en el registro español de esclerodermia (RESCLE), determinar su epidemiología y sus factores predictivos; y 2) investigar si se ha producido algún cambio en la epidemiología de la CRE a lo largo del tiempo e identificar sus posibles causas. En el primer estudio, se identificaron 44 casos de CRE en la cohorte global de 1939 con ES, constatando que la cohorte española de CRE tiene una prevalencia muy baja, del 2.3%. Se identificaron los siguientes factores predictores de CRE: pertenecer al subtipo de esclerosis cutánea difusa (EScd), período de tiempo más corto entre el debut y el diagnóstico de ES, positividad de anticuerpos RNA polimerasa III (ARA) y mayor uso de glucocorticoides (GC) y de análogos de la prostaciclina. Los resultados también mostraron una alta morbilidad y mortalidad en esta serie. En el segundo estudio, se compararon la prevalencia e incidencia de CRE en 2 cohortes temporales distintos, observando un descenso significativo en la prevalencia (3.7% vs. 1.3%) e incidencia (3.5% vs. 1.08%) de la CRE a lo largo del tiempo. Posteriormente, se han comparado las dos cohortes temporales (CR vs. CA) para identificar las causas de esta variación epidemiológica. Se han identificado los siguientes factores asociados a la CR: mayor prevalencia de subtipo de ES inicial y pre-ES, menor cumplimiento de criterios ACR/EULAR 2013, mayor edad en el diagnóstico de ES, mayor exposición al humo del tabaco, menor antecedente de hipertensión arterial (HTA), menor prevalencia de afectación orgánica (úlceras digitales (UD), telangiectasias, calcinosis, enfermedad pulmonar intersticial (MPI), neuropatía periférica y síndrome seco), menor presencia de patrones esclerodérmicos en la capilaroscopia periungueal (CP), menor mortalidad global y un incremento de uso de tratamiento farmacológico por el ES (mayor uso de antagonistas del calcio (ACa), vasodilatadores (VD) específicos, antagonistas del receptor de la endotelina-1 (ARE) ), inhibidores de la enzima convertidora de la angiotensina (IECA), antagonistas del receptor de la angiotensina-II (ARA-II) e inmunosupresores). La mortalidad global fue del 18% pero con una mejora significativa a lo largo del tiempo (29% vs. 11%). Se puede concluir que: 1) la prevalencia de CRE en la cohorte de pacientes con ES es baja (2.3%), aunque se ha asociado a una supervivencia reducida respecto a los pacientes con ES sin CRE; 2) los factores predictivos de CRE identificados en este estudio han sido: el subtipo EScd, un tiempo más corto entre el debut y diagnóstico de ES, mayor presencia de ARA y un mayor uso de GC y análogos de la prostaciclina; 3) la prevalencia de la CRE ha disminuido significativamente a lo largo del tiempo en la cohorte global. Es destacable que ha habido una reducción de la prevalencia en el subtipo EScl, pero no en el subtipo EScd; 4) Los factores asociados a una baja prevalencia de CRE en la CR son: mayor presencia de subtipos precoces, mayor antecedente de tabaquismo y mayor edad al diagnóstico de ES, menor cumplimiento de criterios clasificatorios, menor antecedente de HTA, UD, telangiectasias, calcinosis, MPI, neuropatía periférica y síndrome seco, así como menor presencia de patrones esclerodérmicos en la CP. Asimismo, la CR se ha asociado también con mayor uso de tratamientos farmacológicos, como por ejemplo los ACa, VD específicos, IECA, ARA-II, e inmunosupresores, y a una menor mortalidad. Scleroderma renal crisis (SRC) is a rare but severe Systemic Sclerosis (SSc) complication usually associated with bad prognosis. Some published SSc series in recent decades have shown a downward trend in the SRC prevalence, although other authors have not found any variation in SRC epidemiology. In addition, there are few studies assessing the epidemiological variations of SRC over time, as well as describing the characteristics of patients with SRC and the risk factors for developing it. The objectives were: 1) to characterize the SRC cohort of the Spanish Systemic Sclerosis registry (RESCLE) and to identify its epidemiology and predictors; and 2) to assess whether the epidemiology of SRC had undergone any variation over time and to determine the causes if there was any change. In the first study, 44 SRC cases were identified from the entire SSc cohort (n=1939). Thus, a SRC prevalence of 2.3% was established in this Spanish SSc cohort, which was considered very low but it is in accordance with recent SSc series. Thereafter, the predictive factors were identified: diffuse cutaneous SSc subset, shorter period of time from SSc onset to diagnosis, RNA polymerase III antibodies positivity, and higher use of glucocorticoids and prostacyclin analogues. The results also indicated a high morbidity and mortality in this series. In the second study, the prevalence and incidence of SRC were compared between two different temporal cohorts. An old cohort (OC) of SSc patients diagnosed before December 31st 2002 vs. recent cohort (RC) of SSc patients diagnosed thereafter. A significant decrease in the SRC prevalence (3.7% vs. 1.31%) and incidence (3.5% vs. 1.08%), has been observed over time. Thereafter, both temporal cohorts (RC vs. OC) were compared to assess the causes of this epidemiological variation. Thus, the RC was associated to higher prevalence of early SSc and pre-SSc, lesser 2013 ACR/EULAR criteria fulfilling, older age at diagnosis, higher smoking history, lesser arterial hypertension history, lesser organic involvement (less prevalence of digital ulcers, telangiectasis, calcinosis, interstitial pulmonary disease, peripherical neuropathy and sicca syndrome), lesser scleroderma pattern at nailfold capillaroscopy, lesser overall mortality and to a notably increase in SSc treatment use (higher use of calcium channel blockers, specific vasodilators, Endothelin 1 receptor antagonists, angiotensin converter enzyme inhibitors, angiotensin-II receptor blockers, and immunosuppressants). Overall mortality rate was 18%, but a significant improvement in mortality was observed over time (29% vs. 11%). Considering the data from this large Spanish study of SSc patients from the RESCLE registry, we can conclude that: 1) SRC prevalence is low (2.3%) in SSc cohort, but it was associated with reduced survival respect SSc patients with no SRC; 2) the predictive factors for SRC identified in the present study were: diffuse cutaneous SSc subset, short time from SSc onset to SSc diagnosis, presence of RNA polymerase III and use of glucocorticoids and prostacyclin analogues; 3) the prevalence of SRC has decreased significantly over time in the overall SSc cohort, due to the substantial decrease in prevalence in the limited cutaneous SSc subset but not in the diffuse cutaneous SSc subset; 4) Factors associated with low SRC prevalence in the recent cohort of SSc are: greater presence of early subtypes (early and very early SSc), greater exposure to tobacco, and older age at diagnosis but lesser SSc classification criteria fulfilment, history of hypertension, UD, telangiectasis, calcinosis, MPI, peripheral neuropathy and dry syndrome, as well as lesser presence of scleroderma patter on capillaroscopy. In addition, the recent cohort is also associated with a higher use of pharmacological treatments, specifically calcium channel blockers, angiotensin converting enzyme inhibitors, angiotensin-II receptor blockers, specific vasodilators and immunosuppressants, and lower overall mortality rate.
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- 2022
47. Esclerosis sistémica y enfermedad hepato-biliar: Estudio bidireccional de la relación entre la esclerosis sistémica y la enfermedad hepato-biliar
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Marí Alfonso, Begoña, Tolosa Vilella, Carles, Fonollosa Pla, Vicent, and Universitat Autònoma de Barcelona. Departament de Medicina
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Esclerosis sistémica ,Colangitis biliar primaria ,Primary biliary cholangitis ,Systemic sclerosis ,Enfemedad hepatobiliar ,Hepatobiliary dsease ,Ciències de la Salut - Abstract
La esclerosis sistémica (ES) es una enfermedad autoinmune sistémica (EAS) que puede afectar a la mayoría de órganos internos mediante la acumulación de colágeno en los tejidos y/o desarrollo de una vasculopatía obliterante, en un entorno de disfunción autoinmune. Aunque la disfunción hepato-biliar (DHB) no es un hallazgo infrecuente en la ES, su presencia no se considera característica de la enfermedad y se han publicado pocos estudios que valoren la relación bidireccional entre la ES y la DHB, en particular autoinmune órgano-específica. El primer artículo de esta Tesis doctoral es un estudio observacional multicéntrico Español de 1572 pacientes con ES y como objetivos establece la prevalencia de DHB en pacientes con ES y enumera las causas que son predominantemente autoinmunes órgano-específicas, como la colangitis biliar primaria (CBP) en 4.3% y la hepatitis autoinmune en 1.2% y CBP sin anticuerpos antimitocondria en 0.4%.. La presencia de DHB en pacientes con ES se asocia independientemente a un menor riesgo de padecer una ES cutánea difusa y a una mayor prevalencia de calcinosis cutánea, disfunción diastólica de ventrículo izquierdo, síndrome seco y positividad de los anticuerpos anticentrómero (ACA). La presencia de DHB no influyó en la supervivencia. Según los subtipos cutáneos, la DHB se asocia a: 1) en pacientes con ES cutánea limitada, a un mayor tiempo desde el inicio de la ES hasta su diagnóstico y a una mayor prevalencia de síndrome seco y presencia de ACA; 2) en pacientes con ES sine escleroderma, a una mayor prevalencia de síndrome seco, y 3) en pacientes con ES cutánea difusa, no se identificó ningún rasgo diferencial con respecto a los pacientes sin DHB. El segundo artículo es un estudio observacional que reclutó una cohorte de 62 pacientes con CBP a los que se aplicó un protocolo de estudio dirigido a determinar la prevalencia de EAS asociada a esta entidad. Los resultados muestran que la prevalencia de EAS en pacientes con CBP es del 35.4%. La ES es la entidad más frecuentemente diagnosticada (21%), en particular del subtipo cutáneo limitado (11%). Los ACA son el único parámetro inmunológico identificado con mayor frecuencia en pacientes con CBP y EAS. Los pacientes con CBP-EAS tienen más prevalencia de manifestaciones extrahepáticas como fenómeno de Raynaud, síndrome seco, telangiectasias, artritis y disnea, así como una asociación significativa a ACA y alteraciones capilaroscópicas sugestivas de ES, que pueden ser factores predictores de la asociación de ambas enfermedades. Por estos motivos, recomendamos el empleo de un protocolo de valoración clínica, inmunológica y de una capilaroscòpia periungueal. Systemic sclerosis (SSc) is a chronic autoimmune systemic disease (ASD) that may affect most internal organs caused by an accumulation of collagen in tissues and/or the development of obliterative vasculopathy in the context of autoimmune dysfunction. Although liver disturbance is not a rare finding in SSc, its presence is not considered characteristic of this disease and few studies have assessed the bidirectional relationship between SSc and hepatobiliary dysfunction (HBD), especially addressed to investigating organ-specific diseases. The first article of this doctoral thesis is a Spanish multicenter observational study including 1572 patients with SSc that establishes the prevalence of HBD in patients with SSc at 7.5% and the main causes as organ-specific autoimmune diseases, such as primary biliary cholangitis (PBC) at 4.3%, autoimmune hepatitis at 1.2% and PBC without antimitochondrial antibodies at 0.4%. The presence of HBD in patients with SSc is independently associated with a lower risk of developing diffuse cutaneous SSc and a greater prevalence of cutaneous calcinosis, diastolic dysfunction of left ventricle, Sicca syndrome and positive anticentromere antibodies. The presence of HBD did not influence survival. According to cutaneous subtypes, HBD is associated with: 1) In patients with limited cutaneous SS, a longer time-to-diagnosis since SSc onset to its diagnosis and a greater prevalence of sicca syndrome and presence of anticentromere antibodies; 2) In patients with SSc sine scleroderma, a greater prevalence of sicca syndrome, and 3) In patients with diffuse cutaneous SSc, no distinguishing feature was identified compared to patients without HBD. The second article is an observational study recruiting a cohort of 62 patients with PBC included in a study protocol to determine the prevalence of ASD associated to this entity. The results obtained show that the prevalence of ASD in patients with PBC is 35.4%. SSc is the most prevalent condition (21%), especially in the limited cutaneous subtype (11%). Anticentromere antibodies are the only immunological parameter most frequently identified in patients with PBC and ASD. Patients with PBC-ASD present extrahepatic manifestations such as Raynaud`s phenomenon, sicca syndrome, telangiectases, arthritis and dyspnea, as well as a significant association of anticentromere antibodies and capillaroscopic alterations suggestive of SSc, which may be predictive factors for the association between both diseases. Consequently, we recommend the use of a protocol for clinical and immunological assessment and periungueal capillaroscopy.
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- 2018
48. Las plaquetas reticuladas medidas por citometría de flujo: un marcador indirecto de actividad trombocitopoyética
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Monteagudo Jiménez, Manuel, Tolosa Vilella, Carles, Fonollosa Pla, Vicent, and Universitat Autònoma de Barcelona. Departament de Medicina
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Plaquetas reticuladas ,Trombocitopenias ,616.1 ,Citometría de flujo ,Ciències de la Salut - Abstract
Introducción Las plaquetas reticuladas (PR) son las formas más jóvenes de las plaquetas circulantes, que contienen ARN residual, y por tanto constituyen el equivalente plaquetario de los reticulocitos de la serie roja. La primera descripción se realizó en 1969, a partir de su visión directa en extensiones periféricas de un modelo animal de pérdida de sangre. Los primeros trabajos que investigan su aplicación clínica no aparecen hasta 1990, cuando es posible utilizar la citometría de flujo para su identificación. La determinación de PR por citometría de flujo, no es una técnica estandarizada, se han utilizado distintos sustratos, sangre total o plasma rico en plaquetas, diversos colorantes y diferentes tiempos de incubación. Metodología Entre 2002 y 2005, se recogieron de forma prospectiva pacientes que ingresados o de forma ambulatoria presentaban trombocitopenia, inferior a 100x109/L confirmada por revisión manual de extensión periférica así como un grupo control de pacientes no trombocitopénicos. Se determinó el porcentaje de plaquetas reticuladas mediante citometría de flujo, usando sangr total como sustrato y un doble marcaje de las plaquetas. Se utilizó un anticuerpo monoclonal anti-glicoproteina III (CD61 PerCP®) para identificar las plaquetas y Naranja de Tiazol (Retic-count®) como colorante de ARN plaquetar. La determinación automática de PR, se realizó detrminando la fracción plaquetar inmadura (IPF) mediante el autoanalizador Sysmex XE 2100. Objetivos y Resultados Objetivo 1, Valorar si la determinación de plaquetas reticuladas mediante citometría de flujo utilizando un doble marcaje es una técnica capaz de diferenciar trombocitopenias de diferentes etiologías. Los resultados de este primer trabajo fueron publicados en European Journal of Internal Medicine (M. Monteagudo Jiménez, MJ Amengual Guedán, L Muñoz Martín , JA Soler Campos, I Roig Martínez, C Tolosa Vilella. Measurement of reticulated platelets by simple flow cytometry : an indirect thrombocytopoienic marker. Eur J Intern Med. 2006 Dec;17(8): 541-4). Objetivo 2. Valorar si la determinación de plaquetas reticuladas por citometría de flujo a partir de sangre total puede ser una técnica útil de cribado inicial para el diagnóstico etiológico de las trombocitopenias. Los resultados de este segundo trabajo fueron publicados en Quaterly Journal Medicine (Monteagudo M, Amengual MJ, Muñoz L, Soler JA, Roig I, Tolosa C. Reticulated platelets as a screening test to identify thrombocytopenia aetiology QJM. 2008 Jul;101(7):549-55). Objetivo 3. Analizar la correlación que existe entre la determinación de plaquetas reticuladas por citometría de flujo y el número de megacariocitos en médula ósea determinados por aspirado medular. Los resultados de este tercer trabajo fueron publicados en Medicina Clínica (Monteagudo M, Lucchetti G, Amengual MJ, Muñoz L, Soler JA, Roig I, Tolosa C. Relationship between reticulated platelets and megacaryocyte number in thrombocytopenic patients Med Clin (Barc). 2009 Jun 20;133(3):81-5). Objetivo 4. Valorar la correlación existente entre los valores de PR determinadas por citometría de flujo con los resultados de la fracción plaquetar inmadura (IPF), determinada de forma automática mediante el autoanalizador Sysmex 2100. Los resultados de este último trabajo fueron publicados en European Journal of Haematology (Pons I , Monteagudo M, Lucchetti G, Mun˜ oz L, Perea G, Colomina I, Guiu J, Obiols J. Correlation between immature platelet fraction and reticulated platelets. Usefulness in the etiology diagnosis of thrombocytopenia. Eur J Haematol 2010 August ; 85(2):158-163 ). Conclusiones 1. La determinación de plaquetas reticuladas a partir de sangre total utilizando un doble marcaje, mediante citometría de flujo y sin manipulación de la muestra es una técnica de fácil realización y reproducible. 2. La determinación de plaquetas reticuladas por esta técnica permite diferenciar trombocitopenias de origen central de trombocitopenias de origen periférico. 3. Unos valores de plaquetas reticuladas por encima del 11% mediante esta metodología, tienen una alta sensibilidad (93%) y buena especificidad (85%) para el diagnóstico de trombocitopenia periférica, con actividad megacariocítica medular elevada. 4. Debe tenerse presente la posibilidad de que existan falsos negativos, pero especialmente falsos positivos, en algunos pacientes con síndromes mielodisplásicos y algunas leucemias agudas de estirpe linfoide o mieloide. 5. Las plaquetas reticuladas en sangre periférica son un marcador indirecto de la actividad megacariocítica medular. 6. La presencia de plaquetas reticuladas periféricas superiores al 11%, en trombocitopenias aisladas y agudas, tiene una buena correlación con el número de megacariocitos a nivel medular. 7. Existe una buena correlación entre la determinación de plaquetas reticuladas realizada mediante citometría de flujo, según nuestra metodología, y los valores de plaquetas reticuladas realizadas de forma automática mediante el valor IPF del autoanalizador Sysmex-2100. 8. Los valores de IPF obtenidos en nuestros pacientes tienden a ser globalmente superiores, a los valores de plaquetas reticuladas medidas por citometría de flujo. 9. La sencillez de realización del IPF, su reproducibilidad y su buena correlación con la citometría de flujo pueden convertir su determinación en una técnica de cribado útil para la orientación etiológica inicial en el paciente trombocitopénico, siempre que se tenga en cuanta la valoración global del mismo. Introduction Reticulated platelets (RP) are the youngest forms of circulating platelets, which contain residual RNA, and therefore constitute the platelet equivalent of red cell reticulocytes. The first description was made in 1969, by direct visualization in peripheral blood film in an animal model of blood loss. The first study investigating the clinical application do not appear until 1990, when it is possible to use flow cytometry for their identification. RP measurement by flow cytometry is not a standardized technique. Several dyes for mRNA stain have been used. Two substrates for RP determination, whole blood or platelet rich plasma have been published and different incubation times have been described Methodology From 2002 to 2005, we prospectively enrolled both admitted and out patients being attend at our Hospital with thrombocytopenia, fewer than 100x109 /L, confirmed after peripheral blood film review and a control group of non thrombocytopenic patients. A direct, whole-blood, dual–labelling flow cytometric method was used. Direct, whole-blood double coverage was achieved using a monoclonal anti-glycoprotein III antibody (CD 61 PerCP) for platelet identification and thiazole orange (Retic-count) as platelet mRNA stain.The automatic determination of PR, was measured by determining the immature platelet fraction (IPF) by autoanalyzer Sysmex XE 2100. Objectives and Results Objective 1 To assess whether the determination of reticulated platelets by flow cytometry using double labeling is a technique capable of differentiating different causes thrombocytopenia. The results of this first study were published in European Journal of Internal Medicine (M. Monteagudo Jiménez, MJ Amengual Guedán, L Muñoz Martín, JA Soler Campos, I Roig Martínez, C Tolosa Vilella. Measurement of reticulated platelets by simple flow cytometry: an indirect thrombocytopoienic marker. Eur J Intern Med 2006 Dec; 17 (8): 541-4). Objective 2. Assess whether the determination of reticulated platelets by flow cytometry from whole blood can be a useful initial screening test for the etiological diagnosis of thrombocytopenia. The results of this second study were published in Quarterly Journal of Medicine (Monteagudo M, Amengual MJ, Muñoz L, Soler JA, Roig R, Tolosa C. Reticulated platelets as a screening test to Identify thrombocytopenia aetiology QJM. 2008 Jul; 101 (7) :549-55). Objective 3. To analyze the correlation between the determination of reticulated platelets by flow cytometry and the number of megakaryocytes in bone marrow determined by bone marrow aspiration. The results of this third study were published in Medicina Clínica (Monteagudo M, Lucchetti G, Amengual MJ, Muñoz L, Soler JA, Roig R, Tolosa C. Relationship between reticulated platelets and megacaryocyte number in thrombocytopenic patients. Med Clin (Barc). 2009 June 20, 133 (3) :81-5). Objective 4. To assess the correlation between PR values determined by flow cytometry with the results of the immature platelet fraction (IPF), automatically determined by the autoanalyzer Sysmex 2100. The results of this latest study were published in European Journal of Haematology (Pons I, Monteagudo M, Lucchetti G, Muñoz L, Perea G, Colomina I, Guiu J, Obiols J. Correlation between immature platelet fraction and reticulated platelets. Usefulness etiology in the diagnosis of thrombocytopenia. Eur J Haematol 2010 August, 85 (2) :158-163). Conclusions 1. The determination of reticulated platelets from whole blood using double labeling, flow cytometry and without sample manipulation is a technique easy to perform and reproducible. 2. The determination of reticulated platelets by this technique allow to differentiate between thrombocytopenia of central origin of thrombocytopenia of peripheral origin. 3. Reticulated platelet values above 11% with this methodology, have a high sensitivity (93%) and good specificity (85%) for the diagnosis of peripheral thrombocytopenia with high megakaryocytic marrow activity. 4. Should be considered the possibility of false negatives, but especially false positives, in some patients with myelodysplastic syndromes and some acute leukemias of lymphoid or myeloid. 5. Reticulated platelets in peripheral blood are a surrogate marker for megakaryocytic marrow activity. 6. The presence of peripheral reticulated platelets above 11% in isolated and acute thrombocytopenia, is well correlated with an elevated number of megakaryocytes in bone marrow 7. There is good correlation between the determination of reticulated platelets by flow cytometry carried out according to our methodology, and values of immature platelet fraction (IPF) performed automatically by the autoanalyzer Sysmex IPF-2100. 8.. IPF values obtained in our patients tend to be higher than the values of reticulated platelets measured by flow cytometry 9. The simplicity of implementation of the IPF, reproducibility and good correlation with flow cytometry, makes its determination on an useful screening test for thrombocytopenic patient, provided that we take into account the overall assessment of the patient
- Published
- 2010
49. Anti-Polymyositis/Scl Antibodies in Systemic Sclerosis: Clinical Associations in a Multicentric Spanish Cohort and Review of the Literature.
- Author
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Iniesta Arandia N, Espinosa G, Guillén Del Castillo A, Tolosa-Vilella C, Colunga-Argüelles D, González de Echávarri Pérez de Heredia C, Lledó GM, Comet LS, Ortego-Centeno N, Vargas Hito JA, Rubio-Rivas M, Freire M, Ríos-Blanco JJ, Rodríguez-Carballeira M, Trapiella-Martínez L, Fonollosa-Pla V, and Simeón-Aznar CP
- Subjects
- Antibodies, Autoantibodies, Cohort Studies, Humans, Arthritis, Polymyositis diagnosis, Polymyositis epidemiology, Scleroderma, Systemic diagnosis, Scleroderma, Systemic epidemiology
- Abstract
Objectives: To assess the clinical profile of patients with anti-polymyositis/Scl (PM/Scl) antibodies in a cohort of Spanish patients with systemic sclerosis., Methods: From the Spanish Scleroderma Study Group database, we selected patients in whom PM/Scl antibodies had been tested. We compared demographic, clinical, laboratory, and survival data between patients with and without PM/Scl antibodies., Results: Seventy-two of 947 patients (7.6%) tested positive for PM/Scl antibodies. Patients with PM/Scl antibodies presented initially with more puffy fingers and arthralgias but less Raynaud phenomenon. Regarding cumulative manifestations, myositis and arthritis were more prevalent in patients with PM/Scl antibodies, as well as pulmonary fibrosis. On the contrary, patients with PM/Scl antibodies had less pulmonary hypertension. No difference in terms of survival at 5 and 10 years was noticed between the 2 groups., Conclusions: In systemic sclerosis patients from Spain, PM/Scl antibodies are associated with a distinct clinical profile. However, PM/Scl antibodies did not influence survival., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)
- Published
- 2022
- Full Text
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50. Serodiscordant patients with systemic sclerosis: when antibody does not correspond to skin involvement.
- Author
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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
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