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180 results on '"Fraile G"'

1. Systemic phenotype of sarcoidosis associated with radiological stages. Analysis of 1230 patients

Author: Pérez-Alvarez, R., Brito-Zerón, P., Kostov, B., Feijoo-Massó, C., Fraile, G., Gómez-de-la-Torre, R., De-Escalante, B., López-Dupla, M., Alguacil, A., Chara-Cervantes, J., Pérez-Conesa, M., Rascón, J., Garcia-Morillo, J.S., Perez-Guerrero, P., Fonseca-Aizpuru, E., Akasbi, M., Bonet, M., Callejas, J.L., De-la-Red, G., Calvo, E., Soler, C., Peral-Gutiérrez, E., Gómez-Cerezo, J.F., Cruz-Caparrós, G., Rodríguez-Fernández, S., Pinilla, B., Gato, A., Chamorro, A.J., Morcillo, C., Robles, A., Ojeda, I., Vives, M.J., de Miguel-Campo, B., Penadés, M., De-Vicente, M., Bosch, X., Pérez-de-Lis, M., González-García, A., Yllera, C., Gracia-Tello, B., Perez-Gonzalez, A., Pedrosa-Aragón, M., Tolosa, C., Sisó-Almirall, A., Pallarés, L., and Ramos-Casals, M.
Published: 2019
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2. POS0271 CLINICAL SYMPTOMS AT GIANT CELL ARTERITIS DIAGNOSIS AS PREDICTORS OF PERMANENT VISUAL LOSS.

Author: Mestre-Torres, J., primary, Escalante, B., additional, Fonseca, E., additional, Martinez-Zapico, A., additional, Monteagudo, M., additional, Abdilla, M., additional, Perez Conesa, M., additional, Gracia Tello, B., additional, Prieto-González, S., additional, Fraile, G., additional, and Solans-Laqué, R., additional
Published: 2022
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3. CLINICAL SYMPTOMS AT GIANT CELL ARTERITIS DIAGNOSIS AS PREDICTORS OF PERMANENT VISUAL LOSS

Author: Mestre-Torres J, Escalante B, Fonseca E, Martinez-Zapico A, Monteagudo M, Abdilla M, Conesa M, Tello B, Prieto-Gonzalez S, Fraile G, and Solans-Laque R
Published: 2022

4. Systemic activity and mortality in primary Sjögren syndrome: predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients

Author: Brito-Zerón, P, Kostov, B, Solans, R, Fraile, G, Suárez-Cuervo, C, Casanovas, A, Rascón, F J, Qanneta, R, Pérez-Alvarez, R, Ripoll, M, Akasbi, M, Pinilla, B, Bosch, J A, Nava-Mateos, J, Díaz-López, B, Morera-Morales, M L, Gheitasi, H, Retamozo, S, and Ramos-Casals, M
Published: 2016
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5. Thrombotic microangiopathy (TMA) associated with pregnancy: role of the clinical laboratory in differential diagnosis

Author: Ramos Mayordomo Patricia, Capilla Díez Marta, Ticona Espinoza Danay Areli, Torres Jaramillo María Verónica, Martínez Tejeda Nathalie, Ticona Espinoza Thalia Gloria, Colmenero Calleja Cristina, and Fraile Gutiérrez Virginia
Subjects: microangiopathic hemolytic anemia, eculizumab, thrombotic microangiopathy, preeclampsia, hellp syndrome, atypical hemolytic uremic syndrome, Medical technology, R855-855.5
Abstract: Thrombotic microangiopathy (TMA) is characterized by thrombocytopenia, microangiopathic hemolytic anemia and target organ damage. Pregnancy is associated with several forms of TMA, including preeclampsia (PE), HELLP syndrome, thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). When HUS is secondary to a deregulation of the alternative complement pathway, it is known as atypical HUS (aHUS). Differential diagnosis is challenging, as these forms share clinical characteristics. However, early diagnosis is crucial for a specific treatment to be established and improve prognosis.
Published: 2024
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6. Microangiopatía trombótica (MAT) asociada al embarazo: papel del laboratorio clínico en el diagnóstico diferencial

Author: Ramos Mayordomo Patricia, Capilla Díez Marta, Ticona Espinoza Danay Areli, Torres Jaramillo María Verónica, Martínez Tejeda Nathalie, Ticona Espinoza Thalia Gloria, Colmenero Calleja Cristina, and Fraile Gutiérrez Virginia
Subjects: anemia hemolítica microangiopática, eculizumab, microangiopatía trombótica, preeclampsia, síndrome de hellp, síndrome hemolítico urémico atípico, Medical technology, R855-855.5
Abstract: La microangiopatía trombótica (MAT) se define por trombocitopenia, anemia hemolítica microangiopática y daño de órganos diana. El embarazo está asociado con varias formas de MAT como preeclampsia (PE), síndrome de HELLP, púrpura trombótica trombocitopénica (PTT) y síndrome hemolítico urémico (SHU). Cuando SHU se produce por desregulación de la vía alternativa del complemento se denomina SHU atípico (SHUa). El diagnóstico diferencial es complejo, ya que comparten características clínicas, siendo importante realizarlo precozmente para instaurar tratamiento específico y mejorar el pronóstico.
Published: 2024
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7. How are we treating our systemic patients with primary Sjögren syndrome? Analysis of 1120 patients

Author: Gheitasi, H., Kostov, B., Solans, R., Fraile, G., Suárez-Cuervo, C., Casanovas, A., Rascón, F. J., Qanneta, R., Pérez-Alvarez, R., Ripoll, M., Akasbi, M., Pinilla, B., Bosch, J. A., Nava-Mateos, J., Díaz-López, B., Morera-Morales, M. L., Retamozo, S., Ramos-Casals, M., and Brito-Zerón, P.
Published: 2015
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8. Comparison of the Birmingham Vasculitis Activity Score and the Five-Factor Score to Assess Survival in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Study of 550 Patients From Spain (REVAS Registry)

Author: Solans-Laqué R, Rodriguez-Carballeira M, Rios-Blanco JJ, Fraile G, Sáez-Comet L, Martinez-Zapico A, Frutos B, Solanich X, Fonseca-Aizpuru E, Pasquau-Liaño F, Zamora M, Oristrell J, Fanlo P, Lopez-Dupla M, Abdilla M, García-Sánchez I, Sopeña B, Castillo MJ, Perales I, Callejas JL, Spanish Registry of systemic vasculitis (REVAS), and Autoimmune Systemic Diseases St
Abstract: Objective To compare the accuracy of the Birmingham Vasculitis Activity Score (BVAS), version 3, and the Five Factor Score (FFS), version 1996 and version 2009, to assess survival in antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods A total of 550 patients withAAV(41.1% with granulomatosis with polyangiitis, 37.3% with microscopic polyangiitis, and 21.6% with eosinophilic granulomatosis with polyangiitis), diagnosed between 1990 and 2016, were analyzed. Receiver operating characteristic (ROC) curves and multivariable Cox analysis were used to assess the relationships between the outcome and the different scores. Results Overall mortality was 33.1%. The mean +/- SD BVASat diagnosis was 17.96 +/- 7.82 and was significantly higher in nonsurvivors than in survivors (mean +/- SD20.0 +/- 8.14 versus 16.95 +/- 7.47, respectively;P< 0.001). The mean +/- SD1996FFSand 2009FFSwere 0.81 +/- 0.94 and 1.47 +/- 1.16, respectively, and were significantly higher in nonsurvivors than in survivors (mean +/- SD1996FFS1.17 +/- 1.07 versus 0.63 +/- 0.81 [P< 0.001] and 2009FFS2.13 +/- 1.09 versus 1.15 +/- 1.05 [P< 0.001], respectively). Mortality rates increased according to the different 1996FFSand 2009FFScategories. In multivariate analysis,BVAS, 1996FFS, and 2009FFSwere significantly related to death (P= 0.007,P= 0.020,P< 0.001, respectively), but the stronger predictor was the 2009FFS(hazard ratio 2.9 [95% confidence interval 2.4-3.6]). When the accuracy ofBVAS, 1996FFS, and 2009FFSto predict survival was compared in the global cohort,ROCanalysis yielded area under the curve values of 0.60, 0.65, and 0.74, respectively, indicating that 2009FFShad the best performance. Similar results were obtained when comparing these scores in patients diagnosed before and after 2001 and when assessing the 1-year, 5-year, and long-term mortality. Correlation amongBVASand 1996FFSwas modest (r = 0.49;P< 0.001) but higher than betweenBVASand the 2009FFS(r = 0.28;P< 0.001). Conclusion BVASandFFSare useful to predict survival inAAV, but the 2009FFShas the best prognostic accuracy at any point of the disease course.
Published: 2020

9. Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity

Author: Solans-Laqué R, Fraile G, Rodriguez-Carballeira M, Caminal L, Castillo MJ, Martínez-Valle F, Sáez L, Rios JJ, Solanich X, Oristrell J, Pasquau F, Fonseca E, Zamora M, Callejas JL, Frutos B, Abdilla M, Fanlo P, García-Sánchez I, López-Dupla M, Sopeña B, Pérez-Iglesias A, Bosch JA, Spanish Registry of systemic vasculitis (REVAS) from the Autoimmune Diseases Stu, Spanish Registry of systemic vasculitis (REVAS) from the Autoimmune Diseases Study Group (GEAS) of the Spanish Society of Internal Medicine (SEMI), [Solans-Laqué,R, Martínez-Valle,F, Bosch,JA] Hospital Valle Hebrón, Barcelona. [Fraile,G] Hospital Ramón y Cajal, Madrid. [Rodriguez-Carballeira,M] Mutua Terrassa, Barcelona. [Caminal,L] Hospital Central de Asturias. [Castillo,MJ] Hospital Virgen del Rocío, Sevilla. [Sáez,L] Hospital Miguel Servet, Zaragoza. [Rios,JJ] Hospital La Paz, Madrid. [Solanich,X] Hospital Bellvitge. [Oristrell,J] Hospital Parc Tauli Sabadell, Barcelona. [Pasquau.F] Hospital Marina Baixa, Villajoyosa, Alicante. [Fonseca,E] Hospital Cabueñes, Asturias. [Zamora,M] Hospital Virgen de las Nieves. [Callejas,JL] Hospital Clínico San Cecilio, Granada. [Frutos,B] Hospital Fuenlabrada, Madrid. [Abdilla,O] Hospital La Ribera, Alzira, Valencia. [Fanlo,P] Clínica de Navarra. [García-Sánchez,I] Hospital Infanta Leonor, Madrid. [López-Dupla,M] Hospital Joan XXIII, Tarragona. [Sopeña,B] Centro Hospitalário Vigo. [Pérez-Iglesias,A] Hospital Ourense, Galicia, Spain. [Bosch,JA] Spanish Registry of systemic vasculitis (REVAS) from the Autoimmune Diseases Study Group (GEAS) of the Spanish Society of Internal Medicine (SEMI)., Institut Català de la Salut, [Solans-Laqué R] Hospital Universitari Vall d'Hebron, Barcelona, Spain. [Fraile G] Hospital Ramón y Cajal, Madrid, Spain. [Rodriguez-Carballeira M] Mútua Terrassa, Barcelona, Spain. [Caminal L] Hospital Central de Asturias, Oviedo, Spain. [Castillo MJ] Hospital Virgen del Rocío, Sevilla, Spain. [Martínez-Valle F, Zamora M, Bosch JA] Hospital Universitari Vall d'Hebron, Barcelona, Spain., and Vall d'Hebron Barcelona Hospital Campus
Subjects: Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::Disease Attributes::Recurrence [Medical Subject Headings], Male, Other subheadings::Other subheadings::/epidemiology [Other subheadings], calidad, acceso y evaluación de la atención sanitaria::calidad de la atención sanitaria::factores epidemiológicos::comorbilidad [ATENCIÓN DE SALUD], Phenomena and Processes::Physical Phenomena::Time::Time Factors [Medical Subject Headings], España, Comorbidity, Estudios longitudinales, ANCA-associated vasculitides, Gastroenterology, Anticuerpos anticitoplasma de neutrófilos, Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans [Medical Subject Headings], 0302 clinical medicine, Longitudinal Studies, Young adult, Anciano de 80 o más años, Masculino, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Morbidity::Prevalence [Medical Subject Headings], Adolescente, Aged, 80 and over, General Medicine, eosinophilic granulomatosis with polyangiitis, Geographic Locations::Europe::Spain [GEOGRAPHICALS], Cohort, Microscopic polyangiitis, Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Blood Proteins::Immunoproteins::Immunoglobulins::Antibodies::Autoantibodies::Antibodies, Antineutrophil Cytoplasmic [Medical Subject Headings], Vasculitis asociada a anticuerpos citoplasmáticos antineutrófilos, medicine.medical_specialty, mortality predictors, Anciano, Recurrencia, Check Tags::Male [Medical Subject Headings], 03 medical and health sciences, Estudios retrospectivos, Other subheadings::Other subheadings::Other subheadings::/mortality [Other subheadings], Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Study Characteristics as Topic::Epidemiologic Studies::Cohort Studies::Longitudinal Studies [Medical Subject Headings], Humans, Persons::Persons::Age Groups::Adult [Medical Subject Headings], Aged, Retrospective Studies, Mediana edad, Otros calificadores::Otros calificadores::Otros calificadores::/mortalidad [Otros calificadores], granulomatosis with polyangiitis, Eosinophilic granulomatosis with polyangiitis, Persons::Persons::Age Groups::Adult::Middle Aged [Medical Subject Headings], Mortality predictors, medicine.disease, Diseases::Immune System Diseases::Autoimmune Diseases::Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis [Medical Subject Headings], Check Tags::Female [Medical Subject Headings], Persons::Persons::Age Groups::Adult::Aged::Aged, 80 and over [Medical Subject Headings], Granulomatosis with polyangiitis, Other subheadings::Other subheadings::/physiopathology [Other subheadings], Time Factors, Cardiovascular Diseases::Vascular Diseases::Vasculitis::Systemic Vasculitis::Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis [DISEASES], Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Study Characteristics as Topic::Epidemiologic Studies::Case-Control Studies::Retrospective Studies [Medical Subject Headings], Adulto joven, Recurrence, Health Care::Health Care Quality, Access, and Evaluation::Quality of Health Care::Epidemiologic Factors::Comorbidity [Medical Subject Headings], Prevalence, 030212 general & internal medicine, infections, Persons::Persons::Age Groups::Adult::Aged [Medical Subject Headings], microscopic polyangiitis, Adulto, Otros calificadores::Otros calificadores::/epidemiología [Otros calificadores], Femenino, Middle Aged, Comorbilidad, enfermedades cardiovasculares::enfermedades vasculares::vasculitis::vasculitis sistémica::vasculitis asociada a anticuerpos anticitoplasma de neutrófilos [ENFERMEDADES], Treatment Outcome, outcome, Ubicaciones Geográficas::Europa (Continente)::España [DENOMINACIONES GEOGRÁFICAS], Female, Estudios de seguimiento, Vasculitis, Persons::Persons::Age Groups::Adult::Young Adult [Medical Subject Headings], medicine.drug, Research Article, Adult, Otros calificadores::Otros calificadores::/fisiopatología [Otros calificadores], Cyclophosphamide, Adolescent, Spanish people, Observational Study, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Infections, Persons::Persons::Age Groups::Adolescent [Medical Subject Headings], Antibodies, Antineutrophil Cytoplasmic, Young Adult, Comorbiditat, Internal medicine, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Study Characteristics as Topic::Epidemiologic Studies::Cohort Studies::Longitudinal Studies::Follow-Up Studies [Medical Subject Headings], medicine, Health Care Quality, Access, and Evaluation::Quality of Health Care::Epidemiologic Factors::Comorbidity [HEALTH CARE], Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Prognosis::Treatment Outcome [Medical Subject Headings], 030203 arthritis & rheumatology, Geographical Locations::Geographic Locations::Europe::Spain [Medical Subject Headings], business.industry, Retrospective cohort study, Surgery, Outome, Spain, Factores de tiempo, Vasculitis - Espanya, Resultado del tratamiento, business, Prevalencia, Follow-Up Studies
Abstract: The aim of this study was to describe the clinical characteristics of ANCA-associated vasculitides (AAV) at presentation, in a wide cohort of Spanish patients, and to analyze the impact of the vasculitis type, ANCA specificity, prognostic factors, and treatments administered at diagnosis, in the outcome. A total of 450 patients diagnosed between January 1990 and January 2014 in 20 Hospitals from Spain were included. Altogether, 40.9% had granulomatosis with polyangiitis (GPA), 37.1% microscopic polyangiitis (MPA), and 22% eosinophilic granulomatosis with polyangiitis (EGPA). The mean age at diagnosis was 55.6 +/- 17.3 years, patients with MPA being significantly older (P < 0.001). Fever, arthralgia, weight loss, respiratory, and ear-nose-throat (ENT) symptoms, were the most common at disease onset. ANCAs tested positive in 86.4% of cases: 36.2% C-ANCA-PR3 and 50.2% P-ANCA-MPO. P-ANCA-MPO was significantly associated with an increased risk for renal disease (OR 2.6, P < 0.001) and alveolar hemorrhage (OR 2, P = 0.010), while C-ANCA-PR3 was significantly associated with an increased risk for ENT (OR 3.4, P < 0.001) and ocular involvement (OR 2.3, P = 0.002). All patients received corticosteroids (CS) and 74.9% cyclophosphamide (CYC). The median follow-up was 82 months (IQR 100.4). Over this period 39.9% of patients suffered bacterial infections and 14.6% opportunistic infections, both being most prevalent in patients with high-cumulated doses of CYC and CS (P < 0.001). Relapses were recorded in 36.4% of cases with a mean rate of 2.5 +/- 2.3, and were more frequent in patients with C-ANCA-PR3 (P = 0.012). The initial disease severity was significantly associated with mortality but not with the occurrence of relapses. One hundred twenty-nine (28.7%) patients (74 MPA, 41 GPA, 14 EGPA) died. The mean survival was 58 months (IQR 105) and was significantly lower for patients with MPA (P < 0.001). Factors independently related to death were renal involvement (P = 0.010), cardiac failure (P = 0.029) and age over 65 years old (P < 0.001) at disease onset, and bacterial infections (P < 0.001). An improved outcome with significant decrease in mortality and treatment-related morbidity was observed in patients diagnosed after 2000, and was related to the implementation of less toxic regimens adapted to the disease activity and stage, and a drastic reduction in the cumulated CYC and CS dose.
Published: 2017

10. How immunological profle drives clinical phenotype of primary Sjögren’s syndrome at diagnosis: analysis of 10,500 patients (Sjögren Big Data Project)

Author: Brito Zerón, Pilar, Acar Denizli, Nihan, Ng, Wan Fai, Zeher, Margit, Rasmussen, Astrid, Mandl, Thomas, Seror, Raphaele, Xiaolin, Li, Baldini, Chiara, Gottenberg, Jaques, Danda, Debashish, Quartuccio, Luca, Priori, Roberta, Hernandez Molina, Gabriela, Armagan, Berkan, Kruize, Aike, Kwok, Seung Ki, Kvarnström, Marika, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Solans, R., Rischmueller, M., Suzuki, Y., Isenberg, D. A., Valim, V., Wiland, P., Nordmark, G., Fraile, G., and Retamozo, Maria Soledad
Subjects: Salivary gland biopsy, purl.org/becyt/ford/3.2 [https], Cryoglobulinaemia, Primary Sjögren’s syndrome, Hypocomplementaemia, purl.org/becyt/ford/3 [https], Ro/La autoantibodies
Abstract: To evaluate the influence of the main immunological markers on the disease phenotype at diagnosis in a large international cohort of patients with primary Sjögren´s syndrome (SjS).METHODS:The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. As a first step, baseline clinical information from leading centres on clinical research in SjS of the 5 continents was collected. The centres shared a harmonised data architecture and conducted cooperative online efforts in order to refine collected data under the coordination of a big data statistical team. Inclusion criteria were the fulfillment of the 2002 classification criteria. Immunological tests were carried out using standard commercial assays.RESULTS:By January 2018, the participant centres had included 10,500 valid patients from 22 countries. The cohort included 9,806 (93%) women and 694 (7%) men, with a mean age at diagnosis of primary SjS of 53 years, mainly White (78%) and included from European countries (71%). The frequency of positive immunological markers at diagnosis was 79.3% for ANA, 73.2% for anti-Ro, 48.6% for RF, 45.1% for anti- La, 13.4% for low C3 levels, 14.5% for low C4 levels and 7.3% for cryoglobulins. Positive autoantibodies (ANA, Ro, La) correlated with a positive result in salivary gland biopsy, while hypocomplementaemia and especially cryoglo-bulinaemia correlated with systemic activity (mean ESSDAI score of 17.7 for cryoglobulins, 11.3 for low C3 and 9.2 for low C4, in comparison with 3.8 for negative markers). The immunological markers with a great number of statistically-significant associations (p
Published: 2018

11. Influence of epidemiology and ethnicity on systemic expression of primary Sjögren syndrome in 9974 patients

Author: Retamozo, Soledad, Acar-Denizli, Nihan, Fai Ng, W, Zeher, M, Rasmussen, A, Seror, Raphaèle, Li, X, Baldini, C, Gottenberg, Jacques-Eric, Danda, D, Quartuccio, Luca, Priori, R, Hernandez-Molina, G, Armagan, B, Kruize, Aike A, Kwok, S-K, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Solans, R., Rischmueller, Maureen, Mandl, T, Suzuki, Y, Isenberg, David, Valim, V, Wiland, Piotr, Nordmark, Gunnel, Fraile, G, Bootsma, Hendrika, Nakamura, T, Giacomelli, R., Devauchelle-Pensec, Valérie, Hofauer, Benedikt, Bombardieri, Michele, Fernandes Moça Trevisani, Virginia, Hammenfors, D, S.G., Pasoto, Gheita, Tamer A, Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Brito-Zerón, Pilar, Ramos-Casals, Manel, Rheumatology Unit, Cordoba (Institute University of Biomedical Sciences University of Cordoba (IUCBC), Hospital Clinic (IDIBAPS), INICSA, UNC, CONICED, Cordoba, Mimar Sinan Üniversitesi, Newcastle University [Newcastle], University of Debrecen [Hungary], Oklahoma Medical Research Foundation, Oklahoma Medical Research Foundation (OMRF), Université Paris Sud (Paris 11), Anhui Provincial Hosp, University of Pisa - Università di Pisa, Les Hôpitaux Universitaires de Strasbourg (HUS), Christian Medical College & Hospital, Rheumatology Clinic, Udine (DSMB), Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome], INCMNSS, Mexico, Hacettepe University = Hacettepe Üniversitesi, University Medical Center [Utrecht], Catholic University of Korea, Unit of Experimental Rheumatology, Stockhom (Department of Medicine), Department of Rheumatology, University Medical Centre, Service de médecine interne [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Università degli Studi di Perugia (UNIPG), Vall d'Hebron University Hospital [Barcelona], The University of Western Australia (UWA), Lund University, Malmö University Hospital, Kanazawa Medical University, Centre for Rheumatology - London, Federal University of Espírito Santo, Wroclaw Medical University, Uppsala Universitet [Uppsala], Hospital Universitario Ramón y Cajal [Madrid], Universidad de Alcalá - University of Alcalá (UAH), Department of Rheumatology and Clinical Immunology Groningen (Dep Rheum - GRONINGEN), University Medical Center Groningen [Groningen] (UMCG), Nagasaki University, University of L'Aquila [Italy] (UNIVAQ), CHRU Brest - Service de Rhumatologie (CHU - BREST - Rhumato), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Lymphocyte B et Auto-immunité (LBAI), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO), Hals-Nasen-Ohrenklinik und Poliklinik, Munchen, Queen Mary University London, Université Fédérale de São Paulo (Unifesp), Haukeland University Hospital, University of Bergen (UiB), Hospital das Clinicas, University of Sao Paulo School of Medicine, Cairo University - Faculty of Medicine, Messina and Milan Univ, Milan, Service de Rhumatologie [CHU de Montpellier], CHU Montpellier, German Hosp, Buenos Aires, H. CIMA-Sanitas, Barcelona, and Michel, Geneviève
Subjects: [SDV] Life Sciences [q-bio], [SDV]Life Sciences [q-bio], ComputingMilieux_MISCELLANEOUS
Abstract: International audience
Published: 2018

12. Sjögren big data project, the first example of data sharing in autoimmune diseases: analysis of 10475 worldwide patients

Author: Retamozo, Soledad, Acar-Denizli, Nihan, Fai Ng, W, Zeher, M, Rasmussen, A, Mandl, T, Seror, Raphaèle, Li, X, Baldini, C, Gottenberg, Jacques-Eric, Danda, D, Quartuccio, Luca, A, Minniti, Hernandez-Molina, Gabriela, Kalyoncu, Umut, Kruize, Aike A, Kwok, S-K, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartolini, E, Solans, R., Rischmueller, Maureen, Suzuki, Y, Isenberg, David, Valim, V, Wiland, Piotr, Nordmark, Gunnel, Fraile, G, Bootsma, Hendrika, Nakamura, T, Giacomelli, R., Devauchelle-Pensec, Valérie, Hofauer, Benedikt, Bombardieri, Michèle, Fernandes Moça Trevisani, Virginia, Hammenfors, D, S.G., Pasoto, Gheita, Tamer A, Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Brito-Zerón, Pilar, Ramos-Casals, Manuel, Hospital Clinic (IDIBAPS), Rheumatology Unit, Cordoba (Institute University of Biomedical Sciences University of Cordoba (IUCBC), INICSA, UNC, CONICED, Cordoba, Mimar Sinan Üniversitesi, Newcastle University [Newcastle], University of Debrecen [Hungary], Oklahoma Medical Research Foundation, Oklahoma Medical Research Foundation (OMRF), Lund University, Malmö University Hospital, Université Paris Sud (Paris 11), Anhui Provincial Hosp, University of Pisa - Università di Pisa, Les Hôptaux universitaires de Strasbourg (HUS), CHU Strasbourg, Christian Medical College & Hospital, Rheumatology Clinic, Udine (DSMB), Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome], Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán - National Institute of Medical Science and Nutrition Salvador Zubiran [Mexico], Hacettepe University = Hacettepe Üniversitesi, University Medical Center [Utrecht], Catholic University of Korea, Unit of Experimental Rheumatology, Stockhom (Department of Medicine), Department of Rheumatology, University Medical Centre, Service de médecine interne [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université dePerugia, Vall d'Hebron University Hospital [Barcelona], The University of Western Australia (UWA), Kanazawa University (KU), Centre for Rheumatology - London, Federal University of Espírito Santo, Wroclaw Medical University, Department of Medical Sciences, Hospital Universitario Ramón y Cajal [Madrid], Universidad de Alcalá - University of Alcalá (UAH), Department of Rheumatology and Clinical Immunology Groningen (Dep Rheum - GRONINGEN), University Medical Center Groningen [Groningen] (UMCG), Nagasaki University, University of L'Aquila [Italy] (UNIVAQ), CHRU Brest - Service de Rhumatologie (CHU - BREST - Rhumato), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Lymphocyte B et Auto-immunité (LBAI), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO), Hals-Nasen-Ohrenklinik und Poliklinik, Munchen, The William Harvey Research Institute, Barts and The London School of Medicine, Queen Mary University of London (QMUL), Université Fédérale de São Paulo (Unifesp), Haukeland University Hospital, University of Bergen (UiB), Hospital das Clinicas, University of Sao Paulo School of Medicine, Cairo University - Faculty of Medicine, Messina and Milan Univ, Milan, Service de Rhumatologie [CHU de Montpellier], CHU Montpellier, German Hosp, Buenos Aires, H. CIMA-Sanitas, Barcelona, CELLEX-IDIBAPS Department of Autoimmune Diseases, Barcelona, and Michel, Geneviève
Subjects: [SDV] Life Sciences [q-bio], [SDV]Life Sciences [q-bio], ComputingMilieux_MISCELLANEOUS
Abstract: International audience
Published: 2018

13. Venous thrombosis and relapses in patients with Behcet's disease. Descriptive analysis from Spanish network of Behcet's disease (REGEB cohort)

Author: Rodriguez-Carballeira, M, Solans, R, Larranaga, JR, Garcia-Hernandez, FJ, Rios-Fernandez, R, Nieto, J, Solanich, X, Martinez-Valle, F, Fonseca, E, Munoz, FJ, Fraile, G, de Escalante, B, Boldova, R, Hurtado, R, Espinosa, G, Callejas, JL, Hernandez, FG, Garrido, SL, Vidaller, A, de la Torre, RG, Herranz, MT, Todoli, J, Munoz-Rodriguez, F, Fanlo, P, Garcia-Sanchez, I, Trapiella, L, de Miguel, B, Domingo, S, Vilaplana, R, and Cusacovich, I
Subjects: relapse, immunosuppression, thrombotic recurrence, Behcet's disease, anticoagulation, thrombosis
Abstract: Objective. To describe the characteristics of patients with Behfet's disease (BD) who presented with venous thrombosis. In addition, we identified the factors associated with this venous involvement and those related with recurrent venous thrombosis. Methods. Up to January 2015, 544 BD patients from 20 Spanish hospitals had been included in the REGEB (REGistro de la Enfermedad de Behqet as Spanish nomenclature). We selected those patients who presented venous thrombosis. Descriptive analysis was performed and factors related with venous thrombosis were identified. Results. Overall, 99 (18.2%) BD patients had vascular thrombosis, 91 (16.7%) of them (16.7%) involving venous vessels and 18 (19.7%) suffered from venous thrombotic relapse. Lower limbs were the most common location of deep venous thrombosis present in up to 60% of patients. In 12 (13.2%) patients, venous thrombosis affected two vascular territories simultaneously and in 6 (6.6%) the venous and arterial involvement coincided in time. Overall, at the diagnosis of venous thrombosis, 97.6% of patients presented concomitantly other clinical symptoms attributable to BD. In logistic regression multivariate analysis factors associated to venous thrombosis were male sex (Odds ratio [OR] 4.3, 95% confidence interval [CI] 2.5-7.7), erythema nodosum (OR 2.4, 95% CI 1.4-4.1), fever (OR 2.0, 95% CI 1.1-3.8), and central nervous system (CNS) involvement (OR 2.5, 95% CI 1.3-4.8). Considering relapses, CNS involvement was an independent risk factor according logistic regression. However, Cox multivariate analysis did not confirm this finding. Conclusion. We identified factors related with venous involvement in patients included in the REGEB cohort.
Published: 2018

14. How the different systemic organ involvements are overlapped in patients with primary Sjogren syndrome: analysis using a mathematical model

Author: Retamozo, (Retamozo, S, Soledad)(, 1, 2 3 ), Kostov, (Kostov, B, 4 ), Belchin), Zeher, (Zeher, M, 5 ), Margit), Sivils, (Sivils, K, 6 ), Kathy), Mandl, (Mandl, T, 7 ), Thomas), Seror, (Seror, R, Raphaele)(, 8, Li, 9, (Li, Xm, Xiaomei)( 10, ), Baldini, (Baldini, C, Chiara)( 11, ), Mariette, (Mariette, X, Xavier)(, 8, Gottenberg, 9, (Gottenberg, Je, Jacques-Eric)( 12, ), Danda, (Danda, D, Debashish)( 13, ), Priori, (Priori, R, Roberta)( 14, ), Quartuccio, (Quartuccio, L, Luca)( 15, ), Hernandez-Molina, (Hernandez-Molina, G, Gabriela)( 16, ), Armagan, (Armagan, B, Berkan)( 17, ), Kruize, (Kruize, Aa, ( 18 ), Aike A., Kwok, (Kwok, Sk, Seung-Ki)( 19, ), Wahren-Herlenius, (Wahren-Herlenius, M, Marie)(, 20, 21, ), Praprotnik, (Praprotnik, S, Sonja)( 22, ), Sene, (Sene, D, Damien)( 23, ), Bartoloni, (Bartoloni, E, Elena)( 24, ), Rischmueller, (Rischmueller, M, Maureen)( 25, ), Solans, (Solans, R, Roser)( 26, ), Suzuki, (Suzuki, Y, Yasunori)( 27, ), Isenberg, (Isenberg, D, David)( 28, ), Valim, (Valim, V, Valeria)( 29, ), Wiland, (Wiland, P, Piotr)( 30, ), Nordmark, (Nordmark, G, Gunnel)( 31, ), Fraile, (Fraile, G, Guadalupe)( 32, ), Bootsma, (Bootsma, H, Hendrika)( 33, ), Nakamura, (Nakamura, T, Takashi)( 34, ), Giacomelli, Roberto, (Giacomelli, R, Roberto)( 35, ), Devauchelle-Pensec, (Devauchelle-Pensec, V, Valerie)( 36, ), Hofauer, (Hofauer, B, Benedikt)( 37, ), Bombardieri, (Bombardieri, M, Michele)( 38, ), Trevisani, VFM (Moca Trevisani, Virginia Fernandes)( 39, ), Hammenfors, (Hammenfors, D, Daniel)(, 40, 41, ), Pasoto, (Pasoto, Sg, ( 42 ), Sandra G., Carsons, (Carsons, Se, ( 43 ), Steven E., Gheite, (Gheite, Ta, ( 44 ), Tamer A., Atzeni, (Atzeni, F, Fabiola)( 45, ), Morel, (Morel, J, Jacques)(, 46, 47, ), Vollenveider, (Vollenveider, C, Cristina)( 48, ), Brito-Zeron, (Brito-Zeron, P, Pilar)(, 1, 49, ), Ramos-Casals, (Ramos-Casals, M, and Manuel)
Published: 2018

15. How immunological profile drives clinical phenotype of primary Sjögren's syndrome at diagnosis: analysis of 10,500 patients (Sjögren Big Data Project)

Author: Brito-Zerón, P., Acar-Denizli, N., Ng, Wf, Zeher, M., Rasmussen, A., Mandl, T., Seror, R., Li, X., Baldini, C., Gottenberg, Je, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, Aa, Kwok, Sk, Marika Kvarnström, Praprotnik, S., Sène, D., Bartoloni, E., Solans, R., Rischmueller, M., Suzuki, Y., Isenberg, Da, Valim, V., Wiland, P., Nordmark, G., Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensec, V., Knopf, A., Bombardieri, M., Trevisani, Vf, Hammenfors, D., Pasoto, Sg, Retamozo, S., Gheita, Ta, Atzeni, F., Morel, J., Vollenveider, C., Horvath, If, Sivils, Kl, Olsson, P., Vita, S., Sánchez-Guerrero, J., Kilic, L., Wahren-Herlenius, M., Mariette, X., Ramos-Casals, M., Sjögren Big Data Consortium, Hôpital Bicêtre, Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Service de rhumatologie [Strasbourg], CHU Strasbourg-Hôpital de Hautepierre [Strasbourg], Hôpital Lariboisière, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Lariboisière-Fernand-Widal [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), CHRU Brest - Service de Rhumatologie (CHU - BREST - Rhumato), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Département de Rhumatologie[Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Lapeyronie, Institut de Génétique Moléculaire de Montpellier (IGMM), Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Hôpital Bicêtre-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris-Sud - Paris 11 (UP11), Herrada, Anthony, and Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS)
Subjects: Adult, Male, [SDV.MHEP.RSOA] Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, Complement C4, Complement C3, Middle Aged, Aged, Antibodies, Antinuclear, Autoantibodies, Biomarkers, Cryoglobulins, Female, Humans, Phenotype, Prognosis, Registries, Rheumatoid Factor, Sjogren's Syndrome, Antibodies, [SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, Antinuclear
Abstract: International audience; OBJECTIVES:To evaluate the influence of the main immunological markers on the disease phenotype at diagnosis in a large international cohort of patients with primary Sjögren's syndrome (SjS).METHODS:The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. As a first step, baseline clinical information from leading centres on clinical research in SjS of the 5 continents was collected. The centres shared a harmonised data architecture and conducted cooperative online efforts in order to refine collected data under the coordination of a big data statistical team. Inclusion criteria were the fulfillment of the 2002 classification criteria. Immunological tests were carried out using standard commercial assays.RESULTS:By January 2018, the participant centres had included 10,500 valid patients from 22 countries. The cohort included 9,806 (93%) women and 694 (7%) men, with a mean age at diagnosis of primary SjS of 53 years, mainly White (78%) and included from European countries (71%). The frequency of positive immunological markers at diagnosis was 79.3% for ANA, 73.2% for anti-Ro, 48.6% for RF, 45.1% for anti- La, 13.4% for low C3 levels, 14.5% for low C4 levels and 7.3% for cryoglobulins. Positive autoantibodies (ANA, Ro, La) correlated with a positive result in salivary gland biopsy, while hypocomplementaemia and especially cryoglo-bulinaemia correlated with systemic activity (mean ESSDAI score of 17.7 for cryoglobulins, 11.3 for low C3 and 9.2 for low C4, in comparison with 3.8 for negative markers). The immunological markers with a great number of statistically-significant associations (p
Published: 2018

16. Systemic Sjogren presenting without sicca syndrome: characterization of 240 patients according to the new 2017 ACR/EULAR Classification Criteria

Author: Retamozo, (Retamozo, S, Soledad)(, 1, 2 3 ), Acar-Denizli, (Acar-Denizli, N, 4 ), Nihan), Zeher, (Zeher, M, 5 ), Margit), Sivils, (Sivils, K, 6 ), Kathy), Mandl, (Mandl, T, 7 ), Thomas), Seror, (Seror, R, Raphaele)(, 8, Li, 9, (Li, Xm, Xiaomei)( 10, ), Baldini, (Baldini, C, Chiara)( 11, ), Mariette, (Mariette, X, Xavier)(, 8, Gottenberg, 9, (Gottenberg, Je, Jacques-Eric)( 12, ), Danda, (Danda, D, Debashish)( 13, ), Priori, (Priori, R, Roberta)( 14, ), Quartuccio, (Quartuccio, L, Luca)( 15, ), Hernandez-Molina, (Hernandez-Molina, G, Gabriela)( 16, ), Armagan, (Armagan, B, Berkan)( 17, ), Kruize, (Kruize, Aa, ( 18 ), Aike A., Kwok, (Kwok, Sk, Seung-Ki)( 19, ), Wahren-Herlenius, (Wahren-Herlenius, M, Marie)(, 20, 21, ), Praprotnik, (Praprotnik, S, Sonja)( 22, ), Sene, (Sene, D, Damien)( 23, ), Bartoloni, (Bartoloni, E, Elena)( 24, ), Rischmueller, (Rischmueller, M, Maureen)( 25, ), Solans, (Solans, R, Roser)( 26, ), Suzuki, (Suzuki, Y, Yasunori)( 27, ), Isenberg, (Isenberg, D, David)( 28, ), Valim, (Valim, V, Valeria)( 29, ), Wiland, (Wiland, P, Piotr)( 30, ), Nordmark, (Nordmark, G, Gunnel)( 31, ), Fraile, (Fraile, G, Guadalupe)( 32, ), Bootsma, (Bootsma, H, Hendrika)( 33, ), Nakamura, (Nakamura, T, Takashi)( 34, ), Giacomelli, Roberto, (Giacomelli, R, Roberto)( 35, ), Devauchelle-Pensec, (Devauchelle-Pensec, V, Valerie)( 36, ), Hofauer, (Hofauer, B, Benedikt)( 37, ), Bombardieri, (Bombardieri, M, Michele)( 38, ), Trevisani, VFM (Moca Trevisani, Virginia Fernandes)( 39, ), Hammenfors, (Hammenfors, D, Daniel)(, 40, 41, ), Pasoto, (Pasoto, Sg, ( 42 ), Sandra G., Carsons, (Carsons, Se, ( 43 ), Steven E., Gheita, (Gheita, Ta, ( 44 ), Tamer A., Atzeni, (Atzeni, F, Fabiola)( 45, ), Morel, (Morel, J, Jacques)(, 46, 47, ), Vollenveider, (Vollenveider, C, Cristina)( 48, ), Brito-Zeron, (Brito-Zeron, P, Pilar)(, 1, 49, ), Ramos-Casals, (Ramos-Casals, M, and Manuel)
Published: 2018

17. How ethnicity modifies systemic activity of primary Sjogren syndrome: analysis of baseline ESSDAI scores in a multi-ethnic international cohort

Author: Retamozo, (Retamozo, S, Soledad)(, 1, 2 3 ), Kostov, (Kostov, B, 4 ), Belchin), Zeher, (Zeher, M, 5 ), Margit), Sivils, (Sivils, K, 6 ), Kathy), Mandl, (Mandl, T, 7 ), Thomas), Seror, (Seror, R, Raphaele)(, 8, Li, 9, (Li, X, Xiaomei)( 10, ), Baldini, (Baldini, C, Chiara)( 11, ), Mariette, (Mariette, X, Xavier)(, 8, Gottenberg, 9, (Gottenberg, Je, Jacques-Eric)( 12, ), Danda, (Danda, D, Debashish)( 13, ), Priori, (Priori, R, Roberta)( 14, ), Quartuccio, (Quartuccio, L, Luca)( 15, ), Hernandez-Molina, (Hernandez-Molina, G, Gabriela)( 16, ), Armagan, (Armagan, B, Berkan)( 17, ), Kruize, (Kruize, Aa, ( 18 ), Aike A., Kwok, (Kwok, Sk, Seung-Ki)( 19, ), Wahren-Herlenius, (Wahren-Herlenius, M, Marie)(, 20, 21, ), Praprotnik, (Praprotnik, S, Sonja)( 22, ), Sene, (Sene, D, Damien)( 23, ), Bartoloni, (Bartoloni, E, Elena)( 24, ), Rischmueller, (Rischmueller, M, Maureen)( 25, ), Solans, (Solans, R, Roser)( 26, ), Suzuki, (Suzuki, Y, Yasunori)( 27, ), Isenberg, (Isenberg, D, David)( 28, ), Valim, (Valim, V, Valeria)( 29, ), Wiland, (Wiland, P, Piotr)( 30, ), Nordmark, (Nordmark, G, Gunnel)( 31, ), Fraile, (Fraile, G, Guadalupe)( 32, ), Bootsma, (Bootsma, H, Hendrika)( 33, ), Nakamura, (Nakamura, T, Takashi)( 34, ), Giacomelli, Roberto, (Giacomelli, R, Roberto)( 35, ), Devauchelle-Pensec, (Devauchelle-Pensec, V, Valerie)( 36, ), Hofauer, (Hofauer, B, Benedikt)( 37, ), Bombardieri, (Bombardieri, M, Michele)( 38, ), Trevisani, VFM (Moca Trevisani, Virginia Fernandes)( 39, ), Hammenfors, (Hammenfors, D, Daniel)(, 40, 41, ), Pasoto, (Pasoto, Sg, ( 42 ), Sandra G., Carsons, (Carsons, Se, ( 43 ), Steven E., Gheita, (Gheita, Ta, ( 44 ), Tamer A., Atzeni, (Atzeni, F, Fabiola)( 45, ), Morel, (Morel, J, Jacques)(, 46, 47, ), Vollenveider, (Vollenveider, C, Cristina)( 48, ), Brito-Zeron, (Brito-Zeron, P, Pilar)(, 1, 49, ), Ramos-Casals, (Ramos-Casals, M, and Manuel)
Published: 2018

18. Clinical and immunological disease patterns of primary Sjogren syndrome driven by gender and age at diagnosis

Author: Retamozo, (Retamozo, S, Soledad)(, 1, 2 3 ), Kostov, (Kostov, B, 4 ), Belchin), Zeher, (Zeher, M, 5 ), Margit), Sivils, (Sivils, K, 6 ), Kathy), Mandl, (Mandl, T, 7 ), Thomas), Seror, (Seror, R, Raphaele)(, 8, Li, 9, (Li, Xm, Xiaomei)( 10, ), Baldini, (Baldini, C, Chiara)( 11, ), Mariette, (Mariette, X, Xavier)(, 8, Gottenberg, 9, (Gottenberg, Je, Jacques-Eric)( 12, ), Danda, (Danda, D, Debashish)( 13, ), Priori, (Priori, R, Roberta)( 14, ), Quartuccio, (Quartuccio, L, Luca)( 15, ), Hernandez-Molina, (Hernandez-Molina, G, Gabriela)( 16, ), Armagan, (Armagan, B, Berkan)( 17, ), Kruize, (Kruize, Aa, ( 18 ), Aike A., Kwok, (Kwok, Sk, Seung-Ki)( 19, ), Wahren-Herlenius, (Wahren-Herlenius, M, Marie)(, 20, 21, ), Praprotnik, (Praprotnik, S, Sonja)( 22, ), Sene, (Sene, D, Damien)( 23, ), Bartoloni, (Bartoloni, E, Elena)( 24, ), Rischmueller, (Rischmueller, M, Maureen)( 25, ), Solans, (Solans, R, Roser)( 26, ), Suzuki, (Suzuki, Y, Yasunori)( 27, ), Isenberg, (Isenberg, D, David)( 28, ), Valim, (Valim, V, Valeria)( 29, ), Wiland, (Wiland, P, Piotr)( 30, ), Nordmark, (Nordmark, G, Gunnel)( 31, ), Fraile, (Fraile, G, Guadalupe)( 32, ), Bootsma, (Bootsma, H, Hendrika)( 33, ), Nakamura, (Nakamura, T, Takashi)( 34, ), Giacomelli, Roberto, (Giacomelli, R, Roberto)( 35, ), Devauchelle-Pensec, (Devauchelle-Pensec, V, Valerie)( 36, ), Hofauer, (Hofauer, B, Benedikt)( 37, ), Bombardieri, (Bombardieri, M, Michele)( 38, ), Trevisani, VFM (Moca Trevisani, Virginia Fernandes)( 39, ), Hammenfors, (Hammenfors, D, Daniel)(, 40, 41, ), Pasoto, (Pasoto, Sg, ( 42 ), Sandra G., Carsons, (Carsons, Se, ( 43 ), Steven E., Gheita, (Gheita, Ta, ( 44 ), Tamer A., Atzeni, (Atzeni, F, Fabiola)( 45, ), Morel, (Morel, J, Jacques)(, 46, 47, ), Vollenveider, (Vollenveider, C, Cristina)( 48, ), Brito-Zeron, (Brito-Zeron, P, Pilar)(, 1, 49, ), Ramos-Casals, (Ramos-Casals, M, and Manuel)
Published: 2018

19. A North-South Worldwide Gradient in Systemic Activity of Primary Sjögren Syndrome : Increased Severe Disease in Patients from Southern Countries

Author: Retamozo, S., Acar-Denizli, N., Ng, W. Fai, Zeher, M., Rasmussen, A., Seror, R., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, A. A., Kwok, S. -K, Wahren-Herlenius, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Rischmueller, M., Mandi, T., Suzuki, Y., Isenberg, D., Valim, V., Wiland, P., Nordmark, Gunnel, Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Fernandes Moca Trevisani, V., Hammenfors, D., Pasoto, S. G., Gheita, T. A., Atzeni, F., Morel, J., Vollenveider, C., Brito-Zeron, P., Ramos-Casals, M., Retamozo, S., Acar-Denizli, N., Ng, W. Fai, Zeher, M., Rasmussen, A., Seror, R., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, A. A., Kwok, S. -K, Wahren-Herlenius, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Rischmueller, M., Mandi, T., Suzuki, Y., Isenberg, D., Valim, V., Wiland, P., Nordmark, Gunnel, Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Fernandes Moca Trevisani, V., Hammenfors, D., Pasoto, S. G., Gheita, T. A., Atzeni, F., Morel, J., Vollenveider, C., Brito-Zeron, P., and Ramos-Casals, M.
Published: 2018
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20. Influence of epidemiology and ethnicity on systemic expression of primary Sjögren syndrome in 9974 patients

Author: Retamozo, S., Acar-Denizli, N., Ng, W. Fai, Zeher, M., Rasmussen, A., Seror, R., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, A. A., Kwok, S. -K, Wahren-Herlenius, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Rischmueller, M., Mandl, T., Suzuki, Y., Isenberg, D., Valim, V., Wiland, P., Nordmark, Gunnel, Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensee, V., Hofauer, B., Bombardieri, M., Fernandes Moca Trevisani, V., Hammenfors, D., Pasoto, S. G., Gheita, T. A., Atzeni, F., Morel, J., Vollenveider, C., Brito-Zeron, P., Ramos-Casals, M., Retamozo, S., Acar-Denizli, N., Ng, W. Fai, Zeher, M., Rasmussen, A., Seror, R., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, A. A., Kwok, S. -K, Wahren-Herlenius, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Rischmueller, M., Mandl, T., Suzuki, Y., Isenberg, D., Valim, V., Wiland, P., Nordmark, Gunnel, Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensee, V., Hofauer, B., Bombardieri, M., Fernandes Moca Trevisani, V., Hammenfors, D., Pasoto, S. G., Gheita, T. A., Atzeni, F., Morel, J., Vollenveider, C., Brito-Zeron, P., and Ramos-Casals, M.
Published: 2018
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21. Baseline ESSDAI/DAS scores in 8061 patients with primary sjÖgren syndrome: characterization of systemic disease

Author: Brito-Zerόn, P, Acar-Denizli, N, Zeher, M, Rasmussen, A, Li, X, Baldini, C, Gottenberg, J-E, Danda, D, Quartuccio, L, Hernandez-Molina, G, Kruize, Aa, Park, S-H, Kvarnström, M, Praprotnik, S, Sene, D, Alunno, A, Solans, R, Mandl, T, Suzuki, Y, Rischmueller, M, Nordmark, G, Fraile, G, Wiland, P, Bootsma, H, Nakamura, T, Valim, V, Giacomelli, R, Seror, R, Devauchelle-Pensec, V, Hofauer, B, Bombardieri, M, Trevisani, V, Hammenfors, D, Minniti, A, Pasoto, Sg, Morel, J, Retamozo, S, Gheita, Ta, Atzeni, F, Vollenveider, C, Mariette, X, and Ramos-Casals, M
Published: 2017

22. Measurement Invariance of the Satisfaction With Life Scale Across 26 Countries

Author: Jang, S. Kim, E.S. Cao, C. Allen, T.D. Cooper, C.L. Lapierre, L.M. O’Driscoll, M.P. Sanchez, J.I. Spector, P.E. Poelmans, S.A.Y. Abarca, N. Alexandrova, M. Antoniou, A.-S. Beham, B. Brough, P. Carikci, I. Ferreiro, P. Fraile, G. Geurts, S. Kinnunen, U. Lu, C.-Q. Lu, L. Moreno-Velázquez, I.F. Pagon, M. Pitariu, H. Salamatov, V. Siu, O.-L. Shima, S. Schulmeyer, M.K. Tillemann, K. Widerszal-Bazyl, M. Woo, J.-M.
Abstract: The Satisfaction With Life Scale (SWLS) is a commonly used life satisfaction scale. Cross-cultural researchers use SWLS to compare mean scores of life satisfaction across countries. Despite the wide use of SWLS in cross-cultural studies, measurement invariance of SWLS has rarely been investigated, and previous studies showed inconsistent findings. Therefore, we examined the measurement invariance of SWLS with samples collected from 26 countries. To test measurement invariance, we utilized three measurement invariance techniques: (a) multigroup confirmatory factor analysis (MG-CFA), (b) multilevel confirmatory factor analysis (ML-CFA), and (c) alignment optimization methods. The three methods demonstrated that configural and metric invariances of life satisfaction held across 26 countries, whereas scalar invariance did not. With partial invariance testing, we identified that the intercepts of Items 2, 4, and 5 were noninvariant. Based on two invariant intercepts, factor means of countries were compared. Chile showed the highest factor mean; Spain and Bulgaria showed the lowest. The findings enhance our understanding of life satisfaction across countries, and they provide researchers and practitioners with practical guidance on how to conduct measurement invariance testing across countries. © 2017, © The Author(s) 2017.
Published: 2017

23. SAT0596 Thoracic involvement at diagnosis drives a differentiated clinical presentation of sarcoidosis: analysis of 1245 patients (SARCOGEAS-SEMI)

Author: Retamozo, S., primary, Brito-Zerón, P., additional, Pérez-Alvarez, R., additional, Kostov, B., additional, Feijoo Masso, C., additional, Fraile, G., additional, Gómez-de-la-Torre, R., additional, De-Escalante, B., additional, López-Dupla, M., additional, Alguacil, A., additional, Chara-Cervantes, J., additional, Pérez-Conesa, M., additional, Rascón, J., additional, Garcia-Morillo, J.S., additional, Perez-Guerrero, P., additional, Fonseca, E., additional, Akasbi, M., additional, Bonet, M., additional, Callejas, J.L., additional, De-la-Red, G., additional, Calvo, E., additional, Soler, C., additional, Peral-Gutiérrez, E., additional, Gómez-Cerezo, J.F., additional, Cruz-Caparrós, G., additional, Rodríguez-Fernández, S., additional, Pinilla, B., additional, Gato, A., additional, Rolo, A., additional, Morcillo, C., additional, Robles, A., additional, Ojeda, I., additional, Vives, M.J., additional, de-Miguel, B., additional, Penadés, M., additional, De-Vicente, M., additional, Bosch, X., additional, Pérez-de-Lis, M., additional, González-García, A., additional, Yllera, C., additional, Gracia-Tello, B., additional, Perez-Gonzalez, A., additional, Pedrosa, M., additional, Tolosa, C., additional, Pallarés, L., additional, and Ramos-Casals, M., additional
Published: 2018
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24. SAT0457 SjÖgren big data project, the first example of data sharing in autoimmune diseases: analysis of 10475 worldwide patients

Author: Retamozo, S., primary, Acar-Denizli, N., additional, Fai Ng, W., additional, Zeher, M., additional, Rasmussen, A., additional, Mandl, T., additional, Seror, R., additional, Li, X., additional, Baldini, C., additional, Gottenberg, J.-E., additional, Danda, D., additional, Quartuccio, L., additional, Minniti, A., additional, Hernandez-Molina, G., additional, Kalyoncu, U., additional, Kruize, A.A., additional, Kwok, S.-K., additional, Wahren-Herlenius, M., additional, Praprotnik, S., additional, Sene, D., additional, Bartoloni, E., additional, Solans, R., additional, Rischmueller, M., additional, Suzuki, Y., additional, Isenberg, D., additional, Valim, V., additional, Wiland, P., additional, Nordmark, G., additional, Fraile, G., additional, Bootsma, H., additional, Nakamura, T., additional, Giacomelli, R., additional, Devauchelle-Pensec, V., additional, Hofauer, B., additional, Bombardieri, M., additional, Fernandes Moça Trevisani, V., additional, Hammenfors, D., additional, Pasoto, S.G., additional, Gheita, T.A., additional, Atzeni, F., additional, Morel, J., additional, Vollenveider, C., additional, Brito-Zerón, P., additional, and Ramos-Casals, M., additional
Published: 2018
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25. Analysis Of 9302 Patients From The Big Data International Primary Sjogren Syndrome Cohort : Clinical Presentation At Diagnosis Of European Vs Non-European Patients

Author: Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Priori, R., Quartuccio, L., Hernandez-Molina, G., Kruize, A., Park, S. -H, Kvarnstrom, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Suzuki, Y., Isenberg, D., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Sebastian, A., Vissink, A., Nakamura, T., Valim, V., Giacomelli, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Trevisani, V., Hammenfors, D., Carsons, S. E., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Priori, R., Quartuccio, L., Hernandez-Molina, G., Kruize, A., Park, S. -H, Kvarnstrom, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Suzuki, Y., Isenberg, D., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Sebastian, A., Vissink, A., Nakamura, T., Valim, V., Giacomelli, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Trevisani, V., Hammenfors, D., Carsons, S. E., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., and Ramos-Casals, M.
Published: 2017
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26. Baseline Essdai/ Das Scores In 8061 Patients With Primary Sjögren Syndrome : Characterization Of Systemic Disease

Author: Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Li, X., Baldini, C., Gottenberg, J-E, Danda, D., Quartuccio, L., Hernandez-Molina, G., Kruize, A. A., Park, S-H, Kvarnstrom, M., Praprotnik, S., Sene, D., Alunno, A., Solans, R., Mandl, T., Suzuki, Y., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Wiland, P., Bootsma, H., Nakamura, T., Valim, V., Giacomelli, R., Seror, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Trevisani, V., Hammenfors, D., Minniti, A., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Li, X., Baldini, C., Gottenberg, J-E, Danda, D., Quartuccio, L., Hernandez-Molina, G., Kruize, A. A., Park, S-H, Kvarnstrom, M., Praprotnik, S., Sene, D., Alunno, A., Solans, R., Mandl, T., Suzuki, Y., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Wiland, P., Bootsma, H., Nakamura, T., Valim, V., Giacomelli, R., Seror, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Trevisani, V., Hammenfors, D., Minniti, A., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., and Ramos-Casals, M.
Published: 2017
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27. Predicting Survival In 6240 Patients With Primary Sjögren' Syndrome (Big Data Sjögren Project)

Author: Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Li, X., Baldini, C., Gottenberg, J-E, Danda, D., Quartuccio, L., Hernandez-Molina, G., Kruize, A. A., Park, S-H, Kvarnstrom, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Mandl, T., Suzuki, Y., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Sebastian, A., Bootsma, H., Nakamura, T., Valim, V., Giacomelli, R., Seror, R., Devauchelle-Pensec, V., Hofauer, B., Bombaidieri, M., Trevisani, V., Hammenfors, D., Priori, R., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Li, X., Baldini, C., Gottenberg, J-E, Danda, D., Quartuccio, L., Hernandez-Molina, G., Kruize, A. A., Park, S-H, Kvarnstrom, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Mandl, T., Suzuki, Y., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Sebastian, A., Bootsma, H., Nakamura, T., Valim, V., Giacomelli, R., Seror, R., Devauchelle-Pensec, V., Hofauer, B., Bombaidieri, M., Trevisani, V., Hammenfors, D., Priori, R., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., and Ramos-Casals, M.
Published: 2017
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28. Measurement invariance of the Satisfaction With Life Scale across 26 countries

Author: Jang, S., Kim, E.S., Cao, C., Allen, T.D., Cooper, C.L., Lapierre, L.M., O'Driscoll, M.P., Sanchez, J.I., Spector, P.E., Poelmans, S.A.Y., Abarca, N., Alexandrova, M., Antoniou, A.S., Beham, B., Brough, P., Çarikçi, I., Ferreiro, P., Fraile, G., Geurts, S.A.E., Kinnunen, U., Lu, C.Q., Lu, L., Moreno-Velázquez, I.F., Pagon, M., Pitariu, H., Salamatov, V., Siu, O.L., Shima, S., Schulmeyer, M.K., Tillemann, K., Widerszal-Bazyl, M., Woo, J.M., Jang, S., Kim, E.S., Cao, C., Allen, T.D., Cooper, C.L., Lapierre, L.M., O'Driscoll, M.P., Sanchez, J.I., Spector, P.E., Poelmans, S.A.Y., Abarca, N., Alexandrova, M., Antoniou, A.S., Beham, B., Brough, P., Çarikçi, I., Ferreiro, P., Fraile, G., Geurts, S.A.E., Kinnunen, U., Lu, C.Q., Lu, L., Moreno-Velázquez, I.F., Pagon, M., Pitariu, H., Salamatov, V., Siu, O.L., Shima, S., Schulmeyer, M.K., Tillemann, K., Widerszal-Bazyl, M., and Woo, J.M.
Abstract: Item does not contain fulltext, The Satisfaction With Life Scale (SWLS) is a commonly used life satisfaction scale. Cross-cultural researchers use SWLS to compare mean scores of life satisfaction across countries. Despite the wide use of SWLS in cross-cultural studies, measurement invariance of SWLS has rarely been investigated, and previous studies showed inconsistent findings. Therefore, we examined the measurement invariance of SWLS with samples collected from 26 countries. To test measurement invariance, we utilized three measurement invariance techniques: (a) multigroup confirmatory factor analysis (MG-CFA), (b) multilevel confirmatory factor analysis (ML-CFA), and (c) alignment optimization methods. The three methods demonstrated that configural and metric invariances of life satisfaction held across 26 countries, whereas scalar invariance did not. With partial invariance testing, we identified that the intercepts of Items 2, 4, and 5 were noninvariant. Based on two invariant intercepts, factor means of countries were compared. Chile showed the highest factor mean; Spain and Bulgaria showed the lowest. The findings enhance our understanding of life satisfaction across countries, and they provide researchers and practitioners with practical guidance on how to conduct measurement invariance testing across countries.
Published: 2017

29. Characterization and risk estimate of cancer in patients with primary Sjogren syndrome

Author: Medicina i Cirurgia, Universitat Rovira i Virgili, Brito-Zerón, P; Kostov, B; Fraile, G; Caravia-Durán, D; Maure, B; Rascón, FJ; Zamora, M; Casanovas, A; Lopez-Dupla, M; Ripoll, M; Pinilla, B; Fonseca, E; Akasbi, M; de la Red, G; Duarte-Millán, MA; Fanlo, P; Guisado-Vasco, P; Pérez-Alvarez, R; Chamorro, AJ; Morcillo, C; Jiménez-Heredia, I; Sánchez-Berná, I; López-Guillermo, A; Ramos-Casals, M, Medicina i Cirurgia, Universitat Rovira i Virgili, and Brito-Zerón, P; Kostov, B; Fraile, G; Caravia-Durán, D; Maure, B; Rascón, FJ; Zamora, M; Casanovas, A; Lopez-Dupla, M; Ripoll, M; Pinilla, B; Fonseca, E; Akasbi, M; de la Red, G; Duarte-Millán, MA; Fanlo, P; Guisado-Vasco, P; Pérez-Alvarez, R; Chamorro, AJ; Morcillo, C; Jiménez-Heredia, I; Sánchez-Berná, I; López-Guillermo, A; Ramos-Casals, M
Abstract: Background: The purpose of this study is to characterize the risk of cancer in a large cohort of patients with primary Sjogren syndrome (SjS).Methods: We had analyzed the development of cancer in 1300 consecutive patients fulfilling the 2002 SjS classification criteria. The baseline clinical and immunological characteristics and systemic activity (ESSDAI scores) were assessed at diagnosis as predictors of cancer using Cox proportional hazards regression analysis adjusted for age at diagnosis and gender. The sex-and age-specific standardized incidence ratios (SIR) of cancer were estimated from 2012 Spanish mortality data.Results: After a mean follow-up of 91 months, 127 (9.8%) patients developed 133 cancers. The most frequent type of cancer was B-cell lymphoma (including 27 MALT and 19 non-MALT B-cell lymphomas). Systemic activity at diagnosis of primary SjS correlated with the risk of hematological neoplasia and cryoglobulins with a high risk of either B-cell or non-B-cell lymphoma subtypes. Patients with cytopenias had a high risk of non-MALT B-cell and non-B-cell cancer, while those with low C3 levels had a high risk of MALT lymphomas and those with monoclonal gammopathy and low C4 levels had a high risk of non-MALT lymphomas. The estimated SIR for solid cancer was 1. 13 and 11.02 for hematological cancer. SIRs for specific cancers were 36.17 for multiple myeloma and immunoproliferative diseases, 19.41 for Hodgkin lymphoma, 6.04 for other non-Hodgkin lymphomas, 5.17 for thyroid cancer, 4.81 for cancers of the lip and oral cavity, and 2.53 for stomach cancer.Conclusions: One third of cancers developed by patients with primary SjS are B-cell lymphomas. The prognostic factors identified at SjS diagnosis differed according to the subtype of B-cell lymphoma developed. Prim
Published: 2017

30. Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides Impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity

Author: Medicina i Cirurgia, Universitat Rovira i Virgili, Solans-Laqué, R; Fraile, G; Rodriguez-Carballeira, M; Caminal, L; Castillo, MJ; Martínez-Valle, F; Sáez, L; Rios, JJ; Solanich, X; Oristrell, J; Pasquau, F; Fonseca, E; Zamora, M; Callejas, JL; Frutos, B; Abdilla, M; Fanlo, P; García-Sánchez, I; López-Dupla, M; Sopeña, B; Pérez-Iglesias, A; Bosch, JA, Medicina i Cirurgia, Universitat Rovira i Virgili, and Solans-Laqué, R; Fraile, G; Rodriguez-Carballeira, M; Caminal, L; Castillo, MJ; Martínez-Valle, F; Sáez, L; Rios, JJ; Solanich, X; Oristrell, J; Pasquau, F; Fonseca, E; Zamora, M; Callejas, JL; Frutos, B; Abdilla, M; Fanlo, P; García-Sánchez, I; López-Dupla, M; Sopeña, B; Pérez-Iglesias, A; Bosch, JA
Abstract: The aim of this study was to describe the clinical characteristics of ANCA-associated vasculitides (AAV) at presentation, in a wide cohort of Spanish patients, and to analyze the impact of the vasculitis type, ANCA specificity, prognostic factors, and treatments administered at diagnosis, in the outcome.A total of 450 patients diagnosed between January 1990 and January 2014 in 20 Hospitals from Spain were included. Altogether, 40.9% had granulomatosis with polyangiitis (GPA), 37.1% microscopic polyangiitis (MPA), and 22% eosinophilic granulomatosis with polyangiitis (EGPA). The mean age at diagnosis was 55.6 +/- 17.3 years, patients with MPA being significantly older (P < 0.001). Fever, arthralgia, weight loss, respiratory, and ear-nose-throat (ENT) symptoms, were the most common at disease onset. ANCAs tested positive in 86.4% of cases: 36.2% C-ANCA-PR3 and 50.2% P-ANCA-MPO. P-ANCA-MPO was significantly associated with an increased risk for renal disease (OR 2.6, P < 0.001) and alveolar hemorrhage (OR 2, P = 0.010), while C-ANCA-PR3 was significantly associated with an increased risk for ENT (OR 3.4, P < 0.001) and ocular involvement (OR 2.3, P = 0.002). All patients received corticosteroids (CS) and 74.9% cyclophosphamide (CYC). The median follow-up was 82 months (IQR 100.4). Over this period 39.9% of patients suffered bacterial infections and 14.6% opportunistic infections, both being most prevalent in patients with high-cumulated doses of CYC and CS (P < 0.001). Relapses were recorded in 36.4% of cases with a mean rate of 2.5 +/- 2.3, and were more frequent in patients with C-ANCA-PR3 (P = 0.012). The initial disease severity was significantly associated with mortality but not with the occurrence of relapses. One hundred twenty-nine (28.7%) patients (74 MPA, 41 GPA
Published: 2017

31. DNA polymerase-alpha regulates the activation of type I interferons through cytosolic RNA:DNA synthesis

Author: Starokadomskyy, P, Gemelli, T, Rios, JJ, Xing, C, Wang, RC, Li, HY, Pokatayev, V, Dozmorov, I, Khan, S, Miyata, N, Fraile, G, Raj, P, Xu, Z, Xu, ZG, Ma, L, Lin, ZM, Wang, HJ, Yang, Y, Ben-Amitai, D, Orenstein, N, Mussaffi, H, Baselga, E, Tadini, G, Grunebaum, E, Sarajlija, A, Krzewski, K, Wakeland, EK, Yan, N, de la Morena, MT, Zinn, AR, and Burstein, E
Abstract: Aberrant nucleic acids generated during viral replication are the main trigger for antiviral immunity, and mutations that disrupt nucleic acid metabolism can lead to autoinflammatory disorders. Here we investigated the etiology of X-linked reticulate pigmentary disorder (XLPDR), a primary immunodeficiency with autoinflammatory features. We discovered that XLPDR is caused by an intronic mutation that disrupts the expression of POLA1, which encodes the catalytic subunit of DNA polymerase-alpha. Unexpectedly, POLA1 deficiency resulted in increased production of type I interferons. This enzyme is necessary for the synthesis of RNA: DNA primers during DNA replication and, strikingly, we found that POLA1 is also required for the synthesis of cytosolic RNA: DNA, which directly modulates interferon activation. Together this work identifies POLA1 as a critical regulator of the type I interferon response.
Published: 2016

32. La relación entre la conciliación de la vida familiar y laboral y las motivaciones al trabajo. Un estudio sobre los directivos en Argentina

Author: Fraile, G. (Guillermo) and López-Hernández, D. (Dolores)
Subjects: Trascendencia, Work and family balance, Complementariedad varón mujer, Motivation to work, Argentina, Ciencias Sociales [Materias Investigacion], Motivaciones al trabajo, Male female differences, Éxito conciliación trabajo y familia, Pérez López, Companies
Abstract: La familia y el trabajo son los dos ámbitos donde más se desarrolla la interacción humana. Una investigación realizada en Argentina en el año 2013, nos permite analizar cuáles son las prioridades en las agendas laborales y familiares de los directivos de empresa. La dificultad que manifiestan los hombres de empresa tener para lograr una vida personal más armónica, especialmente las mujeres, es aún un tema no resuelto. Por otro lado, la capacidad de desarrollar motivaciones al trabajo, priorizando el servicio a los demás, no es lo más característico del contenido de su tarea. Intentaremos profundizar el porqué de estas realidades y demostrar que ambas no son independientes, sino que existe una relación entre la principal motivación al trabajo y la capacidad de conciliar trabajo y familia. A través del desarrollo de indicadores de conciliación y trascendencia al trabajo y de diferentes análisis de correlación entre ellos, analizaremos si ambos comportamientos están relacionados. Work and family are two areas where most human interaction takes place. An investigation carried out in Argentina in 2013, allows us to analyze what are the priorities in the work and family agendas of managers of the company. The difficulty manifesting the businessmen have to achieve a personal life more harmonious, especially women, is still an unresolved issue. On the other hand, the ability to develop motivation to work, giving priority to service to others, is not the most characteristic of the content of their work. We will try to deepen why of these realities and demonstrate that both are not independent, but that there is a relationship between the main motivation to work and the ability to the work familiy balance. Through the development of indicators of conciliation and transcendence to work with different analysis of correlation between them, we will analyze if both behaviors are related.
Published: 2015

33. Ethnic Differences Strongly Influence The Phenotypic Expression of Primary Sjögren : Study of 7887 Patients from 20 Countries on 5 Continents (EULAR-SS Task Force Big Data Sjögren Project)

Author: Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Kruize, A., Valim, V., Kvarnstrom, M., Sene, D., Gerli, R., Praprotnik, S., Isenberg, D., Solans, R., Rischmueller, M., Park, S. -H, Nordmark, Gunnel, Suzuki, Y., Giacomelli, R., Saraux, A., Bombardieri, M., Hofauer, B., Bootsma, H., Hammenfors, D., Fraile, G., Carsons, S., Gheita, T., Morel, J., Vollenveider, C., Atzeni, F., Retamozo, S., Horvath, I. -F, Sivils, K., Theander, E., Sandhya, P., De Vita, S., Sanchez-Guerrero, J., van der Heijden, E., Moca-Trevisano, V., Wahren-Herlenius, M., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Kruize, A., Valim, V., Kvarnstrom, M., Sene, D., Gerli, R., Praprotnik, S., Isenberg, D., Solans, R., Rischmueller, M., Park, S. -H, Nordmark, Gunnel, Suzuki, Y., Giacomelli, R., Saraux, A., Bombardieri, M., Hofauer, B., Bootsma, H., Hammenfors, D., Fraile, G., Carsons, S., Gheita, T., Morel, J., Vollenveider, C., Atzeni, F., Retamozo, S., Horvath, I. -F, Sivils, K., Theander, E., Sandhya, P., De Vita, S., Sanchez-Guerrero, J., van der Heijden, E., Moca-Trevisano, V., Wahren-Herlenius, M., Mariette, X., and Ramos-Casals, M.
Published: 2016
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34. Worldwide Heterogeneous Diagnostic Approach To Primary Sjögren Syndrome in 8315 Patients (EULAR-SS Task Force Big Data Sjögren Project)

Author: Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Kruize, A., Valim, V., Kvarnstrom, M., Sene, D., Bartoloni, E., Praprotnik, S., Isenberg, D., Solans, R., Rischmueller, M., Kwok, S. -K, Nordmark, Gunnel, Suzuki, Y., Giacomelli, R., Devauchelle-Pensec, V., Bombardieri, M., Hofauer, B., Bootsma, H., Hammenfors, D., Fraile, G., Carsons, S., Gheita, T., Morel, J., Vollenveider, C., Atzeni, F., Retamozo, S., Horvath, I. -F, Sivils, K., Theander, E., Sandhya, P., De Vita, S., Sanchez-Guerrero, J., van der Heijden, E., Moca-Trevisano, V., Wahren-Herlenius, M., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Kruize, A., Valim, V., Kvarnstrom, M., Sene, D., Bartoloni, E., Praprotnik, S., Isenberg, D., Solans, R., Rischmueller, M., Kwok, S. -K, Nordmark, Gunnel, Suzuki, Y., Giacomelli, R., Devauchelle-Pensec, V., Bombardieri, M., Hofauer, B., Bootsma, H., Hammenfors, D., Fraile, G., Carsons, S., Gheita, T., Morel, J., Vollenveider, C., Atzeni, F., Retamozo, S., Horvath, I. -F, Sivils, K., Theander, E., Sandhya, P., De Vita, S., Sanchez-Guerrero, J., van der Heijden, E., Moca-Trevisano, V., Wahren-Herlenius, M., Mariette, X., and Ramos-Casals, M.
Published: 2016
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35. SAT0287 Ethnic Differences Strongly Influence The Phenotypic Expression of Primary Sjögren: Study of 7887 Patients from 20 Countries on 5 Continents (EULAR-SS Task Force Big Data Sjögren Project)

Author: Brito-Zerόn, P., primary, Acar-Denizli, N., additional, Zeher, M., additional, Rasmussen, A., additional, Seror, R., additional, Mandl, T., additional, Li, X., additional, Baldini, C., additional, Gottenberg, J.-E., additional, Danda, D., additional, Quartuccio, L., additional, Priori, R., additional, Hernández-Molina, G., additional, Kruize, A., additional, Valim, V., additional, Kvarnstrom, M., additional, Sene, D., additional, Gerli, R., additional, Praprotnik, S., additional, Isenberg, D., additional, Solans, R., additional, Rischmueller, M., additional, Park, S.-H., additional, Nordmark, G., additional, Suzuki, Y., additional, Giacomelli, R., additional, Saraux, A., additional, Bombardieri, M., additional, Hofauer, B., additional, Bootsma, H., additional, Hammenfors, D., additional, Fraile, G., additional, Carsons, S., additional, Gheita, T., additional, Morel, J., additional, Vollenveider, C., additional, Atzeni, F., additional, Retamozo, S., additional, Horvath, I.-F., additional, Sivils, K., additional, Theander, E., additional, Sandhya, P., additional, De Vita, S., additional, Sanchez-Guerrero, J., additional, van der Heijden, E., additional, Moça-Trevisano, V., additional, Wahren-Herlenius, M., additional, Mariette, X., additional, and Ramos-Casals, M., additional
Published: 2016
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36. [Untitled]

Author: Serrano M, San Millán J, Fraile G, García M, Villanueva C, and Iglesias A
Subjects: Cancer Research, medicine.medical_specialty, Pathology, Neurology, business.industry, Mammary gland, Gliomatosis cerebri, medicine.disease, Metastatic breast cancer, Central nervous system disease, Breast cancer, medicine.anatomical_structure, Oncology, Glioma, medicine, Neoplasm, Neurology (clinical), business
Abstract: Gliomatosis cerebri (GC) is defined by the World Health Organization as a neoplasm of the glial cells. It is extremely rare, and there exists only 160 documented cases since 1897. There is no known treatment and the median survival rate is one year. The association of extracranial and CNS tumors is unusual, only three cases of breast cancer have been associated with gliomas and meningiomas but none with GC. Below we describe a case of breast cancer associated with GC and review the anatomoclinical and radiological manifestations of Gliomatosis Cerebri.
Published: 1997

37. Cyanoacetic ester pyrolysis in the gas phase

Author: Chuchani, G. and Fraile, G.
Published: 1978
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38. FRI0275 Long-Term Survival and Baseline Prognostic Factors in a Wide Series of Patients with AAV from Spain. Usefulness of Prognostic Scores (Revas Study)

Author: Solans-Laqué, R., primary, Rodriguez-Carballeira, M., additional, Fraile, G., additional, Castillo, M.J., additional, Rios, J., additional, Saez, L., additional, Solanich, X., additional, Caminal, L., additional, Oristrell, J., additional, Pasquau, F., additional, Fonseca, E., additional, Calleja, J., additional, Zamora, M., additional, Fanlo, P., additional, Abdilla, M., additional, Garcia, I., additional, Sopeña, B., additional, Lopez-Dupla, M., additional, Pérez, A., additional, and Frutos, B., additional
Published: 2015
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39. SAT0534 Thrombosis and Re-Thrombosis Manifestations in Patients with Behçet Disease. Descriptive Analysis from Regeb Cohort

Author: Rodríguez Carballeira, M., primary, Espinosa, G., additional, Mejía, J., additional, Solans, R., additional, Larrañaga, J., additional, Castillo, M., additional, Rios, R., additional, Nieto, J., additional, Solanich, X., additional, Fonseca, E., additional, Muñoz, F., additional, Fraile, G., additional, Boldava, R., additional, and Hurtado, R., additional
Published: 2015
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40. THU0408 How we are Treating Our Systemic Patients with Primary Sjögren Syndrome? Analysis of 1120 Patients (GEAS-SS Spanish Registry)

Author: Gheitasi, H., primary, Kostov, B., additional, Solans, R., additional, Fraile, G., additional, Suárez-Cuervo, C., additional, Casanovas, A., additional, Rascόn, F.-J., additional, Qanneta, R., additional, Pérez-Alvarez, R., additional, Ripoll, M., additional, Akasbi, M., additional, Pinilla, B., additional, Bosch, X., additional, Nava-Mateos, J., additional, Díaz-Lόpez, B., additional, Morera-Morales, L., additional, Retamozo, S., additional, Ramos-Casals, M., additional, and Brito Zeron, P., additional
Published: 2015
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41. THU0563 Prognostic Value of the 2014 Hscore in Adult Hemophagocytic Syndrome: Analysis in 111 Consecutive Patients (Reghem-Geas-Semi Spanish Cohort)

Author: Brito Zeron, P., primary, Moral Moral, P., additional, Martínez Zapico, A., additional, Fraile, G., additional, Fonseca, E., additional, Pérez Guerrero, P., additional, Robles, A., additional, Vaquero Herrero, M., additional, Ruiz De Temiño, A., additional, Forner, M.J., additional, Larrañaga, J.R., additional, Prieto, S., additional, Hurtado, R., additional, Ruiz Muñoz, M., additional, Rodriguez, M., additional, Caminal, L., additional, Chamorro, A., additional, Calvo, M.A., additional, Bosch, X., additional, Castro, P., additional, and Ramos-Casals, M., additional
Published: 2015
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42. OP0088 Categorization of Baseline Systemic Activity at Diagnosis Using the Essdai Disease Activity States (DAS) in Primary Sjögren Syndrome: Association with Poor Outcomes

Author: Brito Zeron, P., primary, Kostov, B., additional, Solans, R., additional, Fraile, G., additional, Caravia-Durán, D., additional, Maure, B., additional, Rascόn, F.-J., additional, Villar-Navas, B., additional, Ripoll, M., additional, Pinilla, B., additional, Fonseca, E., additional, Akasbi, M., additional, Pérez-de-Lis, M., additional, Jiménez-Heredia, I., additional, de la Red, G., additional, Gato, A., additional, Ramentol, M., additional, Ruedas, A., additional, Díaz-Lόpez, B., additional, Pallarés, L., additional, Gheitasi, H., additional, and Ramos-Casals, M., additional
Published: 2015
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43. SAT0417 Cancer and Primary SjÖgren Syndrome in 1216 Patients (GEAS-SS Registry): Systemic Activity Measured by the Essdai is Related to Hematological, but not Solid Neoplasia

Author: Brito Zeron, P., primary, Kostov, B., additional, Solans, R., additional, Fraile, G., additional, García-Pérez, A., additional, Maure, B., additional, Rascόn, F.-J., additional, Lopez-Dupla, M., additional, Ripoll, M., additional, Lόpez González-Cobos, C., additional, Fonseca, E., additional, Akasbi, M., additional, Pérez-de-Lis, M., additional, Jiménez-Heredia, I., additional, de la Red, G., additional, Gato, A., additional, Ramentol, M., additional, Ruedas, A., additional, Díaz-Lόpez, B., additional, Pallarés, L., additional, Gheitasi, H., additional, and Ramos-Casals, M., additional
Published: 2015
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44. FRI0604 Antimitochondrial Antibodies and Antibodies Against Subunits of Pyruvate Dehydrogenase as Serological Markers of Primary Biliary Cirrhosis in Patients with Primary SjöGren Syndrome

Author: Fraile, G., primary, Brito Zeron, P., additional, Solans, R., additional, Caravia-Durán, D., additional, Maure, B., additional, Rascόn, F.-J., additional, Lopez-Dupla, M., additional, Ripoll, M., additional, Lόpez-Berastegui, O., additional, Trapiella, L., additional, García-Sánchez, I., additional, Pérez-de-Lis, M., additional, Jiménez-Heredia, I., additional, de la Red, G., additional, Gato, A., additional, Martínez-Valle, F., additional, Nava, J., additional, Díaz-Lόpez, B., additional, Pallarés, L., additional, Gheitasi, H., additional, and Ramos-Casals, M., additional
Published: 2015
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45. Estudio biomecánico experimental del sistema musculo-esquelético masticatorio: Aplicaciones para el estudio de la osteosíntesis

Author: Cebrián Carretero, J.L., Carrascal Morillo, M.T., Vincent Fraile, G., and Ortiz de Artiñano, F.
Subjects: Photoelasticity, Mandíbula, Fotoelasticidad, Ensayos biomecánicos, Biomechanics, Mandible
Abstract: El comportamiento biomecánico del sistema músculoesquelético dista mucho de estar esclarecido. Los modelos matemáticos han mostrado importantes limitaciones, y los ensayos biomecánicos se han visto frustrados por la dificultad de simular las cargas musculares y la distribución de las tensiones en el espesor mandibular. En el presente trabajo se desarrolla un simulador estático del sistema músculo esquelético masticatorio que reproduce fielmente la situación fisiológica, empleándose la foto elasticidad tridimensional para el estudio de los cambios tensionales que ocurren en la estructura mandibular en diversas situaciones fisiopatológicas. Los resultados de los ensayos demuestran que la fotoelasticidad 3D aplicada en el simulador da resultados muy útiles para el análisis de la aplicación hueso-material de osteosíntesis utilizado en la práctica clínica. The biomechanical behavior of the masticatory system is not well known. The mathematical models that have been developed to date are limited and experimental studies have not yet solved the problem of reproducing muscular forces and stress distributions in the internal mandibular structure. A static simulator of the masticatory system was developed in the present study and threedimensional photoelasticity was used to analyze stress distribution in several physiologic and pathologic situations. The results showed that the simulator and 3D photoelasticity were useful for studying interactions and ostheosynthesis materials used in daily clinical practice.
Published: 2009

46. Apoptosis, cambio estructural y generación reactiva de especies reactivas de oxígeno en neutrófilos humanos expuestos a olanzapine, una droga antipsicótica atípica

Author: Vargas, F, Chávez, V, Betancourt, B, León, C, Pérez, K, Quezada, A, Ojeda, L, Paredes, M, Velásquez, M, and Fraile, G
Subjects: neutrofilos, apoptosis, olanzapina, estallido respiratorio, estrés oxidativo
Abstract: Los efectos de olanzapine (olz) sobre la viabilidad y el funcionamiento de células humanas polimorfonucleares (pmn, por sus siglas en inglés) claramente son opuestos a los señalados para la clozapine (clz). En efecto, después de 4-24 h de tratamiento con 20-50 μM olz, se observó una inhibición significativa del estallido respiratorio en pmn activados con zimosan o con forbol acetato miristato, mientras que la inhibición provocada por el formil-metionil-leucil-fenilalanina fue sólo inhibida a 50 μM de olz. En las mismas condiciones, la apoptosis espontánea se aceleró con 20-50 μM olz, mientras que la adición exógena de H2O2 dio lugar a la apoptosis de pmn en dosis dependiente inhibida por olz en el rango entero de concentraciones. Sin embargo, cuando se generó H2O2 intracelular por tratamiento con pma, la apoptosis inducida se disminuyó solamente con 2 μM olz. Las exploraciones de los espectros de absorbancia revelaron que olz puede reaccionar con cantidades equimolares de H2O2 o de HOCL. Estos resultados sugieren que olz inhibe ambos tipos de apoptosis de pmn (la inducida por especies reactivas oxigenadas y por estallido respiratorio debido a atrapadores extracelulares de estas especies reactivas oxigenadas).
Published: 2009

47. Systemic activity and mortality in primary Sjögren syndrome: predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients

Author: Brito-Zerón, P, primary, Kostov, B, additional, Solans, R, additional, Fraile, G, additional, Suárez-Cuervo, C, additional, Casanovas, A, additional, Rascón, F J, additional, Qanneta, R, additional, Pérez-Alvarez, R, additional, Ripoll, M, additional, Akasbi, M, additional, Pinilla, B, additional, Bosch, J A, additional, Nava-Mateos, J, additional, Díaz-López, B, additional, Morera-Morales, M L, additional, Gheitasi, H, additional, Retamozo, S, additional, and Ramos-Casals, M, additional
Published: 2014
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48. SAT0288 Eosinophilic Granulomatosis with Poliangeitis (EGPA): Clinical Features and Outcome in A Large Serie of Spanish Patients

Author: Solans-Laqué, R., primary, Fraile, G., additional, Castillo, M.J., additional, Solanich, X., additional, Caminal, L., additional, Rodriguez, M., additional, Rios, J.J., additional, Zamora, M., additional, Calleja, J.L., additional, Fanlo, P., additional, Garcia, I., additional, Saez, L., additional, Oristrell, J., additional, Abdilla, M., additional, Pasquau, F., additional, Lopez-Dupla, M., additional, Perez, A., additional, Fonseca, E., additional, and Sopeña, B., additional
Published: 2014
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49. THU0398 Adult Hemophagocytic Syndrome: Analysis of 50 Cases (Spanish National Registry Geas-Semi)

Author: Brito Zeron, P., primary, Retamozo, S., additional, Fraile, G., additional, Ruiz de Temino de la Pena, A., additional, Andres Calvo, M., additional, Fonseca, E., additional, Martinez Zapico, A., additional, Rodriguez Caballeira, M., additional, Caminal, L., additional, Larranaga, J.R., additional, Gheitasi, H., additional, Bosch, X., additional, Prieto Gonzalez, S., additional, and Ramos Casals, M., additional
Published: 2014
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50. OP0213 The Degree of Activity Measured with the EULAR-SS Disease Activity Index (ESSDAI) Strongly Correlated with Death in Patients with Primary Sjogren Syndrome (GEAS-SS REGISTRY)

Author: Brito Zeron, P., primary, Retamozo, S., additional, Solans, R., additional, Fraile, G., additional, Morera-Morales, L., additional, Suárez-Cuervo, C., additional, Rascόn, F.J., additional, Qanneta, R., additional, Pérez-de-Lis, M., additional, Akasbi, M., additional, Ripoll, M., additional, Pinilla, B., additional, Kostov, B., additional, Gheitasi, H., additional, and Ramos-Casals, M., additional
Published: 2014
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