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1. Prognostic Factors of Death in 151 Adults With Hemophagocytic Syndrome: Etiopathogenically Driven Analysis

Author: Brito-Zerón, Pilar, Kostov, Belchin, Moral-Moral, Pedro, Martínez-Zapico, Aleida, Díaz-Pedroche, Carmen, Fraile, Guadalupe, Pérez-Guerrero, Patricia, Fonseca, Eva, Robles, Angel, Vaquero-Herrero, María P., Calvo, María Andrés, Forner, María José, Morcillo, Cesar, Larrañaga, José, Rodriguez-Carballeira, Monica, Ruiz-Muñoz, Manuel, Hurtado-García, Robert, Prieto-González, Sergio, Aguilar, Asun Aljibe, Caminal-Montero, Luis, Hernández-Jiménez, Pilar, Fernández-Viagas, Cristina Rodríguez, Castro, Pedro, Massó, Victoria Morell, Flores-Chavez, Alejandra, and Ramos-Casals, Manuel
Published: 2018
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2. 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, Roser, Fraile, Guadalupe, Rodriguez-Carballeira, Monica, Caminal, Luis, Castillo, Maria J., Martínez-Valle, Ferran, Sáez, Luis, Rios, Juan J., Solanich, Xavier, Oristrell, Joaquim, Pasquau, Francisco, Fonseca, Eva, Zamora, Monica, Callejas, Jose L., Frutos, Begoña, Abdilla, Monica, Fanlo, Patricia, García-Sánchez, Isabel, López-Dupla, Miguel, Sopeña, Bernardo, Pérez-Iglesias, Almudena, and Bosch, Josep A.
Published: 2017
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3. Systemic involvement in primary Sjögren’s syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry)

Author: Ramos-Casals, Manuel, Brito-Zerón, Pilar, Solans, Roser, Camps, María-Teresa, Casanovas, Arnau, Sopeña, Bernardo, Díaz-López, Bernardino, Rascón, Francisco-Javier, Qanneta, Rami, Fraile, Guadalupe, Pérez-Alvarez, Roberto, Callejas, José-Luis, Ripoll, Mar, Pinilla, Blanca, Akasbi, Miriam, Fonseca, Eva, Canora, Jesús, Nadal, María-Elvira, Red, Gloria de la, Fernández-Regal, Inés, Jiménez-Heredia, Iratxe, Bosch, Josep-Angel, Ayala, María-del-Mar, Morera-Morales, Lluisa, Maure, Brenda, Mera, Arantxa, Ramentol, Marc, Retamozo, Soledad, and Kostov, Belchin
Published: 2014
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4. Epidemiological profile and north–south gradient driving baseline systemic involvement of primary Sjögren’s syndrome

Author: Brito-Zeron, Pilar, Acar-Denizli, Nihan, Ng, Wan-Fai, Horvath, Ildiko Fanny, Rasmussen, Astrid, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Gottenberg, Jacques-Eric, Danda, Debashish, Quartuccio, Luca, Priori, Roberta, Hernandez-Molina, Gabriela, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Kvarnstrom, Marika, Praprotnik, Sonja, Sene, Damien, Gerli, Roberto, Solans, Roser, Rischmueller, Maureen, Mandl, Thomas, Suzuki, Yasunori, Isenberg, David, Valim, Valeria, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Hideki, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Trevisani, Virginia Fernandes Moca, Hammenfors, Daniel, Pasoto, Sandra G., Retamozo, Soledad, Gheita, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenweider, Cristina, Zeher, Margit, Sivils, Kathy, Xu, Bei, Bombardieri, Stefano, Sandhya, Pulukool, De Vita, Salvatore, Minniti, Antonina, Sanchez-Guerrero, Jorge, Kilic, Levent, van der Heijden, Eefje, Park, Sung-Hwan, Wahren-Herlenius, Marie, Mariette, Xavier, Ramos-Casals, Manuel, Brito-Zeron, Pilar, Acar-Denizli, Nihan, Ng, Wan-Fai, Horvath, Ildiko Fanny, Rasmussen, Astrid, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Gottenberg, Jacques-Eric, Danda, Debashish, Quartuccio, Luca, Priori, Roberta, Hernandez-Molina, Gabriela, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Kvarnstrom, Marika, Praprotnik, Sonja, Sene, Damien, Gerli, Roberto, Solans, Roser, Rischmueller, Maureen, Mandl, Thomas, Suzuki, Yasunori, Isenberg, David, Valim, Valeria, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Hideki, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Trevisani, Virginia Fernandes Moca, Hammenfors, Daniel, Pasoto, Sandra G., Retamozo, Soledad, Gheita, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenweider, Cristina, Zeher, Margit, Sivils, Kathy, Xu, Bei, Bombardieri, Stefano, Sandhya, Pulukool, De Vita, Salvatore, Minniti, Antonina, Sanchez-Guerrero, Jorge, Kilic, Levent, van der Heijden, Eefje, Park, Sung-Hwan, Wahren-Herlenius, Marie, Mariette, Xavier, and Ramos-Casals, Manuel
Abstract: Objective: To characterize the systemic phenotype of primary Sjögren’s syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. Methods: The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren’s syndrome from the five continents. Results: The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P < 0.001) compared with females, as did patients diagnosed at <35 years (6.7 vs 5.6 in patients diagnosed at >65 years, P < 0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P < 0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P < 0.001). Conclusion: The systemic phenotype of primary Sjögren’s syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.
Published: 2020
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5. Combination therapy with rituximab and intravenous or oral fludarabine in the first-line, systemic treatment of patients with extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type

Author: Salar, Antonio, Domingo-Domenech, Eva, Estany, Cristina, Canales, Miguel A., Gallardo, Fernando, Servitje, Octavio, Fraile, Guadalupe, and Montalbán, Carlos
Published: 2009
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6. Gliomatosis cerebri mimicking a metastatic breast cancer: Fatal outcome

Author: Iglesias, Angel, García, Mercedes, Millaán, Juan San, Villanueva, Carmen, Fraile, Guadalupe, and Serrano, Manuel
Published: 1997
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7. Comparison of the birmingham vasculitis activity score and the five factors score to assess survival in anca-associated vasculitis

Author: Solans, Roser, Rodríguez-Carballeira, Mónica, Rios-Blanco, Juan Jose, Fraile, Guadalupe, Saez-Comet, Luis, Martinez-Zapico, Aleida, Frutos, Begona, Solanich, Xavier, Fonseca-Aizpuru, Eva, Pasquau-Liano, Francisco, Zamora, Monica, Oristrell, Joaquim, Fanlo, Patricia, López-Dupla, Miguel, Abdilla, Monica, Garcia-Sanchez, Isabel, Sopena, Bernardo, Castillo, María Jesús, Perales, Isabel, and Callejas, Jose Luis
Subjects: education
Abstract: On behalf of the Spanish Registry of systemic vasculitis (REVAS); Autoimmune Systemic Diseases Study Group (GEAS); Spanish Society of Internal Medicine (SEMI). This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record., [Objective] to compare the accuracy of Birmingham Vasculitis score (BVAS ) v.3, and Five Factors Score (FFS ) v.1996 and v.2009, to assess survival in ANCA ‐associated Vasculitis (AAV ). [Methods] 550 patients with AAV (41.1% GPA , 37.3% MPA , 21.6% EGPA ) diagnosed between 1990‐2016 were analyzed. 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 BVAS at diagnosis was 17.96±7.82, and was significantly higher in non‐survivors than in survivors (20.0±8.14 vs. 16.95±7.47, p
Published: 2019

8. How immunological profile drives clinical phenotype of primary Sjögren’s syndrome at diagnosis: analysis of 10.500 patients (Sjögren Big data project

Author: Retamozo, Soledad, Acar-Denizli, Nihan, Ng, Wan-Fai, Rasmussen, Astrid, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Gottenberg, Jacques-Eric, Danda, Debashish, Quartuccio, Luca, Priori, Roberta, Hernandez-Molina, Gabriala, Armagan, Berkan, Kruize, Aike A, Kwok, Seung-Ki, Kvarnström, Marika, Praprotnik, Sonja, Sène, Damien, Bartoloni, Elena, Solans, Roser, Rischmueller, Maureen, Suzuki, Yasunori, Isenberg, David A, Valim, Valeria, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valérie, Knopf, Andreas, Bombardieri, Michele, Trevisani, Virginia Fernandes, Hammenfors, Daniel S, Pasoto, Sandra G, Gheita, Tamer A, Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Horvath, Ildiko-Fanny, Sivils, Kathy L, Olsson, Peter, De Vita, Salvatore, Sánchez-Guerrero, Jorge, Kilic, Levent, Wahren-Herlenius, Marie, Mariette, Xavier, Ramos-Casals, Manuel, Brito-Zerón, Pilar, INICSA, UNC, CONICED, Cordoba, Rheumatology Unit, Cordoba (Institute University of Biomedical Sciences University of Cordoba (IUCBC), Department of Statistics, Istanbul, Mimar Sinan Üniversitesi, Institute of Cellular Medicine [Newcastle], Newcastle University [Newcastle], Oklahoma Medical Research Foundation (OMRF), Department of Rheumatology, Skane University Hospital, Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Departement of Rheumatology and Immunology, Hefei (Anhui Provincial Hospital), Rheumatology Unit (Rheum Unit - PISA), University of Pisa - Università di Pisa, Immuno-Rhumatologie Moléculaire, Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Department of Clinical Immunology and Rheumatology, Vellore (Christian Medical College & Hospital), Rheumatology Clinic, Academic Hospital S.M. Della Misericordia, Udine, Department of Internal Medicine [Rome, Italy] (DMISM), Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome], Immunology and Rheumatology Department, Mexico City, Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Hacettepe University = Hacettepe Üniversitesi, University Medical Center [Utrecht], Seoul St Mary's Hospital, Seoul, Unit of Experimental Rheumatology, Stockhom (Department of Medicine), Department of Rheumatology (Dep Rheumato - Ljubljana - SLOVENIE), University Medical Centre Slovenia, Hôpital Lariboisière, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Lariboisière-Fernand-Widal [APHP], Department of Medicine [Perugia, Italy] (Rheumatology, Unit), Università degli Studi di Perugia (UNIPG), Vall d'Hebron University Hospital [Barcelona], The Queen Elizabeth Hospital, Adelaide, Human Information Processing Laboratory - Japanese Advances Institute of Science and Technology [Ishikawa] (HIP/JAIST), JAIST - Ishikawa - Japon, Centre for Rheumatology and Connective Tissue Diseases (University College London, Royal Free Hospital), Centre for Rheumatology - London, Department of Medicine, Universidade Federal do Espirito Santo, Vitoria, Department of Rheumatology and Internal Medicine, Wroclaw, Department of Medical Sciences, Uppsala (Section of Rheumatology), Department of Internal Medicine, Madrid, Department of Rheumatology and Clinical Immunology Groningen (Dep Rheum - GRONINGEN), University Medical Center Groningen [Groningen] (UMCG), Department of Radiology and Cancer Biology, Nagasaki, University of L'Aquila [Italy] (UNIVAQ), 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), CHRU Brest - Service de Rhumatologie (CHU - BREST - Rhumato), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Otorhinolaryngology/Head and Neck Surgery, Munich, Centre for Experimental Medicine and Rheumatology, London, Federal University of São Paulo, Department of Clinical Science and Department of Rheumatology, Bergen, Rheumatology Division, Hospital das Clinicas, Sao Paulo (HCFMUSP), Rheumatology Department, Cairo, Istituto Ortopedico Galeazzi-IRCCS, Département de Rhumatologie[Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Lapeyronie, German Hospital, Buenos Aires, Department of Immunology [Debrecen, Hungary], University of Debrecen [Hungary], Department of Rheumatology (Dep Rheumato - Malmo - SUEDE), Skåne University Hospital, Department of Medical Area, University Hospital Santa Maria della Misericordia, Udine, Ospedale 'Santa Maria della Misericordia' = University Hospital 'Santa Maria della Misericordia', Department of Immunology [Mexico City], Natl Inst Pediat, Immunodeficiencies Res Unit, Mexico City, DF, Mexico, Rheumatology, Hacetttepe University Faculty of Medicine, Ankara, Department of Medicine Solna, CHU Le Kremlin-Bicêtre (Rheumatology Department), Department of Rheumatology, University of Barcelona, CELLEX-IDIBAPS Department of Autoimmune Diseases, Barcelona, Systemic Autoimmune Disease Unit, Department of Internal Medicine, Vall d'Hebron University Hospital, Barcelona, Spain, Department of Medicine, Universitat Autonòma, Barcelona, CHU Cochin [AP-HP], Sapienza University [Rome], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Lariboisière, University Hospital 'Santa Maria della Misericordia', and Michel, Geneviève
Subjects: [SDV] Life Sciences [q-bio], [SDV]Life Sciences [q-bio], ComputingMilieux_MISCELLANEOUS
Abstract: International audience
Published: 2018

9. Severe, life-threatening phenotype of primary Sjögren's syndrome: Clinical characterisation and outcomes in 1580 patients (GEAS-SS Registry)

Author: Chavez Flores, Alejandra Teresa, Kostov, Belchin, Solans Laqué, Roser, Fraile, Guadalupe, Maure, Brenda, Feijoo-Massó, Carlos, Rascon, Francisco Javier, Perez Alvarez, Roberto, Zamora, Mónica, García-Pérez, Alicia, Lopez-Dupla, Miguel, Duarte Millán, Miguel Ángel, Ripoll, Mar, Fonseca, Eva, Guisado, Pablo, Pinilla,Blanca, De la Red, Gloria, Chamorro, Antonio J., Morcillo, César, Fanlo, Patricia, Soto-Cárdenas, María José, Retamozo, Maria Soledad, and Ramos Casals, Manuel
Subjects: Vasculitis, CIENCIAS MÉDICAS Y DE LA SALUD, Lymphoma, Primary Sjögren’s syndrome, Medicina Clínica, Mortality, Reumatología
Abstract: To analyse the clinical features and outcomes of patients presenting with life-threatening systemic disease in a large cohort of Spanish patients with primary Sjögren´s syndrome (SS).METHODS:The GEAS-SS multicentre registry was formed in 2005 with the aim of collecting a large series of Spanish patients with primary SS, and included more than 20 Spanish reference centres with substantial experience in the management of SS patients. By January 2018, the database included 1580 consecutive patients fulfilling the 2002 classification criteria for primary SS. Severe, life-threatening systemic disease was defined as an activity level scored as "high" in at least one ESSDAI domain.RESULTS:Among 1580 patients, 208 (13%) were classified as presenting a severe, potentially life-threatening systemic disease: 193 presented one ESSDAI domain classified as high, 14 presented two high scored domains and only one presented three high activity domains. The ESSDAI domains involved consisted of lymphadenopathy in 78 (37%) cases, CNS in 28 (13%), PNS in 25 (12%), pulmonary in 25 (12%), renal in 21 (10%), cutaneous in 19 (9%), articular in 18 (9%), haematological in 7 (3%) and muscular in 4 (2%). Patients with severe systemic disease were more frequently men (p=0.001) and had a higher frequency of anaemia (p
Published: 2018

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

Author: Retamozo, Soledad, Kostov, Belchin, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Eric, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Hernandez-Molina, Gabriela, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Valim, Valeria, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Moca Trevisani, Virginia Fernandes, Hammenfors, Daniel, Pasoto, Sandra G., Carsons, Steven E., Gheita, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Brito-Zeron, Pilar, Ramos-Casals, Manuel, Retamozo, Soledad, Kostov, Belchin, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Eric, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Hernandez-Molina, Gabriela, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Valim, Valeria, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Moca Trevisani, Virginia Fernandes, Hammenfors, Daniel, Pasoto, Sandra G., Carsons, Steven E., Gheita, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Brito-Zeron, Pilar, and Ramos-Casals, Manuel
Published: 2018

11. Clinical and immunological disease patterns of primary Sjögren syndrome driven by gender and age at diagnosis

Author: Retamozo, Soledad, Kostov, Belchin, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Eric, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Hernandez-Molina, Gabriela, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Valim, Valeria, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Moca Trevisani, Virginia Fernandes, Hammenfors, Daniel, Pasoto, Sandra G., Carsons, Steven E., Gheita, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Brito-Zeron, Pilar, Ramos-Casals, Manuel, Retamozo, Soledad, Kostov, Belchin, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Eric, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Hernandez-Molina, Gabriela, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Valim, Valeria, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Moca Trevisani, Virginia Fernandes, Hammenfors, Daniel, Pasoto, Sandra G., Carsons, Steven E., Gheita, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Brito-Zeron, Pilar, and Ramos-Casals, Manuel
Published: 2018

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

Author: Retamozo, Soledad, Kostov, Belchin, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Eric, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Hernandez-Molina, Gabriela, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Valim, Valeria, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Moca Trevisani, Virginia Fernandes, Hammenfors, Daniel, Pasoto, Sandra G., Carsons, Steven E., Gheite, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Brito-Zeron, Pilar, Ramos-Casals, Manuel, Retamozo, Soledad, Kostov, Belchin, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Eric, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Hernandez-Molina, Gabriela, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Valim, Valeria, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Moca Trevisani, Virginia Fernandes, Hammenfors, Daniel, Pasoto, Sandra G., Carsons, Steven E., Gheite, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Brito-Zeron, Pilar, and Ramos-Casals, Manuel
Published: 2018

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

Author: Retamozo, Soledad, Acar-Denizli, Nihan, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Eric, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Hernandez-Molina, Gabriela, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Valim, Valeria, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Moca Trevisani, Virginia Fernandes, Hammenfors, Daniel, Pasoto, Sandra G., Carsons, Steven E., Gheita, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Brito-Zeron, Pilar, Ramos-Casals, Manuel, Retamozo, Soledad, Acar-Denizli, Nihan, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Eric, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Hernandez-Molina, Gabriela, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Valim, Valeria, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Moca Trevisani, Virginia Fernandes, Hammenfors, Daniel, Pasoto, Sandra G., Carsons, Steven E., Gheita, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Brito-Zeron, Pilar, and Ramos-Casals, Manuel
Published: 2018

14. Epidemiologic subsets drive a differentiated clinical and immunological presentation of primary Sjögren syndrome: analysis of 9302 patients from the big data International Sjögren Cohort

Author: Retamozo, Soledad, Brito-Zeron, Pilar, Zeher, Margit, Sivils Kathy, L, Seror, Raphaele, Mandl, Thomas, Li, Xiaomei, Baldini, Chiara, Gottenberg, Jacques-Eric, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Hernandez-Molina, Gabriela, Kruize Aike, A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Sene, Damien, Gerli, Roberto, Solans, Roser, Suzuki, yasunori, Isenberg David, A., Rischmueller, Maureen, Nordmark, Gunnel, Fraile, Guadalupe, Wiland, Piotr, Bootsma, Hendrika, Nakamura, Takashi, Valim, Valeria, Giacomelli, Roberto, Devauchelle-Pensec, Valérie, Hofauer, Benedikt, Bombardieri, Michèle, Fernandes Moça Trevisani, Virginia, Hammenfors, Daniel S., Carsons Steven, E., Pasoto, Gofinet, Morel, Jacques, Gheita, Tamer, Atzeni, Fabiola, Vollenweider, Cristina F., Kostov, Belchin, Mariette, Xavier, Ramos-Casals, Manuel, Rheumatology Unit, Cordoba (Institute University of Biomedical Sciences University of Cordoba (IUCBC), Autoimmune Diseases Unit, Barcelona (Department of Medicine), Department of Immunology [Debrecen, Hungary], University of Debrecen [Hungary], Oklahoma Medical Research Foundation, Medical Research Foundation, Center for Immunology of Viral Infections and Autoimmune Diseases, Le Kremlin Bicêtre, Department of Rheumatology, Skane University Hospital, Department of Rheumatology and Immunology, Hefei, Anhui, Rheumatology Unit (Rheum Unit - PISA), University of Pisa - Università di Pisa, Rheumatology [CHU Strasbourg], CHU Strasbourg, Clinical Immunology & Rheumatology, Vellore, UO Complessa Reumatologia, Rome, Rheumatology Clinic, Udine (DSMB), Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán - National Institute of Medical Science and Nutrition Salvador Zubiran [Mexico], University Medical Center [Utrecht], Division of Rheumatology, Seoul (Department of Internal Medicine), Unit of Experimental Rheumatology, Stockhom (Department of Medicine), Département de Médecine interne [Lariboisière], Université Paris Diderot - Paris 7 (UPD7)-Hôpital Lariboisière-Fernand-Widal [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), University and Azienda Ospedaliera of Perugia, Autoimmune Systemic Diseases Unit, Barcelona (Department of Internal Medicine), Division of Rheumatology Kanazawa University Graduate School of Medicine, Kanazawa, Centre for Rheumatology and Connective Tissue Diseases (University College London, Royal Free Hospital), Centre for Rheumatology - London, The Queen Elizabeth Hospital, Adelaide, Department of Medical Sciences, Uppsala (Section of Rheumatology), Autoimmune Diseases Department, Madrid, Wroclaw Medical University, University of Groningen [Groningen], Department of Radiology and Cancer Biology, Nagasaki, Department of Medicine, Universidade Federal do Espirito Santo, Vitoria, 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), University of Bergen (UiB), New York University Langone Medical Center (NYU Langone Medical Center), NYU System (NYU), Department of Internal Medicine. Faculty of Medicine. University of São Paulo-RP, Département de Rhumatologie[Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Lapeyronie, Rheumatology Department, Cairo, Rheumatology Unit, Milano (ASST Fatebenefratelli-Sacco), Rheumatology, Buenos Aires, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Universitat de Barcelona (UB), Université Paris-Sud - Paris 11 (UP11), CELLEX-IDIBAPS Department of Autoimmune Diseases, Barcelona, Michel, Geneviève, Hôpital Lariboisière-Fernand-Widal [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Lymphocytes B, Autoimmunité et Immunothérapies (LBAI), and Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-LabEX IGO Immunothérapie Grand Ouest-Institut Brestois Santé Agro Matière (IBSAM)
Subjects: [SDV] Life Sciences [q-bio], [SDV]Life Sciences [q-bio], ComputingMilieux_MISCELLANEOUS
Abstract: International audience
Published: 2017

15. Characterization and risk estimate of cancer in patients with primary Sjögren syndrome

Author: Brito-Zerón, Pilar, Kostov, Belchin, Fraile, Guadalupe, Caravia-Durán, Daniel, Maure, Brenda, Rascón, Francisco-Javier, Zamora, Mónica, Casanovas, Arnau, Lopez-Dupla, Miguel, Ripoll, Mar, Pinilla, Blanca, Fonseca, Eva, Akasbi, Miriam, de la Red, Gloria, Duarte-Millán, Miguel-Angel, Fanlo, Patricia, Guisado-Vasco, Pablo, Pérez-Alvarez, Roberto, Chamorro, Antonio J, Morcillo, César, Jiménez-Heredia, Iratxe, Sánchez-Berná, Isabel, López-Guillermo, Armando, Ramos-Casals, Manuel, SS Study Group GEAS-SEMI, The SS Study Group GEAS-SEMI, [Morcillo,C, Brito-Zerón,P] Autoimmune Diseases Unit, Department of Internal Medicine, Hospital CIMA-Sanitas, Barcelona, Spain. [Brito-Zerón, P: Sánchez-Berná,I, Ramos-Casals,M] Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Barcelona, Spain. [Kostov,B] Transversal group for research in primary care, IDIBAPS, Consorci d’Atenció Primària de Salut Barcelona Esquerre (CAPSBE), Barcelona, Spain. [Fraile,G] Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain. [Caravia-Durán,D] Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain. [Maure,B] Department of Internal Medicine, Complejo Hospitalario Universitario, Vigo, Spain. [Rascón,FJ] Department of Internal Medicine, Hospital Son Espases, Palma de Mallorca, Spain. [Zamora,M, and Sánchez-Berná,I] Department of Internal Medicine, Hospital Virgen de las Nieves, Granada, Spain. [Casanovas,A] Department of Internal Medicine, Hospital Parc Taulí, Sabadell, Spain. [ Lopez-Dupla,M] Department of Internal Medicine, Hospital Joan XXIII, Tarragona, Spain. [Ripoll,M] Department of Internal Medicine, Hospital Infanta Sofía, Madrid, Spain. [Pinilla,B] Department of Internal Medicine, Hospital Gregorio Marañón, Madrid, Spain. [Fonseca,E] Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain. [Akasbi,M] Department of Internal Medicine, Hospital Infanta Leonor, Madrid, Spain. [de la Red,G] Department of Internal Medicine, Hospital Esperit Sant, Santa Coloma de Gramenet, Spain. [Duarte-Millán,MA] Department of Internal Medicine, Hospital de Fuenlabrada, Fuenlabrada, Spain. [Fanlo,P] Department of Internal Medicine, Hospital Virgen del Camino, Pamplona, Spain. [Guisado-Vasco,P] Department of Internal Medicine, Complejo Hospitalario Ruber Juan Bravo, Madrid, Spain. [Pérez-Alvarez,R] Department of Internal Medicine, Hospital Alvaro Cunqueiro, Vigo, Spain. [Chamorro,AJ] Department of Internal Medicine, Hospital de Salamanca, Salamanca, Spain. [ Jiménez-Heredia,i] Department of Internal Medicine, Hospital de Sagunto, Valencia, Spain. [López-Guillermo,A] Department of Hematology, ICMHO, Hospital Clinic, Barcelona, Spain. [Ramos-Casals,M] Department of Medicine, University of Barcelona, Barcelona, Spain.
Subjects: Oncology, Male, Cancer Research, Pathology, Lymphoma, Databases, Factual, modelos de riesgos proporcionales, humanos, Diseases::Neoplasms::Neoplasms by Site::Endocrine Gland Neoplasms::Thyroid Neoplasms [Medical Subject Headings], Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans [Medical Subject Headings], Cohort Studies, Enfermedad de hodgkin, 0302 clinical medicine, immune system diseases, hemic and lymphatic diseases, Neoplasms, Análisis de regresión, evaluación de riesgos, Gammopatía monoclonal de relevancia indeterminada, Diseases::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Plasma Cell::Multiple Myeloma [Medical Subject Headings], Sjogren syndrome, Stomach cancer, estudios de cohortes, Thyroid cancer, Neoplasias de la tiroides, Multiple myeloma, mediana edad, Síndrome de sjögren, Cancer, Labio, neoplasias, anciano, Hematology, Incidence, Pronóstico, Diseases::Neoplasms::Neoplasms by Histologic Type::Lymphoma::Hodgkin Disease [Medical Subject Headings], Diseases::Hemic and Lymphatic Diseases::Hematologic Diseases::Blood Protein Disorders::Hypergammaglobulinemia::Monoclonal Gammopathy of Undetermined Significance [Medical Subject Headings], lcsh:Diseases of the blood and blood-forming organs, adulto, Middle Aged, lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens, Prognosis, humanities, pronóstico, Sjogren's Syndrome, 030220 oncology & carcinogenesis, Hematologic Neoplasms, Linfoma de células b de la zona marginal, Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Globulins::Serum Globulins::Immunoglobulins::Paraproteins::Cryoglobulins [Medical Subject Headings], Female, Estudios de seguimiento, Incidencia, Anatomy::Stomatognathic System::Mouth::Lip [Medical Subject Headings], Adult, medicine.medical_specialty, Neoplasias gástricas, Lymphoma, B-Cell, lcsh:RC254-282, Risk Assessment, incidencia, 03 medical and health sciences, Crioglobulinas, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Morbidity::Incidence [Medical Subject Headings], 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, Diseases::Immune System Diseases::Autoimmune Diseases::Arthritis, Rheumatoid::Sjogren's Syndrome [Medical Subject Headings], Humans, linfoma, Molecular Biology, Aged, Proportional Hazards Models, 030203 arthritis & rheumatology, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Regression Analysis [Medical Subject Headings], Proportional hazards model, business.industry, lcsh:RC633-647.5, Diseases::Neoplasms::Neoplasms by Site::Digestive System Neoplasms::Gastrointestinal Neoplasms::Stomach Neoplasms [Medical Subject Headings], Research, Mieloma múltiple, medicine.disease, neoplasias hematológicas, Diseases::Neoplasms::Neoplasms by Histologic Type::Lymphoma::Lymphoma, Non-Hodgkin::Lymphoma, B-Cell::Lymphoma, B-Cell, Marginal Zone [Medical Subject Headings], Sjögren syndrome, stomatognathic diseases, Risk Estimate, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Prognosis [Medical Subject Headings], business
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. Primary SjS is also associated with the development of some non-hematological cancers (thyroid, oral cavity, and stomach)., This work is supported by the Grant Fondo de Investigaciones Sanitarias (MRC, INT15/00085) and by the CERCA Programme/Generalitat de Catalunya.
Published: 2017

16. 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, Roser, Rodriguez‐Carballeira, Monica, Rios‐Blanco, Juan Jose, Fraile, Guadalupe, Sáez‐Comet, Luis, Martinez‐Zapico, Aleida, Frutos, Begoña, Solanich, Xavier, Fonseca‐Aizpuru, Eva, Pasquau‐Liaño, Francisco, Zamora, Monica, Oristrell, Joaquim, Fanlo, Patricia, Lopez‐Dupla, Miguel, Abdilla, Monica, García‐Sánchez, Isabel, Sopeña, Bernardo, Castillo, Maria Jesus, Perales, Isabel, and Callejas, Jose Luis
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 with AAV (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 BVAS at diagnosis was 17.96 ± 7.82 and was significantly higher in nonsurvivors than in survivors (mean ± SD 20.0 ± 8.14 versus 16.95 ± 7.47, respectively; P < 0.001). The mean ± SD 1996 FFS and 2009 FFS were 0.81 ± 0.94 and 1.47 ± 1.16, respectively, and were significantly higher in nonsurvivors than in survivors (mean ± SD 1996 FFS 1.17 ± 1.07 versus 0.63 ± 0.81 [P < 0.001] and 2009 FFS 2.13 ± 1.09 versus 1.15 ± 1.05 [P < 0.001], respectively). Mortality rates increased according to the different 1996 FFS and 2009 FFS categories. In multivariate analysis, BVAS, 1996 FFS, and 2009 FFS were significantly related to death (P = 0.007, P = 0.020, P < 0.001, respectively), but the stronger predictor was the 2009 FFS (hazard ratio 2.9 [95% confidence interval 2.4-3.6]). When the accuracy of BVAS, 1996 FFS, and 2009 FFS to predict survival was compared in the global cohort, ROC analysis yielded area under the curve values of 0.60, 0.65, and 0.74, respectively, indicating that 2009 FFS had 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 among BVAS and 1996 FFS was modest (r = 0.49; P < 0.001) but higher than between BVAS and the 2009 FFS (r = 0.28; P < 0.001).Conclusion: BVAS and FFS are useful to predict survival in AAV, but the 2009 FFS has the best prognostic accuracy at any point of the disease course. [ABSTRACT FROM AUTHOR]
Published: 2020
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17. Influence of geolocation and ethnicity on the phenotypic expression of primary Sjögren's syndrome at diagnosis in 8310 patients : a cross-sectional study from the Big Data Sjögren Project Consortium

Author: Brito-Zerón, Pilar, Acar-Denizli, Nihan, Zeher, Margit, Rasmussen, Astrid, Seror, Raphaele, Theander, Elke, Li, Xiaomei, Baldini, Chiara, Gottenberg, Jacques-Eric, Danda, Debashish, Quartuccio, Luca, Priori, Roberta, Hernandez-Molina, Gabriela, Kruize, Aike A, Valim, Valeria, Kvarnstrom, Marika, Sene, Damien, Gerli, Roberto, Praprotnik, Sonja, Isenberg, David, Solans, Roser, Rischmueller, Maureen, Kwok, Seung-Ki, Nordmark, Gunnel, Suzuki, Yasunori, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Bombardieri, Michele, Hofauer, Benedikt, Bootsma, Hendrika, Brun, Johan G, Fraile, Guadalupe, Carsons, Steven E, Gheita, Tamer A, Morel, Jacques, Vollenveider, Cristina, Atzeni, Fabiola, Retamozo, Soledad, Horvath, Ildiko Fanny, Sivils, Kathy, Mandl, Thomas, Sandhya, Pulukool, De Vita, Salvatore, Sanchez-Guerrero, Jorge, van der Heijden, Eefje, Trevisani, Virginia Fernandes Moça, Wahren-Herlenius, Marie, Mariette, Xavier, Ramos-Casals, Manuel, Brito-Zerón, Pilar, Acar-Denizli, Nihan, Zeher, Margit, Rasmussen, Astrid, Seror, Raphaele, Theander, Elke, Li, Xiaomei, Baldini, Chiara, Gottenberg, Jacques-Eric, Danda, Debashish, Quartuccio, Luca, Priori, Roberta, Hernandez-Molina, Gabriela, Kruize, Aike A, Valim, Valeria, Kvarnstrom, Marika, Sene, Damien, Gerli, Roberto, Praprotnik, Sonja, Isenberg, David, Solans, Roser, Rischmueller, Maureen, Kwok, Seung-Ki, Nordmark, Gunnel, Suzuki, Yasunori, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Bombardieri, Michele, Hofauer, Benedikt, Bootsma, Hendrika, Brun, Johan G, Fraile, Guadalupe, Carsons, Steven E, Gheita, Tamer A, Morel, Jacques, Vollenveider, Cristina, Atzeni, Fabiola, Retamozo, Soledad, Horvath, Ildiko Fanny, Sivils, Kathy, Mandl, Thomas, Sandhya, Pulukool, De Vita, Salvatore, Sanchez-Guerrero, Jorge, van der Heijden, Eefje, Trevisani, Virginia Fernandes Moça, Wahren-Herlenius, Marie, Mariette, Xavier, and Ramos-Casals, Manuel
Abstract: OBJECTIVES: To analyse the influence of geolocation and ethnicity on the clinical presentation of primary Sjögren's syndrome (SjS) at diagnosis. METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry designed in 2014. By January 2016, 20 centres from five continents were participating. Multivariable logistic regression analyses were performed. RESULTS: We included 7748 women (93%) and 562 men (7%), with a mean age at diagnosis of primary SjS of 53 years. Ethnicity data were available for 7884 patients (95%): 6174 patients (78%) were white, 1066 patients (14%) were Asian, 393 patients (5%) were Hispanic, 104 patients (1%) were black/African-American and 147 patients (2%) were of other ethnicities. SjS was diagnosed a mean of 7 years earlier in black/African-American compared with white patients; the female-to-male ratio was highest in Asian patients (27:1) and lowest in black/African-American patients (7:1); the prevalence of sicca symptoms was lowest in Asian patients; a higher frequency of positive salivary biopsy was found in Hispanic and white patients. A north-south gradient was found with respect to a lower frequency of ocular involvement in northern countries for dry eyes and abnormal ocular tests in Europe (OR 0.46 and 0.44, respectively) and Asia (OR 0.18 and 0.49, respectively) compared with southern countries. Higher frequencies of antinuclear antibodies (ANAs) were reported in northern countries in America (OR=1.48) and Asia (OR=3.80) while, in Europe, northern countries had lowest frequencies of ANAs (OR=0.67) and Ro/La (OR=0.69). CONCLUSIONS: This study provides the first evidence of a strong influence of geolocation and ethnicity on the phenotype of primary SjS at diagnosis.
Published: 2017
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18. A Rare Case of Zosteriform Cutaneous Metastases from a Nasopharyngeal Carcinoma

Author: González García, Andrés, Fernández, Emiliano Grillo, Barbolla Díaz, Ignacio, Ballester, Asunción, Pian, Héctor, and Fraile, Guadalupe
Subjects: integumentary system, Article Subject
Abstract: From a clinical point of view, the most common presentations of cutaneous metastatic disease are papules and nodules. However, a wide morphological spectrum of lesions has been described, including erythematous patches or plaques, inflammatory erysipelas-like lesions, diffuse sclerodermiform lesions with induration of the skin, telangiectatic papulovesicles, purpuric plaques mimicking vasculitis, and alopecia areata like scalp lesions. The so-called zosteriform pattern has been described to be in few cases and to the best of our knowledge has never been described associated with a metastasis of a nasopharyngeal carcinoma. This case highlights the relevance of including cutaneous metastases in the differential diagnosis of patients with nonhealing herpes zoster-like lesions, especially in those with underlying neoplasm recently diagnosed.
Published: 2015
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19. Influence of geolocation and ethnicity on the phenotypic expression of primary Sjögren's syndrome at diagnosis in 8310 patients: a cross-sectional study from the Big Data Sjögren Project Consortium

Author: Brito-Zerón, Pilar, primary, Acar-Denizli, Nihan, additional, Zeher, Margit, additional, Rasmussen, Astrid, additional, Seror, Raphaele, additional, Theander, Elke, additional, Li, Xiaomei, additional, Baldini, Chiara, additional, Gottenberg, Jacques-Eric, additional, Danda, Debashish, additional, Quartuccio, Luca, additional, Priori, Roberta, additional, Hernandez-Molina, Gabriela, additional, Kruize, Aike A, additional, Valim, Valeria, additional, Kvarnstrom, Marika, additional, Sene, Damien, additional, Gerli, Roberto, additional, Praprotnik, Sonja, additional, Isenberg, David, additional, Solans, Roser, additional, Rischmueller, Maureen, additional, Kwok, Seung-Ki, additional, Nordmark, Gunnel, additional, Suzuki, Yasunori, additional, Giacomelli, Roberto, additional, Devauchelle-Pensec, Valerie, additional, Bombardieri, Michele, additional, Hofauer, Benedikt, additional, Bootsma, Hendrika, additional, Brun, Johan G, additional, Fraile, Guadalupe, additional, Carsons, Steven E, additional, Gheita, Tamer A, additional, Morel, Jacques, additional, Vollenveider, Cristina, additional, Atzeni, Fabiola, additional, Retamozo, Soledad, additional, Horvath, Ildiko Fanny, additional, Sivils, Kathy, additional, Mandl, Thomas, additional, Sandhya, Pulukool, additional, De Vita, Salvatore, additional, Sanchez-Guerrero, Jorge, additional, van der Heijden, Eefje, additional, Trevisani, Virginia Fernandes Moça, additional, Wahren-Herlenius, Marie, additional, Mariette, Xavier, additional, and Ramos-Casals, Manuel, additional
Published: 2016
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20. DNA polymerase-α regulates the activation of type I interferons through cytosolic RNA:DNA synthesis

Author: Starokadomskyy, Petro, primary, Gemelli, Terry, additional, Rios, Jonathan J, additional, Xing, Chao, additional, Wang, Richard C, additional, Li, Haiying, additional, Pokatayev, Vladislav, additional, Dozmorov, Igor, additional, Khan, Shaheen, additional, Miyata, Naoteru, additional, Fraile, Guadalupe, additional, Raj, Prithvi, additional, Xu, Zhe, additional, Xu, Zigang, additional, Ma, Lin, additional, Lin, Zhimiao, additional, Wang, Huijun, additional, Yang, Yong, additional, Ben-Amitai, Dan, additional, Orenstein, Naama, additional, Mussaffi, Huda, additional, Baselga, Eulalia, additional, Tadini, Gianluca, additional, Grunebaum, Eyal, additional, Sarajlija, Adrijan, additional, Krzewski, Konrad, additional, Wakeland, Edward K, additional, Yan, Nan, additional, de la Morena, Maria Teresa, additional, Zinn, Andrew R, additional, and Burstein, Ezra, additional
Published: 2016
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21. Infection is the major trigger of hemophagocytic syndrome in adult patients treated with biological therapies

Author: Brito-Zerón, Pilar, primary, Bosch, Xavier, additional, Pérez-de-Lis, Marta, additional, Pérez-Álvarez, Roberto, additional, Fraile, Guadalupe, additional, Gheitasi, Hoda, additional, Retamozo, Soledad, additional, Bové, Albert, additional, Monclús, Ester, additional, Escoda, Ona, additional, Moreno, Asunción, additional, López-Guillermo, Armando, additional, Khamashta, Munther A., additional, and Ramos-Casals, Manuel, additional
Published: 2016
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22. Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) in a man with a diagnosis of X-linked reticulate pigmentary disorder (PDR)

Author: Fraile, Guadalupe, Norman, Francesca, Eugenia Reguero, Ma, Defargues, Vicente, and Redondo, Clara
Subjects: Adult, Male, medicine.medical_specialty, Duodenum, Protein-Losing Enteropathies, Context (language use), Gastroenterology, Enteritis, Internal medicine, medicine, Rare syndrome, Humans, Enteropathy, Pigmentation disorder, Ulcer, Superficial ulceration, Skin, business.industry, X-linked reticulate pigmentary disorder, medicine.disease, Duodenal Obstruction, Intestinal stenosis, business, Pigmentation Disorders
Abstract: Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare syndrome characterized by episodes of intestinal suboclusion caused by intestinal stenosis with superficial ulceration. Histological findings in the cases described in the literature are similar, even though they are not specific. The cause of the syndrome is as yet unknown. We report on the case of a 25-year-old male with a protein losing enteropathy (PLE) in the context of the CMUSE syndrome. At a later date the patient was also diagnosed with an X-linked recessive reticulate pigmentary disorder (PDR). The clinical features and tests which led to the diagnosis are described. The reported cases of CMUSE and of the gastrointestinal symptoms in the three families with PDR are reviewed in order to search for an association between these two entities. To date, there is no evidence to ascertain whether these two rare syndromes observed in the same patient are related or coincidental.
Published: 2008

23. CHARACTERISTICS OF PATIENTS WITH CASTLEMAN'S DISEASE IN AN INTERNAL MEDICINE DEPARTMENT

Author: Rojas-Marcos, Jorge, primary, González, Andrés, additional, Escribano, Marta, additional, Montalbán, Carlos, additional, Navas, Enrique, additional, Fraile, Guadalupe, additional, Garcia Cossio, Monica, additional, and Luis Patier, José, additional
Published: 2011
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24. Abscesos de psoas y paravesicales por Achromobacter xylosoxidans: descripción de un caso

Author: Fraile, Guadalupe, primary, Gallego, José Ignacio, additional, Martínez-Molina, Enrique, additional, and Meseguer, María Antonia, additional
Published: 2010
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25. First-Line Treatment with Rituximab Combined with Intravenous or Oral Fludarabine for Patients with Extranodal Mucosa Associated Lymphoid Tissue (MALT) Lymphoma.

Author: Salar, Antonio, primary, Domingo-Domènech, Eva, primary, Estany, Cristina, primary, Canales, Miguel, primary, Servitge, Octavio, primary, Gallardo, Fernando, primary, Fraile, Guadalupe, primary, and Montalbán, Carlos, primary
Published: 2008
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26. Thymic cyst presenting as Horner's syndrome

Author: Fraile, Guadalupe, Rodriguez-Garcia, Jose Luis, Monroy, Carlos, Fogue, Luis, and Millan, Jose Maria
Subjects: Horner's syndrome -- Causes of -- Diagnosis, Cysts -- Diagnosis, Thymus, Health, Diagnosis, Causes of
Abstract: We describe a case of Horner's syndrome secondary to a thymic cyst. Following successful surgical removal of the cyst, the patient's symptoms resolved. To the best of our knowledge, a similar case has not been reported. (Chest 1992; 101:1170-71), Horner's syndrome consists of the classic clinical manifestations of miosis, partial ptosis, and apparent enophthalmos and is occasionally accompanied by anhidrosis and pain. The pupil is variably miotic, depending on [...]
Published: 1992

27. Recurrent massive pleural effusion as a late complication of radiotherapy in Hodgkin's disease

Author: Rodriguez-Garcia, Jose L., Fraile, Guadalupe, Moreno, Maria A., Sanchez-Corral, Juan A., and Panalver, Rafael
Subjects: Hodgkin's disease -- Complications and side effects -- Diagnosis, Radiotherapy, Pleural effusions -- Diagnosis -- Complications and side effects, Health, Diagnosis, Complications and side effects
Abstract: We report a very unusual case of a patient with recurrent massive PEs eight years after mediastinal radiotherapy for HD, in which evidence of lymphomatous recurrence could not be demonstrated. [...]
Published: 1991

28. Hyperkeratosis of the Nipple and Areola in a Patient with Cutaneous T-Cell Lymphoma

Author: Allegue, Francisco, primary, Soria, Caridad, additional, Rocamora, Antonio, additional, Fraile, Guadalupe, additional, and Ledo, Antonio, additional
Published: 1990
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29. SYSTEMIC SJOGREN PRESENTING WITHOUT SICCA SYNDROME: CHARACTERIZATION OF 240 PATIENTS ACCORDING TO THE NEW 2017 ACR/EULAR CLASSIFICATION CRITERIA

Author: Retamozo, Soledad, Hernandez-Molina, Gabriela, Valim, Valeria, Moca Trevisani, Virginia Fernandes, Pasoto, Sandra G., Acar-Denizli, Nihan, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Ericn, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Damien SENE, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Hammenfors, Daniel, Carsons, Steven E., Gheita, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Ramos-Casals, Manuel, and Brito-Zeron, Pilar

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

Author: Retamozo, Soledad, Kostov, Belchin, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Eric, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Hernandez-Molina, Gabriela, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Valim, Valeria, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Moca Trevisani, Virginia Fernandes, Hammenfors, Daniel, Pasoto, Sandra G., Carsons, Steven E., Gheite, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Brito-Zeron, Pilar, and Manuel Ramos-Casals

31. Epidemiologic Subsets Drive a Differentiated Thoracic and Extrathoracic Presentation of Sarcoidosis: Analysis of 1082 Patients from the Sarcogeas-SEMI Registry

Author: Retamozo, Soledad, Perez-Alvarez, Roberto, Fraile, Guadalupe, La Torre, Ricardo Gomez, Dupla, Miguel Lopez, Yanguela, Begona Escalante, Alguacil, Ana, Chara-Cervantes, Joel, Marco, Jose Velilla, Rascon, Francisco Javier, Morillo, Jose Salvador Garcia, Tolosa, Carles, Aizpuru, Eva Fonseca, Bonet, Mariona, Callejas, Jose Luis, La Red, Gloria, Begueria, Eva Calvo, Ferrer, Cristina Soler I., Ceballos, Enrique Peral Gutierrez, Cerezo, Jorge Francisco Gomez, Caparros, Gracia Cruz, Guerrero, Patricia Perez, Fernandez, Sergio Rodriguez, Pinilla, Blanca, Diez, Alberto Gato, Akasbi, Miriam, Robles, Angel, Ojeda, Inmaculada, Vives, Maria Jose, Morcillo, Cesar, Vidal, Maria Penades, Vicente, Moises, Kostov, Belchin, Manuel Ramos-Casals, Pallares, Lucio, and Brito-Zeron, Pilar

32. How Does Sarcoidosis Present in Spain? Characteristics at Diagnosis of 979 Patients from the Sarcogeas-SEMI Registry

Author: Brito-Zeron, Pilar, Kostov, Belchin, Perez Lis, Marta, Fraile, Guadalupe, Gomez La Torre, Ricardo, Roca Herrera, Maria, Escalante Yanguela, Begona, Alguacil, Ana, Perez Conesa, Mercedes Pilar, Javier Rascon, Francisco, Garcia Morillo, Jose Salvador, Feijoo Masso, Carlos, Fonseca Aizpuru, Eva, Bonet, Mariona, Faro Minguez, Naya, La Red Bellvis, Gloria, Calvo Begueria, Eva, Gomez Lozano, Albert, Gutierrez Ceballos, Enrique Peral, Gomez Cerezo, Jorge Francisco, Cruz Caparros, Gracia, Perez Guerrero, Patricia, Rodriguez Fernandez, Sergio, Gato Diez, Alberto, Toledo Samaniego, Neera, Akasbi, Miriam, Robles, Angel, Ojeda, Inmaculada, Jose Vives, Maria, Penades Vidal, Maria, Morcillo, Cesar, Vicente, Moises, Retamozo, Soledad, Pallares, Lucio, Manuel Ramos-Casals, and Perez-Alvarez, Roberto

33. Epidemiologic Subsets Drive a Differentiated Clinical and Immunological Presentation of Primary Sjogren Syndrome: Analysis of 9302 Patients from the Big Data International Sjogren Cohort

Author: Retamozo, Soledad, Brito-Zeron, Pilar, Zeher, Margit, Sivils, Kathy L., Seror, Raphaele, Mandl, Thomas, Li, Xiaomei, Baldini, Chiara, Gottenberg, Jacques-Eric, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Hernandez-Molina, Gabriela, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Gerli, Roberto, Solans, Roser, Suzuki, Yasunori, Isenberg, David A., Rischmueller, Maureen, Nordmark, Gunnel, Fraile, Guadalupe, Wiland, Piotr, Bootsma, Hendrika, Nakamura, Takashi, Valim, Valeria, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Moca Trevisani, Virginia Fernandes, Hammenfors, Daniel S., Carsons, Steven E., Pasoto, Sandra Gofinet, Morel, Jacques, Gheita, Tamer, Atzeni, Fabiola, Vollenweider, Cristina F., Kostov, Belchin, Mariette, Xavier, and Manuel Ramos-Casals

34. HOW DIFFERENT SYSTEMIC ORGAN INVOLVEMENTS OVERLAP IN PATIENTS WITH PRIMARY SJOGREN SYNDROME: ANALYSIS USING A MATHEMATICAL MODEL

Author: Retamozo, Soledad, Hernandez-Molina, Gabriela, Valim, Valeria, Moca Trevisani, Virginia Fernandes, Pasoto, Sandra G., Acar-Denizli, Nihan, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Ericn, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Damien SENE, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Hammenfors, Daniel, Carsons, Steven E., Gheita, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Ramos-Casals, Manuel, and Brito-Zeron, Pilar

35. Isolated Anti-La/SS-B Positivity in Patients Diagnosed with Primary Sjogren Syndrome: Analysis of 222 Patients from the Sjogren Big Data Cohort

Author: Brito-Zeron, Pilar, Acar-Denizli, Nihan, Zeher, Margit, Rasmussen, Astrid, Seror, Raphaele, Theander, Elke, Li, Xiaomei, Baldini, Chiara, Gottenberg, Jacques-Eric, Danda, Debashish, Quartuccio, Luca, Priori, Roberta, Hernandez-Molina, Gabriela, Kruize, Aike A., Valim, Valeria, Kvarnstrom, Marika, Damien SENE, Gerli, Roberto, Praprotnik, Sonja, Isenberg, David A., Solans, Rorer, Rischmueller, Maureen, Kwok, Seung-Ki, Nordmark, Gunnel, Suzuki, Yasunori, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Bombardieri, Michele, Hofauer, Benedikt, Bootsma, Hendrika, Brun, Johan G., Fraile, Guadalupe, Carsons, Steven E., Gheita, Tamer, Morel, Jacques, Vollenweider, Cristina F., Atzeni, Fabiola, Retamozo, Soledad, Horvath, Ildike-Fanny, Sivils, Kathy, Mandl, Thomas, Sandhya, Pulukool, Vita, Salvatore, Sanchez-Guerrero, Jorge, Heijden, Eefje, Trevisano, Virginia Moca, Wahren-Herlenius, Marie, and Ramos-Casals, Manuel

36. Development of high systemic activity in primary Sjogren syndrome: analysis of 1487 Spanish patients (GEAS-SS Registry)

Author: Retamozo, Soledad, Flores-Chavez, Alejandra, Solans, Roser, Fraile, Guadalupe, Maure, Brenda, Feijoo, Carles, Perez-Alvarez, Roberto, Javier Rascon, Francisco, Zamora, Monica, Lopez-Dupla, Miguel, Angel Duarte-Millan, Miguel, Ripoll, Mar, Guisado-Vasco, Pablo, Fonseca, Eva, De-La-Red, Gloria, Chamorro, Antonio, Sanchez Vizcaino, Elena, Pinilla, Blanca, Jose Soto-Cardenas, Maria, Akasbi, Miriam, Fanlo, Patricia, Gato, Alberto, Jimenez-Heredia, Iratxe, De-Miguel, Borja, Arteaga, Sofia, Kostov, Belchin, Manuel Ramos-Casals, and Brito-Zeron, Pilar

37. Differentiated Phenotypes at Diagnosis of Sarcoidosis According to the Scadding Classification: Analysis in 1230 Patients

Author: Retamozo, Soledad, Perez-Alvarez, Roberto, Brito-Zeron, Pilar, Kostov, Belchin, Feijoo Masso, Carlos, Fraile, Guadalupe, Gonzalez-Garcia, A., Gomez La Torre, Ricardo, De-Escalante, Begona, Lopez-Dupla, Miguel, Alguacil, Ana, Chara-Cervantes, Joel, Perez-Conesa, M., Rascon, Francisco-Javier, José-Salvador García-Morillo, Perez Guerrero, Patricia, Fonseca Aizpuru, Eva, Akasbi, Miriam, Bonet, Mariona, Luis Callejas, Jose, La Red Bellvis, Gloria, Calvo Begueria, Eva, Soler I Ferrer, Cristina, Gutierrez Ceballos, Enrique Peral, Gomez-Cerezo, J. F., Cruz Caparros, Gracia, Rodriguez Fernandez, Sergio, Pinilla, Blanca, Gato Diez, Alberto, Rolo, A., Morcillo, Cesar, Robles, Angel, Ojeda, Inmaculada, Vives, M. J., De-Miguel, Borja, Penades Vidal, Maria, Vicente, Moises, Bosh, Xavier, Perez-De-Lis, Marta, Yllera Gutierrez, Carmen, Gracia-Tello, B., Pedrosa, M., Perez-Gonzalez, A., Tolosa, Cartes, Siso-Almirall, Antoni, Pallares, Lucio, and Ramos-Casals, Manuel

38. HOW ETHNICITY MODIFIES SYSTEMIC ACTIVITY OF PRIMARY SJOGREN SYNDROME: ANALYSIS OF BASELINE ESSDAI SCORES IN A MULTI-ETHNIC INTERNATIONAL COHORT

Author: Retamozo, Soledad, Hernandez-Molina, Gabriela, Valim, Valeria, Moca Trevisani, Virginia Fernandes, Pasoto, Sandra G., Acar-Denizli, Nihan, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Ericn, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Hammenfors, Daniel, Carsons, Steven E., Gheita, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Manuel Ramos-Casals, and Brito-Zeron, Pilar

39. CLINICAL AND IMMUNOLOGICAL PRIMARY SJOGREN SYNDROME' DISEASE PATTERNS ARE DRIVEN BY GENDER AND AGE AT DIAGNOSIS

Author: Retamozo, Soledad, Hernandez-Molina, Gabriela, Valim, Valeria, Moca Trevisani, Virginia Fernandes, Pasoto, Sandra G., Acar-Denizli, Nihan, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Ericn, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Hammenfors, Daniel, Carsons, Steven E., Gheita, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Manuel Ramos-Casals, and Brito-Zeron, Pilar

40. How Does Primary Sjogren Syndrome Present in Biopsy-Proven Patients without Circulating Ro/La Autoantibodies? Characteristics at Diagnosis of 2073 Patients from the SjoGren Big Data Project

Author: Brito-Zeron, Pilar, Acar-Denizli, Nihan, Zeher, Margit, Rasmussen, Astrid, Seror, Raphaele, Theander, Elke, Li, Xiaomei, Baldini, Chiara, Gottenberg, Jacques-Eric, Danda, Debashish, Quartuccio, Luca, Priori, Roberta, Hernandez-Molina, Gabriela, Kruize, Aike A., Valim, Valeria, Kvarnstrom, Marika, Sene, Damien, Gerli, Roberto, Praprotnik, Sonja, Isenberg, David A., Solans, Roser, Rischmueller, Maureen, Kwok, Seung-Ki, Nordmark, Gunnel, Suzuki, Yasunori, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Bombardieri, Michele, Hofauer, Benedikt, Bootsma, Hendrika, Brun, Johan G., Fraile, Guadalupe, Carsons, Steven E., Gheita, Tamer, More, Jacques, Vollenweider, Cristina F., Atzeni, Fabiola, Retamozo, Soledad, Horvath, Ildike-Fanny, Sivils, Kathy, Mand, Thomas, Sandhya, Pulukool, Vita, Salvatore, Sanchez-Guerrero, Jorge, Heijden, Eefje, Trevisano, Virginia Moca, Wahren-Herlenius, Marie, Mariette, Xavier, and Manuel Ramos-Casals

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

Author: Retamozo, Soledad, Kostov, Belchin, Zeher, Margit, Sivils, Kathy, Mandl, Thomas, Seror, Raphaele, Li, Xiaomei, Baldini, Chiara, Mariette, Xavier, Gottenberg, Jacques-Eric, Danda, Debashish, Priori, Roberta, Quartuccio, Luca, Hernandez-Molina, Gabriela, Armagan, Berkan, Kruize, Aike A., Kwok, Seung-Ki, Wahren-Herlenius, Marie, Praprotnik, Sonja, Sene, Damien, Bartoloni, Elena, Rischmueller, Maureen, Solans, Roser, Suzuki, Yasunori, Isenberg, David, Valim, Valeria, Wiland, Piotr, Nordmark, Gunnel, Fraile, Guadalupe, Bootsma, Hendrika, Nakamura, Takashi, Giacomelli, Roberto, Devauchelle-Pensec, Valerie, Hofauer, Benedikt, Bombardieri, Michele, Moca Trevisani, Virginia Fernandes, Hammenfors, Daniel, Pasoto, Sandra G., Carsons, Steven E., Gheita, Tamer A., Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Brito-Zeron, Pilar, and Manuel Ramos-Casals

42. Sjogren Big Data Project: Influence of Geolocation on the Phenotypic Expression at Diagnosis in 8310 Patients (North-to-South Gradient)

Author: Brito-Zeron, Pilar, Retamozo, Soledad, Zeher, Margit, Rasmussen, Astrid, Seror, Raphaele, Theander, Elke, Li, Xiaomei, Baldini, Chiara, Gottenberg, Jacques-Eric, Danda, Debashish, Quartuccio, Luca, Priori, Roberta, Hernandez-Molina, Gabriela, Kruize, Aike A., Valim, Valeria, Kvarnstrom, Marika, Damien SENE, Gerli, Roberto, Praprotnik, Sonja, Isenberg, David A., Solans, Roser, Rischmueller, Maureen, Kwok, Seung-Ki, Nordmark, Gunnel, Suzuki, Yasunori, Giacomelli, Roberto, Devauchelle, Valerie, Bombardieri, Michele, Hofauer, Benedikt, Bootsma, Hendrika, Brun, Johan G., Fraile, Guadalupe, Carsons, Steven E., Gheita, Tamer, Morel, Jacques, Vollenweider, Cristina F., Atzeni, Fabiola, Acar-Denizli, Nihan, Horvath, Ildike-Fanny, Sivils, Kathy, Mandl, Thomas, Sandhya, Pulukool, Vita, Salvatore, Sanchez-Guerrero, Jorge, Heijden, Eefje, Trevisano, Virginia Moca, Wahren-Herlenius, Marie, Mariette, Xavier, and Ramos-Casals, Manuel

43. Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjögren's syndrome.

Author: Brito-Zerón P, Acar-Denizli N, Ng WF, Horváth IF, Rasmussen A, Seror R, Li X, Baldini C, Gottenberg JE, Danda D, Quartuccio L, Priori R, Hernandez-Molina G, Armagan B, Kruize AA, Kwok SK, Kvarnstrom M, Praprotnik S, Sene D, Gerli R, Solans R, Rischmueller M, Mandl T, Suzuki Y, Isenberg D, Valim V, Wiland P, Nordmark G, Fraile G, Bootsma H, Nakamura H, Giacomelli R, Devauchelle-Pensec V, Hofauer B, Bombardieri M, Trevisani VFM, Hammenfors D, Pasoto SG, Retamozo S, Gheita TA, Atzeni F, Morel J, Vollenweider C, Zeher M, Sivils K, Xu B, Bombardieri S, Sandhya P, De Vita S, Minniti A, Sánchez-Guerrero J, Kilic L, van der Heijden E, Park SH, Wahren-Herlenius M, Mariette X, and Ramos-Casals M
Subjects: Black or African American statistics & numerical data, Asian People statistics & numerical data, Cohort Studies, Female, Hispanic or Latino statistics & numerical data, Humans, Information Dissemination, Male, Middle Aged, Phenotype, Registries, Severity of Illness Index, Sjogren's Syndrome ethnology, White People statistics & numerical data, Ethnicity statistics & numerical data, Racial Groups statistics & numerical data, Sjogren's Syndrome epidemiology
Abstract: Objective: To characterize the systemic phenotype of primary Sjögren's syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores., Methods: The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren's syndrome from the five continents., Results: The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P < 0.001) compared with females, as did patients diagnosed at <35 years (6.7 vs 5.6 in patients diagnosed at >65 years, P < 0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P < 0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P < 0.001)., Conclusion: The systemic phenotype of primary Sjögren's syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis., (© The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)
Published: 2020
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44. Venous thrombosis and relapses in patients with Behçet's disease. Descriptive analysis from Spanish network of Behçet's disease (REGEB cohort).

Author: Rodríguez-Carballeira M, Solans R, Larrañaga JR, García-Hernández FJ, Rios-Fernández R, Nieto J, Solanich X, Martínez-Valle F, Fonseca E, Muñoz FJ, Fraile G, de Escalante B, Boldova R, Hurtado R, and Espinosa G
Subjects: Adolescent, Adult, Behcet Syndrome diagnosis, Female, Humans, Male, Prognosis, Recurrence, Registries, Retrospective Studies, Risk Assessment, Risk Factors, Spain epidemiology, Time Factors, Venous Thrombosis diagnosis, Young Adult, Behcet Syndrome epidemiology, Venous Thrombosis epidemiology
Abstract: Objectives: To describe the characteristics of patients with Behçet'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 Behçet 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., Conclusions: We identified factors related with venous involvement in patients included in the REGEB cohort.
Published: 2018

45. 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, Kvarnström M, 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, De Vita S, Sánchez-Guerrero J, Kilic L, Wahren-Herlenius M, Mariette X, and Ramos-Casals M
Subjects: Adult, Aged, Antibodies, Antinuclear blood, Biomarkers blood, Female, Humans, Male, Middle Aged, Phenotype, Prognosis, Registries, Rheumatoid Factor blood, Sjogren's Syndrome diagnosis, Sjogren's Syndrome epidemiology, Autoantibodies blood, Complement C3 analysis, Complement C4 analysis, Cryoglobulins analysis, Sjogren's Syndrome immunology
Abstract: 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<0.001) in the organ-by-organ ESS- DAI evaluation were cryoglobulins (9 domains), low C3 (8 domains), anti-La (7 domains) and low C4 (6 domains)., Conclusions: We confirm the strong influence of immunological markers on the phenotype of primary SjS at diagnosis in the largest multi-ethnic international cohort ever analysed, with a greater influence for cryoglobulinaemic-related markers in comparison with Ro/La autoantibodies and ANA. Immunological patterns play a central role in the phenotypic expression of the disease already at the time of diagnosis, and may guide physicians to design a specific personalised management during the follow-up of patients with primary SjS.
Published: 2018

46. Severe, life-threatening phenotype of primary Sjögren's syndrome: clinical characterisation and outcomes in 1580 patients (GEAS-SS Registry).

Author: Flores-Chávez A, Kostov B, Solans R, Fraile G, Maure B, Feijoo-Massó C, Rascón FJ, Pérez-Alvarez R, Zamora-Pasadas M, García-Pérez A, Lopez-Dupla M, Duarte-Millán MÁ, Ripoll M, Fonseca-Aizpuru E, Guisado-Vasco P, Pinilla B, de-la-Red G, Chamorro AJ, Morcillo C, Fanlo P, Soto-Cárdenas MJ, Retamozo S, Ramos-Casals M, and Brito-Zerón P
Subjects: Adult, Aged, Decision Support Techniques, Disease Progression, Female, Glucocorticoids therapeutic use, Humans, Immunosuppressive Agents therapeutic use, Male, Middle Aged, Phenotype, Predictive Value of Tests, Registries, Risk Assessment, Risk Factors, Severity of Illness Index, Sjogren's Syndrome diagnosis, Sjogren's Syndrome mortality, Sjogren's Syndrome therapy, Spain epidemiology, Treatment Outcome, Sjogren's Syndrome epidemiology
Abstract: Objectives: To analyse the clinical features and outcomes of patients presenting with life-threatening systemic disease in a large cohort of Spanish patients with primary Sjögren's syndrome (SS)., Methods: The GEAS-SS multicentre registry was formed in 2005 with the aim of collecting a large series of Spanish patients with primary SS, and included more than 20 Spanish reference centres with substantial experience in the management of SS patients. By January 2018, the database included 1580 consecutive patients fulfilling the 2002 classification criteria for primary SS. Severe, life-threatening systemic disease was defined as an activity level scored as "high" in at least one ESSDAI domain., Results: Among 1580 patients, 208 (13%) were classified as presenting a severe, potentially life-threatening systemic disease: 193 presented one ESSDAI domain classified as high, 14 presented two high scored domains and only one presented three high activity domains. The ESSDAI domains involved consisted of lymphadenopathy in 78 (37%) cases, CNS in 28 (13%), PNS in 25 (12%), pulmonary in 25 (12%), renal in 21 (10%), cutaneous in 19 (9%), articular in 18 (9%), haematological in 7 (3%) and muscular in 4 (2%). Patients with severe systemic disease were more frequently men (p=0.001) and had a higher frequency of anaemia (p<0.001), lymphopenia (p<0.001), rheumatoid factor (p=0.021), low C3 levels (p=0.015), low C4 levels (p<0.001) and cryoglobulins (p<0.001). From a therapeutic point of view, systemic patients received more frequently glucocorticoids (p<0.001), immunosuppressants (p<0.001), intravenous immunoglobulins (p=0.008) and rituximab (p<0.001). We found an overall mortality rate of 20% in severe systemic patients, a rate that reached to 33% in patients presenting two or more high systemic involvements; these patients had a higher frequency of low C4 levels (p=0.012) and cryoglobulins (p=0.001) in comparison with those with a single severe organ involved., Conclusions: 13% of patients with primary SS develop a potentially life-threatening systemic disease (mainly lymphoma, but also severe internal organ involvements including nervous system, the lungs and the kidneys). This subset of patients requires intensive therapeutic management with a mortality rate of nearly 20% of cases.
Published: 2018

47. Influence of geolocation and ethnicity on the phenotypic expression of primary Sjögren's syndrome at diagnosis in 8310 patients: a cross-sectional study from the Big Data Sjögren Project Consortium.

Author: Brito-Zerón P, Acar-Denizli N, Zeher M, Rasmussen A, Seror R, Theander E, Li X, Baldini C, Gottenberg JE, Danda D, Quartuccio L, Priori R, Hernandez-Molina G, Kruize AA, Valim V, Kvarnstrom M, Sene D, Gerli R, Praprotnik S, Isenberg D, Solans R, Rischmueller M, Kwok SK, Nordmark G, Suzuki Y, Giacomelli R, Devauchelle-Pensec V, Bombardieri M, Hofauer B, Bootsma H, Brun JG, Fraile G, Carsons SE, Gheita TA, Morel J, Vollenveider C, Atzeni F, Retamozo S, Horvath IF, Sivils K, Mandl T, Sandhya P, De Vita S, Sanchez-Guerrero J, van der Heijden E, Trevisani VFM, Wahren-Herlenius M, Mariette X, and Ramos-Casals M
Subjects: Adult, Aged, Antibodies, Antinuclear blood, Cross-Sectional Studies, Eye Diseases etiology, Female, Humans, Male, Middle Aged, Phenotype, Prevalence, Sjogren's Syndrome blood, Sjogren's Syndrome complications, Sjogren's Syndrome diagnosis, Spatial Analysis, Black or African American statistics & numerical data, Asian People statistics & numerical data, Hispanic or Latino statistics & numerical data, Registries, Sjogren's Syndrome ethnology, White People statistics & numerical data
Abstract: Objectives: To analyse the influence of geolocation and ethnicity on the clinical presentation of primary Sjögren's syndrome (SjS) at diagnosis., Methods: The Big Data Sjögren Project Consortium is an international, multicentre registry designed in 2014. By January 2016, 20 centres from five continents were participating. Multivariable logistic regression analyses were performed., Results: We included 7748 women (93%) and 562 men (7%), with a mean age at diagnosis of primary SjS of 53 years. Ethnicity data were available for 7884 patients (95%): 6174 patients (78%) were white, 1066 patients (14%) were Asian, 393 patients (5%) were Hispanic, 104 patients (1%) were black/African-American and 147 patients (2%) were of other ethnicities. SjS was diagnosed a mean of 7 years earlier in black/African-American compared with white patients; the female-to-male ratio was highest in Asian patients (27:1) and lowest in black/African-American patients (7:1); the prevalence of sicca symptoms was lowest in Asian patients; a higher frequency of positive salivary biopsy was found in Hispanic and white patients. A north-south gradient was found with respect to a lower frequency of ocular involvement in northern countries for dry eyes and abnormal ocular tests in Europe (OR 0.46 and 0.44, respectively) and Asia (OR 0.18 and 0.49, respectively) compared with southern countries. Higher frequencies of antinuclear antibodies (ANAs) were reported in northern countries in America (OR=1.48) and Asia (OR=3.80) while, in Europe, northern countries had lowest frequencies of ANAs (OR=0.67) and Ro/La (OR=0.69)., Conclusions: This study provides the first evidence of a strong influence of geolocation and ethnicity on the phenotype of primary SjS at diagnosis., Competing Interests: Competing interests: None declared., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)
Published: 2017
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48. Characterization and risk estimate of cancer in patients with primary Sjögren syndrome.

Author: 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, and Ramos-Casals M
Subjects: Adult, Aged, Cohort Studies, Databases, Factual, Female, Hematologic Neoplasms etiology, Humans, Incidence, Lymphoma, B-Cell etiology, Male, Middle Aged, Prognosis, Proportional Hazards Models, Risk Assessment, Neoplasms etiology, Sjogren's Syndrome complications
Abstract: Background: The purpose of this study is to characterize the risk of cancer in a large cohort of patients with primary Sjögren 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. Primary SjS is also associated with the development of some non-hematological cancers (thyroid, oral cavity, and stomach).
Published: 2017
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49. [Psoas and paravesical abscess formation due to Achromobacter xylosoxidans: a case report].

Author: Fraile G, Gallego JI, Martínez-Molina E, and Meseguer MA
Subjects: Aged, Female, Humans, Abscess diagnosis, Abscess therapy, Achromobacter denitrificans, Gram-Negative Bacterial Infections diagnosis, Gram-Negative Bacterial Infections therapy, Psoas Abscess diagnosis, Psoas Abscess therapy
Published: 2010
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50. X-linked reticulate pigmentary disorder: report of a new family.

Author: Fernandez-Guarino M, Torrelo A, Fernandez-Lorente M, Fraile G, García-Sagredo JM, and Jaén P
Subjects: Adult, Female, Genetic Diseases, X-Linked genetics, Humans, Hyperpigmentation pathology, Male, Skin pathology, Genetic Diseases, X-Linked pathology, Hyperpigmentation genetics
Published: 2008
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