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1. SARS-CoV-2 infection in patients with primary Sjögren syndrome: characterization and outcomes of 51 patients

Author: Brito-Zerón, Pilar, Melchor, Sheila, Seror, Raphaèle, Priori, Roberta, Solans, Roser, Kostov, Belchin, Baldini, Chiara, Carubbi, Francesco, Callejas, Jose Luis, Guisado-Vasco, Pablo, Hernández-Molina, Gabriela, Pasoto, Sandra G, Valim, Valeria, Sisó-Almirall, Antoni, Mariette, Xavier, Carreira, Patricia, Ramos-Casals, Manuel, Brito-Zerón, P, Morcillo, C, Flores-Chávez, A, Ramos-Casals, M, Acar-Denizli, N, Horvath, I F, Szanto, A, Tarr, T, Seror, R, Mariette, X, Mandl, T, Olsson, P, Li, X, Xu, B, Baldini, C, Bombardieri, S, Gottenberg, J E, Gandolfo, S, De Vita, S, Priori, R, Giardina, F, Hernandez-Molina, G, Sánchez-Guerrero, J, Kruize, A A, Hinrichs, A, Valim, V, Isenberg, D, Solans, R, Rischmueller, M, Downie-Doyle, S, Kwok, S-K, Park, S-H, Nordmark, G, Suzuki, Y, Kawano, M, Giacomelli, R, Devauchelle-Pensec, V, Saraux, A, Hofauer, B, Knopf, A, Bootsma, H, Vissink, A, Morel, J, Vollenveider, C, Atzeni, F, Retamozo, S, Moça Trevisano, V, Armagan, B, Kilic, L, Kalyoncu, U, Pasoto, S G, Kostov, B, Sisó-Almirall, A, Consani-Fernández, S, Carubbi, F, Callejas, J L, López-Dupla, M, Pérez-Alvarez, R, Akasbi, M, Guisado-Vasco, P, Sánchez, I, Hospital Universitario 12 de Octubre [Madrid], EULAR standing committee of People with Arthritis/Rheumatism in Europe (PARE), H. CIMA-Sanitas, Barcelona, CELLEX-IDIBAPS Department of Autoimmune Diseases, Barcelona, Instituto Mexicano del Seguro Social [Mexico City, Mexico] (IMSS), Universidad de Colima [Mexico], Hospital Clinic [Barcelona, Spain], İstanbul Üniversitesi, İstanbul, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary, MTA-Debreceni Egyetem, Immunologie des maladies virales, auto-immunes, hématologiques et bactériennes (IMVA-HB), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay, Center for Immunology of Viral Infections and Autoimmune Diseases, Le Kremlin Bicêtre, Skåne University Hospital [Malmö, Suède], Stockholm University, Wuhan University [China], Pisa University Hospital, Nuclear Medicine Unit, Humanitas Gavazzeni, Bergamo, Italy, University Hospitals, Strasbourg, Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome] (UNIROMA), Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán - National Institute of Medical Science and Nutrition Salvador Zubiran [Mexico], Federal University of Espírito Santo, University Hospital Vall d’Hebròn [Barcelona, Spain], Lymphocytes B, Autoimmunité et Immunothérapies (LBAI), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-LabEX IGO Immunothérapie Grand Ouest, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Institut de Génétique Moléculaire de Montpellier (IGMM), Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Institut d'Investigaciones Biomèdiques August Pi i Sunye (IDIBAPS), Università degli studi di Palermo - University of Palermo, Hospitales Universitarios de Granada/Universidad de Granada, Hospital Universitario Quironsalud, Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome], Lymphocyte B et Auto-immunité (LBAI), Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Rheumatology Unit, Department of Internal Medicine, University of Palermo, Palermo, Italy, and Michel, Geneviève
Subjects: Male, medicine.medical_specialty, Multivariate analysis, [SDV.IMM] Life Sciences [q-bio]/Immunology, Coronavirus disease 2019 (COVID-19), Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Comorbidity, Laboratory abnormality, comorbidities, outcomes, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Risk Factors, Internal medicine, medicine, Humans, In patient, Pharmacology (medical), 030212 general & internal medicine, Registries, Risk factor, Primary Sjögren Syndrome, AcademicSubjects/MED00360, ComputingMilieux_MISCELLANEOUS, 030203 arthritis & rheumatology, business.industry, SARS-CoV-2, COVID-19, Primary SS, SARS-Cov-2, Middle Aged, medicine.disease, 3. Good health, Hospitalization, Sjogren's Syndrome, [SDV.IMM]Life Sciences [q-bio]/Immunology, Original Article, Female, business
Abstract: Objective To analyse the prognosis and outcomes of SARS-CoV-2 infection in patients with primary SS. Methods We searched for patients with primary SS presenting with SARS-CoV-2 infection (defined following and according to the European Centre for Disease Prevention and Control guidelines) among those included in the Big Data Sjögren Registry, an international, multicentre registry of patients diagnosed according to the 2002/2016 classification criteria. Results A total of 51 patients were included in the study (46 women, mean age at diagnosis of infection of 60 years). According to the number of patients with primary SS evaluated in the Registry (n = 8211), the estimated frequency of SARS-CoV-2 infection was 0.62% (95% CI 0.44, 0.80). All but two presented with symptoms suggestive of COVID-19, including fever (82%), cough (57%), dyspnoea (39%), fatigue/myalgias (27%) and diarrhoea (24%), and the most frequent abnormalities included raised lactate dehydrogenase (LDH) (88%), CRP (81%) and D-dimer (82%) values, and lymphopenia (70%). Infection was managed at home in 26 (51%) cases and 25 (49%) required hospitalization (five required admission to ICU, four died). Compared with patients managed at home, those requiring hospitalization had higher odds of having lymphopenia as laboratory abnormality (adjusted OR 21.22, 95% CI 2.39, 524.09). Patients with comorbidities had an older age (adjusted OR 1.05, 95% CI 1.00, 1.11) and showed a risk for hospital admission six times higher than those without (adjusted OR 6.01, 95% CI 1.72, 23.51) in the multivariate analysis. Conclusion Baseline comorbidities were a key risk factor for a more complicated COVID-19 in patients with primary SS, with higher rates of hospitalization and poor outcomes in comparison with patients without comorbidities.
Published: 2020
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2. Sarcoidosis

Author: Retamozo, Maria Soledad, Brito Zeron, Pilar, Pérez Álvarez, Roberto, Achad, Mario Oscar, Pallarés, Lucio, Cuestas, Eduardo, and Ramos Casals, Manuel
Subjects: Anti tnf, Sarcoidosis, Glucocorticoides, purl.org/becyt/ford/3.2 [https], Tratamiento, purl.org/becyt/ford/3 [https], Azatioprina, Metotrexato
Abstract: La sarcoidosis es una enfermedad sistémica de etiología desconocida que se caracteriza por el desarrollo de granulomas epiteloides no caseificantes. Los pulmones son los órganos más afectados (>90 % de los casos), seguidos de los ganglios linfáticos, la piel y los ojos. Esta revisión resume las principales manifestaciones clínicas y las opciones actuales de farmacoterapia. Los glucocorticoides son la primera línea de tratamiento para la sarcoidosis. Para los pacientes con las formas más severas de sarcoidosis (que necesitarán glucocorticoides durante largos períodos de tiempo) y para aquellos que son intolerantes o resistentes al tratamiento, se utilizan medicamentos inmunosupresores como agentes ahorradores de glucocorticoides. El manejo de la sarcoidosis extratorácica debe adaptarse al órgano u órgano específico involucrado;sin embargo, hay datos limitados de ensayos controlados para guiar el tratamiento de estos pacientes. La aparición de terapias biológicas ha aumentado el arsenal terapéutico disponible para tratar la sarcoidosis, siendo los agentes anti-TNF monoclonales los más prometedores, pero su uso todavía está limitado por la falta de licencias y costos. Sarcoidosis is a systemic disease of unknown etiology characterized by the development of non-caseating epitheloid granulomas. The lungs are the most commonly involved organ (>90% of cases), followed by the lymph nodes, the skin, and the eyes. Areas covered: This review summarizes current pharmacotherapy options and future directions for the development of new therapies. Glucocorticoids are the first-line therapy for sarcoidosis. For patients with the most severe forms of sarcoidosis (who will need glucocorticoids for long periods) and for those intolerant or refractory, immunosuppressive drugs are used as sparing agents. The management of extrathoracic sarcoidosis must be tailored to the specific organ or organs involved;however, there is limited data from controlled trials to guide the treatment of these patients. The emergence of biological therapies has increased the therapeutic armamentarium available to treat sarcoidosis, with monoclonal anti-TNF agents being the most promising, but their use is still limited by a lack of licensing and costs Fil: Retamozo, Maria Soledad. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto de Investigaciones en Ciencias de la Salud. Universidad Nacional de Córdoba. Instituto de Investigaciones en Ciencias de la Salud; Argentina. Instituto Universitario de Ciencias Biomédicas de Córdoba; Argentina Fil: Brito Zeron, Pilar. Hospital Cima-sanitas; España Fil: Pérez Álvarez, Roberto. Sociedad Española de Medicina Interna; España Fil: Achad, Mario Oscar. Universidad Nacional de Córdoba; Argentina Fil: Pallarés, Lucio. Sociedad Española de Medicina Interna; España Fil: Cuestas, Eduardo. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto de Investigaciones en Ciencias de la Salud. Universidad Nacional de Córdoba. Instituto de Investigaciones en Ciencias de la Salud; Argentina Fil: Ramos Casals, Manuel. Sociedad Española de Medicina Interna; España
Published: 2019

3. Osteopontin versus disease activity and damage accrual in recent-onset systemic lupus erythematosus: Results from the Systemic Lupus International Collaborating Clinics Inception Cohort

Author: Wirestam, Lina, Enocsson, Helena, Skogh, Thomas, Padyukov, Leonid, Jönsen, Andreas, Urowitz, Murray, Gladman, Dafna, Romero-Diaz, Juanita, Bae, Sang-Cheol, Fortin, Paul R, Sanchez-Guerrero, Jorge, Clarke, Ann, Bernatsky, Sasha, Gordon, Caroline, Hanly, John G, Wallace, Daniel, Isenberg, David, Rahman, Anisur, Merrill, Joan, Ginzler, Ellen, Alarcón, Graciela S, Chatham, W Winn, Petri, Michelle, Khamashta, Munther, Aranow, Cynthia, Mackay, Meggan, Dooley, Mary Anne, Manzi, Susan, Ramsey-Goldman, Rosalind, Nived, Ola, Steinsson, Kristjan, Zoma, Asad, Ruiz-Irastorza, Guillermo, Lim, Sam, Kalunian, Ken, Inanc, Murat, van Vollenhoven, Ronald, Ramos-Casals, Manuel, Kamen, Diane L, Jacobsen, SØren, Peschken, Christine, Askanase, Anca, Stoll, Thomas, Bruce, Ian N, Wetterö, Jonas, and Sjöwall, Christopher
Subjects: Adult, Male, Asia, Internationality, Adolescent, Enzyme-Linked Immunosorbent Assay, Severity of Illness Index, Article, Young Adult, Sex Factors, stomatognathic system, Reference Values, Humans, Lupus Erythematosus, Systemic, skin and connective tissue diseases, Child, Aged, Age Factors, Middle Aged, Europe, Cross-Sectional Studies, Logistic Models, Multivariate Analysis, North America, Disease Progression, Female, Osteopontin, Biomarkers, Follow-Up Studies
Abstract: In cross-sectional studies, elevated osteopontin (OPN) levels have been proposed to reflect, and/or precede, progressive organ damage and disease severity in systemic lupus erythematosus (SLE). We aimed, in a cohort of patients with recent-onset SLE, to determine whether raised serum OPN levels precede damage and/or are associated with disease activity or certain disease phenotypes.We included 344 patients from the Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort who had 5 years of followup data available. All patients fulfilled the 1997 American College of Rheumatology (ACR) criteria. Baseline sera from patients and from age- and sex-matched population-based controls were analyzed for OPN using ELISA. Disease activity and damage were assessed at each annual followup visit using the SLE Disease Activity Index 2000 (SLEDAI-2K) and the SLICC/ACR damage index (SDI), respectively.Compared to controls, baseline OPN was raised 4-fold in SLE cases (p0.0001). After relevant adjustments in a binary logistic regression model, OPN levels failed to significantly predict global damage accrual defined as SDI ≥ 1 at 5 years. However, baseline OPN correlated with SLEDAI-2K at enrollment into the cohort (r = 0.27, p0.0001), and patients with high disease activity (SLEDAI-2K ≥ 5) had raised serum OPN (p0.0001). In addition, higher OPN levels were found in patients with persistent disease activity (p = 0.0006), in cases with renal involvement (p0.0001) and impaired estimated glomerular filtration rate (p = 0.01).The performance of OPN to predict development of organ damage was not impressive. However, OPN associated significantly with lupus nephritis and with raised disease activity at enrollment, as well as over time.
Published: 2019

4. Phenotype of biopsy-proven patients with primary Sjögren syndrome lacking ro autoantibodies: high frequency of dryness symptoms with low systemic activity (big data Sjögren projct)

Author: Retamozo, Soledad, Acar-Denizli, Nihan, Ng, Wan Fai, Horvath, Ildiko Fanny, Rasmussen, Astrid, Seror, Raphaele, Xiaomei, LI, Baldini, Chiara, Gottenberg, Jacques-Eric, Sandhya, Pulukool, Quartuccio, Luca, Priori, Roberta, Hernandez-Molina, Gabriela, Armagan, Berkan, Kruize, Aike A, Kwok, Seung-Ki, Kvarnström, Marika, Praprotnik, Sonja, Sène, Damien, Gerli, Roberto, Solans-Laqué, Roser, Rischmueller, Maureen, Mandl, Thomas, Suzuki, Yasunori, Isenberg, David, Valim, Valeria, Sebastian, Agata, Nordmark, Gunnel, Bootsma, Hendrika, Nakamura, Hideki, Giacomelli, Roberto, Devauchelle-Pensec, Valérie, Hofauer, Benedikt, Bombardieri, Michele, Trevisani, Virginia Fernandes Moça, Hammenfors, Daniel, Pasoto, Sandra, Gheita, Tamer A, Atzeni, Fabiola, Morel, Jacques, Vollenveider, Cristina, Consani-Fernández, Sandra, Mariette, Xavier, Ramos-Casals, Manuel, Brito-Zerón, Pilar, Michel, Geneviève, INICSA, UNC, CONICED, Cordoba, Mimar Sinan Üniversitesi, University of Newcastle, Newcastle upon Tyne, UK, University of Debrecen Egyetem [Debrecen], Oklahoma Medical Research Foundation (OMRF), INSERM UMR972, SFR André Lwoff, Université Paris Sud, APHP Paul Brousse, Anhui Provincial Hosp, University of Pisa - Università di Pisa, Institut de Physique et Chimie des Matériaux de Strasbourg (IPCMS), Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS)-Matériaux et nanosciences d'Alsace (FMNGE), Université de Strasbourg (UNISTRA)-Université de Haute-Alsace (UHA) Mulhouse - Colmar (Université de Haute-Alsace (UHA))-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut de Chimie du CNRS (INC)-Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA)-Université de Haute-Alsace (UHA) Mulhouse - Colmar (Université de Haute-Alsace (UHA))-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut de Chimie du CNRS (INC)-Centre National de la Recherche Scientifique (CNRS)-Réseau nanophotonique et optique, Université de Strasbourg (UNISTRA)-Université de Haute-Alsace (UHA) Mulhouse - Colmar (Université de Haute-Alsace (UHA))-Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS), Christian Medical College & Hospital, Rheumatology Clinic, Academic Hospital S.M. Della Misericordia, Udine, 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], Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Hacettepe University = Hacettepe Üniversitesi, University Medical Center [Utrecht], Catholic University of Korea, Unit of Experimental Rheumatology, Stockhom (Department of Medicine), Department of Rheumatology (Dep Rheumato - Ljubljana - SLOVENIE), University Medical Centre Slovenia, Université Paris Diderot - Paris 7 (UPD7), University and Azienda Ospedaliera of Perugia, Vall d'Hebron University Hospital [Barcelona], The University of Western Australia (UWA), Malmö University Hospital, Lund University, Kanazawa University (KU), University College of London [London] (UCL), Department of Medicine, Universidade Federal do Espirito Santo, Vitoria, University of Wrocław [Poland] (UWr), Uppsala Universitet [Uppsala], 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), Lymphocytes B, Autoimmunité et Immunothérapies (LBAI), 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), Université de Brest (UBO), Technische Universität Munchen - Université Technique de Munich [Munich, Allemagne] (TUM), Centre for Experimental Medicine and Rheumatology, London, Queen Mary University of London (QMUL), Federal University of Sao Paulo (Unifesp), Haukeland University Hospital, University of Bergen (UiB), Hospital das Clinicas, Cairo University, Messina and Milan Univ, Milan, University Hospital of Montpellier, German Hospital, Buenos Aires, Hospital Maciel, Université Paris-Sud - Paris 11 (UP11), Hospital Clinic (IDIBAPS), H. CIMA-Sanitas, Barcelona, Université de Strasbourg (UNISTRA)-Matériaux et nanosciences d'Alsace (FMNGE), Institut de Chimie du CNRS (INC)-Université de Strasbourg (UNISTRA)-Université de Haute-Alsace (UHA) Mulhouse - Colmar (Université de Haute-Alsace (UHA))-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Institut de Chimie du CNRS (INC)-Université de Strasbourg (UNISTRA)-Université de Haute-Alsace (UHA) Mulhouse - Colmar (Université de Haute-Alsace (UHA))-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre National de la Recherche Scientifique (CNRS)-Réseau nanophotonique et optique, Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA)-Université de Haute-Alsace (UHA) Mulhouse - Colmar (Université de Haute-Alsace (UHA))-Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA), 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)-Institut Brestois Santé Agro Matière (IBSAM), and Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: [SDV.IMM] Life Sciences [q-bio]/Immunology, [SDV.IMM]Life Sciences [q-bio]/Immunology, ComputingMilieux_MISCELLANEOUS
Abstract: International audience
Published: 2019

5. 2019 EULAR RECOMMENDATIONS FOR THE MANAGEMENT OF SJOGREN'S SYNDROME WITH TOPICAL AND SYSTEMIC THERAPIES

Author: Ramos-Casals, Manuel Brito-Zeron, Pilar Bombardieri, Stefano and Bootsma, Hendrika De Vita, Salvatore Doerner, Thomas Fisher, Benjamin A. Gottenberg, Jacques-Eric Hernandez-Molina, Gabriela and Kocher, Agnes Kostov, Belchin Kruize, Aike A. Mandl, Thomas Ng, Wan-Fai Retamozo, Soledad Seror, Raphaele and Shoenfeld, Yehuda Siso-Almirall, Antoni Tzioufas, Athanasios G. and Vitali, Claudio Bowman, Simon Mariette, Xavier and EULAR-Sjogren Syndrome Task Force
Published: 2019

6. 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

7. Phenotyping Sjögren's syndrome: towards a personalised management of the disease

Author: Brito Zeron, Pilar, Retamozo, Maria Soledad, and Ramos-Casals Manuel
Subjects: CIENCIAS MÉDICAS Y DE LA SALUD, Phenotyping, Sjögren's syndrome, Medicina Clínica, Reumatología
Abstract: Sjögren´s syndrome (SS) is a systemic autoimmune disease that mainly targets the exocrine glands. The disease overwhelmingly affects women around 30-60 years old, and more than 95% of patients present with oral and/or ocular dryness, although they may also develop a wide number of organ-specific systemic manifestations. The variable presentation is often linked to the influence of multiple personal determinants. In this review, we analyse the main geoepidemiological, immunological and histopathological determinants involved in the phenotypic expression of SS. With respect to sicca involvement, some patients (Asian, young-onset diagnosis, males and Ro-carriers) present with a less pronounced involvement in contrast to others with more pronounced dryness (seronegative, isolated La-carriers). With respect to the risk of developing systemic disease/poor outcomes, we propose a phenotypic-driven prognostic classification into patients at low risk (elderly-onset diagnosis, seronegative, isolated La-carriers), moderate risk (Black/African-american, young-onset diagnosis, Ro-carriers) and high risk (males, high focus score or presence of germinal centers in histopathological studies, RF-carriers, cryoglobulinaemic and hypocomplementaemic patients). Phenotype-based clustering of systemic autoimmune diseases may help physicians to offer a more personalised, cost-effective medical care of patients affected by these complex chronic diseases. Fil: Brito Zeron, Pilar. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; España. Hospital Cima-sanitas, Barcelona; España. Department Of Autoimmune Diseases, Hospital Clinic; España. Sjögren Syndrome Research Group (agaur); España Fil: Retamozo, Maria Soledad. Department Of Autoimmune Diseases, Hospital Clinic; España. Instituto Universitario de Ciencias Biomédicas de Córdoba; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto de Investigaciones en Ciencias de la Salud. Universidad Nacional de Córdoba. Instituto de Investigaciones en Ciencias de la Salud; Argentina. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; España. Sjögren Syndrome Research Group (agaur); España Fil: Ramos-Casals Manuel. Universidad de Barcelona; España. Department Of Autoimmune Diseases, Hospital Clinic; España. Sjögren Syndrome Research Group (agaur); España. Lab Enf Autoinmunes Josep Font, Cellex, Idibaps; España
Published: 2018

8. 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

9. 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

10. Cerebrovascular Events in Systemic Lupus Erythematosus

Author: Hanly, John G., Li, Qiuju, Su, Li, Urowitz, Murray B., Gordon, Caroline, Bae, Sang-Cheol, Romero-Diaz, Juanita, Sanchez-Guerrero, Jorge, Bernatsky, Sasha, Clarke, Ann E., Wallace, Daniel J., Isenberg, David A., Rahman, Anisur, Merrill, Joan T., Fortin, Paul, Gladman, Dafna D., Bruce, Ian N., Petri, Michelle, Ginzler, Ellen M., Dooley, M. A., Steinsson, Kristjan, Ramsey-Goldman, Rosalind, Zoma, Asad A., Manzi, Susan, Nived, Ola, Jonsen, Andreas, Khamashta, Munther A., Alarcón, Graciela S., Chatham, Winn, van Vollenhoven, Ronald F., Aranow, Cynthia, Mackay, Meggan, Ruiz-Irastorza, Guillermo, Ramos-Casals, Manuel, Lim, S. Sam, Inanc, Murat, Kalunian, Kenneth C., Jacobsen, Soren, Peschken, Christine A., Kamen, Diane L., Askanase, Anca, Theriault, Chris, Farewell, Vernon, AII - Inflammatory diseases, Clinical Immunology and Rheumatology, AMS - Amsterdam Movement Sciences, and AII - Amsterdam institute for Infection and Immunity
Abstract: To determine the frequency, associations and outcomes of cerebrovascular events (CerVEs) in a multi-ethnic/racial, prospective, SLE disease inception cohort. Patients were assessed annually for 19 neuropsychiatric (NP) events including 5 types of CerVEs: (i) Stroke; (ii) Transient ischemia; (iii) Chronic multifocal ischemia; (iv) Subarachnoid/intracranial hemorrhage; (v) Sinus thrombosis. Global disease activity (SLEDAI-2K), SLICC/ACR damage index (SDI) and SF-36 scores were collected. Time to event, linear and logistic regressions and multi-state models were used as appropriate. Of 1,826 SLE patients, 88.8% were female, 48.8% Caucasian, mean±SD age 35.1±13.3 years, disease duration 5.6±4.2 months and follow-up 6.6±4.1 years. CerVEs were the fourth most frequent NP event: 82/1,826 (4.5%) patients had 109 events, 103/109 (94.5%) were attributed to SLE and 44/109 (40.4%) were identified at enrollment. The predominant events were stroke [60/109 (55.0%)] and transient ischemia [28/109 (25.7%)]. CerVEs were associated with other NP events attributed to SLE (HR (95% CI): (3.16; 1.73-5.75) (p
Published: 2018

11. 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

12. Off-label use of rituximab for systemic lupus erythematosus in Europe

Author: Ryden-Aulin, Monica, Boumpas, Dimitrios, Bultink, Irene, Rubio, Jose Luis Callejas, Caminal-Montero, Luis, Castro, Antoni, Colodro Ruiz, Agustin, Doria, Andrea, Domer, Thomas, Gonzalez-Echavarri, Cristina, Gremese, Elisa, Houssiau, Frederic A., Huizinga, Tom, Inana, Murat, Isenberg, David, Luliano, Annamaria, Jacobsen, Soren, Jimenez-Alonso, Juan, Kovacs, Laszlo, Mariette, Xavier, Mosca, Marta, Nived, Ola, Oristrell, Joaquim, Ramos-Casals, Manuel, Rascon, Javier, Ruiz-Irastorza, Guillermo, Saez-Comet, Luis, Salvador Cervello, Gonzalo, Cervello, Gonzalo Salvador, Sebastiani, Gian Domenico, Squatrito, Danilo, Szucs, Gabriella, Voskuyl, Alexandre, van Vollenhoven, Ronald, Rheumatology, AII - Inflammatory diseases, [Ryden-Aulin, Monica] Karolinska Univ Hosp, Karolinska Inst, Dept Med, Unit Clin Therapy Res, Stockholm, Sweden, [van Vollenhoven, Ronald] Karolinska Univ Hosp, Karolinska Inst, Dept Med, Unit Clin Therapy Res, Stockholm, Sweden, [Boumpas, Dimitrios] Natl & Kapodestrian Univ Athens, Attikon Univ Hosp, Joint Acad Rheumatol Program, Med Sch, Athens, Greece, [Boumpas, Dimitrios] Natl & Kapodestrian Univ Athens, Attikon Univ Hosp, Dept Med 4, Med Sch, Athens, Greece, [Bultink, Irene] Amsterdam Rheumatol & Immunol Ctr, Tepartment Rheumatol, Amsterdam, Netherlands, [Rubio, Jose Luis Callejas] Hosp San Cecilio, Unit Autoimmune Dis, Granada, Spain, [Caminal-Montero, Luis] Hosp Univ Cent Asturias, Internal Med Dept, Autoimmune System Dis Unit, Oviedo, Spain, [Castro, Antoni] Rovira & Virgili Univ URV, Univ Hosp Sant Joan Reus, lnternal Med Dept Univ, IISPV, Reus, Spain, [Colodro Ruiz, Agustin] Pasaje Nueva Victoria,2,2do C, Jaen, Spain, [Doria, Andrea] Univ Padua, Dept Med, Rheumatol Unit, Padua, Italy, [Domer, Thomas] Charite Univ Med Berlin, Dept Med Rheumatol & Clin Immunol, Berlin, Germany, [Gonzalez-Echavarri, Cristina] Univ Basque Country, Cruces Univ Hosp, BioCruces Hlth Res Inst, Autoimmune Dis Res Unit,Dept Internal Med, Baracaldo, Spain, [Ruiz-Irastorza, Guillermo] Univ Basque Country, Cruces Univ Hosp, BioCruces Hlth Res Inst, Autoimmune Dis Res Unit,Dept Internal Med, Baracaldo, Spain, [Gremese, Elisa] Univ Cattolica Sacro Cuore, Inst Rheumatol & Affine Sci IRSA, Rome, Italy, [Houssiau, Frederic A.] Catholic Univ Louvain, Pole Pathol Rhumatismales Inflammatoires & Syst, Clin Univ St Luc, Serv Rhumatol, Brussels, Belgium, [Huizinga, Tom] Leiden Univ, Med Ctr, Dept Rheumatol, C1-41, Leiden, Netherlands, [Inana, Murat] Istanbul Univ, Istanbul Fac Med, Dept Internal Med, Div Rheumatol, Istanbul, Turkey, [Isenberg, David] UCL, Rayne Bldg, London, England, [Luliano, Annamaria] San Camillo Hosp, Rheumatol Unit, Rome, Italy, [Jacobsen, Soren] Univ Copenhagen, Rigshosp, Ctr Rheumatol & Spine Dis, Copenhagen Lupus & Vasculitis Clin, Copenhagen, Denmark, [Jimenez-Alonso, Juan] Univ Virgen Nieves Hosp, lnternal Dept, Granada, Spain, [Kovacs, Laszlo] Univ Szeged, Albert Szent Gyorgyi Hlth Ctr, Fac Med, Dept Rheumatol, Szeged, Hungary, [Mariette, Xavier] Univ Paris Sud, Hopitaux Univ Paris Sud, Rhumatol Responsable Unite Rech Clin, INSERM U1184, Paris, France, [Mosca, Marta] Rheumatol Unit, Pisa, Italy, [Nived, Ola] Skane Univ Hosp, Rheumatol Clin, Lund, Sweden, [Oristrell, Joaquim] Univ Autonoma Barcelona, Hosp Sabadell, Internal Med Dept, Catalunya, Spain, [Ramos-Casals, Manuel] CELLEX IDIBAPS, Josep Font Autoimmune Lab, ICMiD, Dept Autoimmune Dis, Barcelona, Spain, [Rascon, Javier] Carrer Sabateres, 9-1,Alare, Islas Baleares, Spain, [Saez-Comet, Luis] Hosp Univ Miguel Servet Zaragoza, Unidad Enfermedades Autoinmunes Sistem, Paseo Isabel Catolica, Zaragoza, Spain, [Salvador Cervello, Gonzalo] Hosp Univ & Politecn La Fe, Inmunopathol & Autoimmune Area, Dept Internal Med, Valencia, Spain, [Sebastiani, Gian Domenico] Osped San Camillo, UOC Reumatol, Circonvallaz Gianicolense 87, Rome, Italy, [Squatrito, Danilo] Univ Florence, Dept Sperimental & Clin Med, Florence, Italy, [Szucs, Gabriella] Univ Debrecen, Inst Med, Dept Rheumatol, Debrecen, Hungary, [Voskuyl, Alexandre] Vrije Univ Amsterdam, Med Ctr, Amsterdam Rheumatol & Immunol Ctr ARC, Amsterdam, Netherlands, [van Vollenhoven, Ronald] Karolinska Univ Hosp Stockholm, Rheumatol Clin, Stockholm, Sweden, [van Vollenhoven, Ronald] Amsterdam Rheumatol & Immunol Ctr ARC, AMC Mail F4-105, Amsterdam, Netherlands, MSD, Lilly Netherlands, Roche, UCB, Sanofi, and RvV
Subjects: medicine.medical_specialty, Disease duration, Immunology, Lupus nephritis, DMARDs (biologic), Klinikai orvostudományok, Off-label use, Systemic Lupus Erythematosus, Article, Disease activity, 03 medical and health sciences, 0302 clinical medicine, immune system diseases, Internal medicine, medicine, Lupus Nephritis, Medicine (all), In patient, 030212 general & internal medicine, skin and connective tissue diseases, 030203 arthritis & rheumatology, business.industry, Orvostudományok, General Medicine, medicine.disease, Disease control, 3. Good health, Rituximab, business, medicine.drug
Abstract: OBJECTIVES: Rituximab (RTX) is a biological treatment used off-label in patients with systemic lupus erythematosus (SLE). This survey aimed to investigate the off-label use of RTX in Europe and compare the characteristics of patients receiving RTX with those receiving conventional therapy.METHODS: Data on patients with SLE receiving RTX were taken from the International Registry for Biologics in SLE retrospective registry and complemented with data on patients with SLE treated with conventional therapy. For nationwide estimates of RTX use in patients with SLE, investigators were asked to provide data through case report forms (CRFs). Countries for which no data were submitted through CRFs, published literature and/or personal communication were used, and for European countries where no data were available, estimates were made on the assumption of similarities with neighbouring countries.RESULTS: The estimated off-label use of RTX in Europe was 0.5%-1.5% of all patients with SLE. In comparison with patients with SLE on conventional therapy, patients treated with RTX had longer disease duration, higher disease activity and were more often treated with immunosuppressives. The most frequent organ manifestations for which either RTX or conventional therapy was initiated were lupus nephritis followed by musculoskeletal and haematological. The reason for treatment was, besides disease control, corticosteroid-sparing for patients treated with conventional therapy.CONCLUSIONS: RTX use for SLE in Europe is restrictive and appears to be used as a last resort in patients for whom other reasonable options have been exhausted.
Published: 2016
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13. Life-Threatening Cryoglobulinemic Patients With Hepatitis C

Author: Retamozo, Soledad, Díaz-Lagares, Cándido, Bosch, Xavier, Bové, Albert, Brito-Zerón, Pilar, Gómez, Maria-Eugenia, Yagüe, Jordi, Forns, Xavier, Cid, Maria C., and Ramos-Casals, Manuel
Subjects: Original Study
Abstract: Cryoglobulinemia is characterized by a wide range of causes, symptoms, and outcomes. Hepatitis C virus (HCV) infection is detected in 30%–100% of patients with cryoglobulins. Although more than half the patients with cryoglobulinemic vasculitis present a relatively benign clinical course, some may present with potentially life-threatening situations. We conducted the current study to analyze the clinical characteristics and outcomes of HCV patients presenting with life-threatening cryoglobulinemic vasculitis. We evaluated 181 admissions from 89 HCV patients diagnosed with cryoglobulinemic vasculitis consecutively admitted to our department between 1995 and 2010. In addition, we performed a systematic analysis of cases reported to date through a MEDLINE search. The following organ involvements were considered to be potentially life-threatening in HCV patients with cryoglobulinemic vasculitis: cryoglobulinemic, biopsy-proven glomerulonephritis presenting with renal failure; gastrointestinal vasculitis; pulmonary hemorrhage; central nervous system (CNS) involvement; and myocardial involvement. A total of 279 patients (30 from our department and 249 from the literature search) fulfilled the inclusion criteria: 205 presented with renal failure, 45 with gastrointestinal vasculitis, 38 with CNS involvement, 18 with pulmonary hemorrhage, and 3 with myocardial involvement; 30 patients presented with more than 1 life-threatening cryoglobulinemic manifestation. There were 146 (52%) women and 133 (48%) men, with a mean age at diagnosis of cryoglobulinemia of 54 years (range, 25–87 yr) and a mean age at life-threatening involvement of 55 years (range, 25–87 yr). In 232 (83%) patients, life-threatening involvement was the first clinical manifestation of cryoglobulinemia. Severe involvement appeared a mean of 1.2 years (range, 1–11 yr) after the diagnosis of cryoglobulinemic vasculitis. Patients were followed for a mean of 14 months (range, 3–120 mo) after the diagnosis of life-threatening cryoglobulinemia. Sixty-three patients (22%) died. The main cause of death was sepsis (42%) in patients with glomerulonephritis, and cryoglobulinemic vasculitis itself in patients with gastrointestinal, pulmonary, and CNS involvement (60%, 57%, and 62%, respectively). In conclusion, HCV-related cryoglobulinemia may result in progressive (renal involvement) or acute (pulmonary hemorrhage, gastrointestinal ischemia, CNS involvement) life-threatening organ damage. The mortality rate of these manifestations ranges between 20% and 80%. Unfortunately, this may be the first cryoglobulinemic involvement in almost two-thirds of cases, highlighting the complex management and very elevated mortality of these cases.
Published: 2013

14. 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

15. Early diagnosis of primary Sjögren's syndrome : EULAR-SS task force clinical recommendations

Author: Brito-Zerón, Pilar, Theander, Elke, Baldini, Chiara, Seror, Raphaèle, Retamozo, Soledad, Quartuccio, Luca, Bootsma, Hendrika, Bowman, Simon J, Dörner, Thomas, Gottenberg, Jacques-Eric, Mariette, Xavier, Bombardieri, Stefano, de Vita, Salvatore, Mandl, Thomas, Ng, Wan-Fai, Kruize, Aike A, Tzioufas, Athanasios, Vitali, Claudio, Buyon, Jill, Izmirly, Peter, Fox, Robert, Ramos-Casals, Manuel, and Eular Sjögren Syndrome Task Force
Subjects: Research Support, Non-U.S. Gov't, Journal Article, Sjögren syndrome, anti-Ro/SS-A antibodies, systemic activity, ESSDAI, classification criteria, focal lymphocytic sialadenitis, early diagnosis, Review
Abstract: Sjögren's syndrome (SjS) is a systemic autoimmune disease that mainly affects the exocrine glands, leading to generalized mucosal dryness. However, primary SjS may initially present with non-sicca (systemic) manifestations. When these features appear before the onset of an overt sicca syndrome, we may talk of an underlying 'occult' SjS. The European League Against Rheumatism (EULAR) has promoted and supported an international collaborative study group (EULAR-SS Task Force) aimed at developing consensual recommendations to provide a homogeneous approach to the patient with primary SjS presenting with systemic involvement. This review summarizes the key factors that should be taken into account in the diagnostic approach in a patient with suspected SjS according to the main clinical patterns of presentation, and is especially focused on organ-specific systemic disease presentations, including a consensus set of recommendations in order to reach an early diagnosis. Close collaboration with the different specialties involved through a comprehensive multidisciplinary approach is essential in SjS patients presenting with systemic involvements.
Published: 2016

16. Off-label use of rituximab for systemic lupus erythematosus in Europe IB: speaker fees from MSD and Lilly Netherlands. TD: study support by Roche, UCB and Sanofi; honoraria for scientific advice from Roche, Chugai, Sanofi, Eli Lilly and UCB. FAH and MI: research grants from Roche. DI: acted as an adviser to a number of companies in the past 5 years, including GlaxoSmithKline, Eli Lilly and Roche; the honoraria offered are passed on to a local arthritis charity. RvV: research support and grants. For performing the analyses reported here, which were requested by the European Medicines Agency (EMA), the authors received an unrestricted grant from Roche. Other: AbbVie, Amgen, BMS, GSK, Pfizer, UCB. Consultancy, honoraria: AbbVie, Biotest, BMS, Celgene, Crescendo, GSK, Janssen, Lilly, Merck, Novartis, Pfizer, Roche, UCB and Vertex

Author: Rydén-Aulin, Monica, Boumpas, Dimitrios, Bultink, Irene, Callejas Rubio, Jose Luis, Caminal-Montero, Luis, Castro, Antoni, Colodro Ruiz, Agustín, Doria, Andrea, Dörner, Thomas, Gonzalez-Echavarri, Cristina, Gremese, Elisa, Houssiau, Frederic A, Huizinga, Tom, Inanç, Murat, Isenberg, David, Iuliano, Annamaria, Jacobsen, Søren, Jimenéz-Alonso, Juan, Kovács, Lászlo, Mariette, Xavier, Mosca, Marta, Nived, Ola, Oristrell, Joaquim, Ramos-Casals, Manuel, Rascón, Javier, Ruiz-Irastorza, Guillermo, Sáez-Comet, Luis, Salvador Cervelló, Gonzalo, Sebastiani, Gian Domenico, Squatrito, Danilo, Szücs, Gabriella, Voskuyl, Alexandre, and van Vollenhoven, Ronald
Subjects: immune system diseases, DMARDs (biologic), skin and connective tissue diseases, Systemic Lupus Erythematosus, Lupus Nephritis
Abstract: Rituximab (RTX) is a biological treatment used off-label in patients with systemic lupus erythematosus (SLE). This survey aimed to investigate the off-label use of RTX in Europe and compare the characteristics of patients receiving RTX with those receiving conventional therapy. Data on patients with SLE receiving RTX were taken from the International Registry for Biologics in SLE retrospective registry and complemented with data on patients with SLE treated with conventional therapy. For nationwide estimates of RTX use in patients with SLE, investigators were asked to provide data through case report forms (CRFs). Countries for which no data were submitted through CRFs, published literature and/or personal communication were used, and for European countries where no data were available, estimates were made on the assumption of similarities with neighbouring countries. The estimated off-label use of RTX in Europe was 0.5%-1.5% of all patients with SLE. In comparison with patients with SLE on conventional therapy, patients treated with RTX had longer disease duration, higher disease activity and were more often treated with immunosuppressives. The most frequent organ manifestations for which either RTX or conventional therapy was initiated were lupus nephritis followed by musculoskeletal and haematological. The reason for treatment was, besides disease control, corticosteroid-sparing for patients treated with conventional therapy. RTX use for SLE in Europe is restrictive and appears to be used as a last resort in patients for whom other reasonable options have been exhausted.
Published: 2016

17. International diagnostic guidelines for patients with HCV-related extrahepatic manifestations. A multidisciplinary expert statement

Author: Ferri, Clodoveo, Ramos-Casals, Manuel, Zignego Anna Linda, Arcaini, Luca, Roccatello, Dario, Antonelli, Alessandro, Saadoun, David, Desbois Anne Claire, Sebastiani, Marco, Casato, Milvia, Lamprecht, Peter, Mangia, Alessandra, Tzioufas Athanasios, G, Younossi Zobair, M, Cacoub, Patrice, ISG-EHCV, Coauthors, and De Santis, Adriano.
Subjects: Vasculitis, medicine.medical_specialty, Referral, Lymphoma, Health Planning Guidelines, Diagnostic guidelines, Immunology, Autoimmunity, Hepacivirus, Sicca syndrome, Hepatitis, 03 medical and health sciences, Therapeutic approach, 0302 clinical medicine, medicine, Humans, Immunology and Allergy, Stage (cooking), Intensive care medicine, 030203 arthritis & rheumatology, Thyroid, Arthritis, Diabetes, Extrahepatic disorders, HCV, Hepatitis C virus, Mixed cryoglobulinemia, Neuropathy, Porphyria, Sjögren's syndrome, Hepatitis C, business.industry, virus diseases, medicine.disease, digestive system diseases, 030211 gastroenterology & hepatology, business, Neurocognitive
Abstract: Hepatitis C virus (HCV) infection is responsible for both hepatic and extra-hepatic disorders (HCV-EHDs); these latter are correlated on one hand clearly with HCV lymphotropism causing immune-system dysregulation as well as with viral oncogenic potential, and on the other hand probably with chronic inflammatory status causing cardio-metabolic complications as well as neurocognitive disturbances. The spectrum of HCV-EHDs ranges from mild or moderate manifestations, such as arthralgia, sicca syndrome, peripheral neuropathy, to severe, life-threatening complications, mainly vasculitis and neoplastic conditions. Given the clinical heterogeneity of HCV-EHDs, HCV-infected individuals are inevitably referred to different specialists according to the presenting/prevalent symptom(s); therefore, the availability of comprehensive diagnostic guidelines is necessary for a patient's whole assessment that is decisive for early diagnosis and correct therapeutic approach of various hepatic and HCV-EHDs, regardless of the specific competencies of different physicians or referral centers. In this respect, a multidisciplinary network of experts, the International Study Group of Extrahepatic Manifestations Related to Hepatitis C Virus Infection (ISG-EHCV), was organized with the intention to formulate diagnostic guidelines for the work-up of possible HCV-EHDs. There was a broad consensus among ISG-EHCV members on the proposed guidelines, which essentially are based on two main levels of patient's assessment. At the referral stage, it is proposed that all patients with HCV infection should be invariably examined by means of first-line diagnostic procedures including virological and hepatic parameter evaluation, as well as the detection of clinical findings that may suggest one or more HCV-EHDs. This preliminary assessment should reveal specific HCV-EHDs, which will be deeper analyzed by means of second-line, targeted investigations. The proposed multidisciplinary expert statement represents the first attempt to draw comprehensive diagnostic guidelines for HCV-infected individuals encompassing the entire spectrum of HCV-related disorders, namely typical hepatic manifestations along with less common, often unpredictable HCV-EHDs. The HCV-EHDs may compromise to a substantial degree the overall disease outcome in a significant number of HCV-infected individuals that renders their timely identification and treatment an imperative. In conclusion, the application of standardized but thorough diagnostic guidelines of HCV-EHDs is advisable at the referral stage as well as during the follow-up period of HCV infected patients. It is envisioned that the proposed strategy will result in improvement of clinical outcomes in such patients.
Published: 2016

18. Mood Disorders in Systemic Lupus Erythematosus: Results From an International Inception Cohort Study

Author: Hanly, John G., Su, Li, Urowitz, Murray B., Romero-Diaz, Juanita, Gordon, Caroline, Bae, Sang-Cheol, Bernatsky, Sasha, Clarke, Ann E., Wallace, Daniel J., Merrill, Joan T., Isenberg, David A., Rahman, Anisur, Ginzler, Ellen M., Petri, Michelle, Bruce, Ian N., Dooley, M. A., Fortin, Paul, Gladman, Dafna D., Sanchez-Guerrero, Jorge, Steinsson, Kristjan, Ramsey-Goldman, Rosalind, Khamashta, Munther A., Aranow, Cynthia, Alarcón, Graciela S., Fessler, Barri J., Manzi, Susan, Nived, Ola, Sturfelt, Gunnar K., Zoma, Asad A., van Vollenhoven, Ronald F., Ramos-Casals, Manuel, Ruiz-Irastorza, Guillermo, Lim, S. Sam, Kalunian, Kenneth C., Inanc, Murat, Kamen, Diane L., Peschken, Christine A., Jacobsen, Soren, Askanase, Anca, Theriault, Chris, Thompson, Kara, Farewell, Vernon, Su, Li [0000-0003-0919-3462], Farewell, Vernon [0000-0001-6704-5295], and Apollo - University of Cambridge Repository
Subjects: Adult, Male, Internationality, Mood Disorders, Incidence, Black People, Hispanic or Latino, Middle Aged, Article, White People, Cohort Studies, Asian People, Disease Progression, Quality of Life, Humans, Lupus Erythematosus, Systemic, Regression Analysis, Female, Prospective Studies, Autoantibodies
Abstract: OBJECTIVE: To examine the frequency, characteristics, and outcome of mood disorders, as well as clinical and autoantibody associations, in a multiethnic/racial, prospective inception cohort of patients with systemic lupus erythematosus (SLE). METHODS: Patients were assessed annually for mood disorders (4 types, according to the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition) and 18 other neuropsychiatric events. Global disease activity scores (SLE Disease Activity Index 2000 [SLEDAI-2K]), damage scores (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index [SDI]), and Short Form 36 subscales, mental and physical component summary scores were collected. Time to event, linear and ordinal regressions, and multi-state models were used as appropriate. RESULTS: Among the 1,827 patients with SLE, 88.9% were female, and 48.9% were Caucasian. The mean ± SD age of the patients was 35.1 ± 13.3 years, disease duration was 5.6 ± 4.8 months, and the length of followup was 4.7 ± 3.5 years. During the course of the study, 863 (47.2%) of the 1,827 patients had 1,627 neuropsychiatric events. Mood disorders occurred in 232 (12.7%) of 1,827 patients, and 98 (38.3%) of 256 mood disorder events were attributed to SLE. The estimated cumulative incidence of any mood disorder after 10 years was 17.7% (95% confidence interval 15.1, 20.2%). A greater risk of mood disorder was associated with concurrent neuropsychiatric events (P ≤ 0.01), and a lower risk was associated with Asian race/ethnicity (P = 0.01) and treatment with immunosuppressive drugs (P = 0.003). Mood disorders were associated with lower mental health and mental component summary scores but not with the SLEDAI-2K, SDI, or lupus autoantibodies. Among the 232 patients with depression, 168 (72.4%) were treated with antidepressants. One hundred twenty-six (49.2%) of 256 mood disorders resolved in 117 (50.4%) of 232 patients. CONCLUSION: Mood disorders, the second most frequent neuropsychiatric event in patients with SLE, have a negative impact on health-related quality of life and improve over time. The lack of association with global SLE disease activity, cumulative organ damage, and lupus autoantibodies emphasizes the multifactorial etiology of mood disorders and a role for non-lupus-specific therapies.
Published: 2015

19. Factors associated with damage accrual in patients with systemic lupus erythematosus: results from the Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort

Author: Bernatsky, Sasha, Ramsey-Goldman, Rosalind, Bae, Sang-Cheol, Rahman, Anisur, Mackay, Meggan, Wallace, Daniel J, Dooley, Mary Anne, Hanly, John G, Sturfelt, Gunnar K, Ginzler, Ellen M, O'Keeffe, Aidan G, Manzi, Susan, Merrill, Joan T, Fortin, Paul R, Ramos-Casals, Manuel, Alarcón, Graciela S, Aranow, Cynthia, Nived, Ola, Steinsson, Kristjan, Bruce, Ian N, Petri, Michelle, Khamashta, Munther A, Sanchez-Guerrero, Jorge, Clarke, Ann E, Gordon, Caroline, Fessler, Barri J, Su, Li, Gladman, Dafna D, Isenberg, David A, Zoma, Asad A, Romero-Diaz, Juanita, and Farewell, Vern
Abstract: Background and aimsWe studied damage accrual and factors determining development and progression of damage in an international cohort of systemic lupus erythematosus (SLE) patients.MethodsThe Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort recruited patients within 15 months of developing four or more 1997 American College of Rheumatology (ACR) criteria for SLE; the SLICC/ACR damage index (SDI) was measured annually. We assessed relative rates of transition using maximum likelihood estimation in a multistate model. The Kaplan–Meier method estimated the probabilities for time to first increase in SDI score and Cox regression analysis was used to assess mortality.ResultsWe recruited 1722 patients; mean (SD) age 35.0 (13.4) years at cohort entry. Patients with damage at enrolment were more likely to have further worsening of SDI (SDI 0 vs ≥1; p
Published: 2015
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20. Impact of early disease factors on metabolic syndrome in systemic lupus erythematosus: data from an international inception cohort

Author: Alarcón, Graciela S, Parker, Ben, Clarke, Ann E, Sanchez-Guerrero, Jorge, Manzi, Susan, Gordon, Caroline, Rahman, Anisur, Sturfelt, Gunnar K, Urowitz, Murray B, Donn, Rachelle, Fortin, Paul R, Petri, Michelle, Ginzler, Ellen M, Isenberg, David A, Wallace, Daniel J, Ramos-Casals, Manuel, Fessler, Barri J, Merrill, Joan T, Dooley, Mary Anne, Khamashta, Munther A, Lunt, Mark, Bernatsky, Sasha, Ramsey-Goldman, Rosalind, Steinsson, Kristjan, Hanly, John G, Gladman, Dafna D, Aranow, Cynthia, Romero-Diaz, Juanita, Mackay, Meggan, Bae, Sang-Cheol, Zoma, Asad A, and Nived, Ola
Abstract: BackgroundThe metabolic syndrome (MetS) may contribute to the increased cardiovascular risk in systemic lupus erythematosus (SLE). We examined the association between MetS and disease activity, disease phenotype and corticosteroid exposure over time in patients with SLE.MethodsRecently diagnosed (1, higher disease activity, increasing age and Hispanic or Black African race/ethnicity were independently associated with MetS over the first 2 years of follow-up in the cohort.ConclusionsMetS is a persistent phenotype in a significant proportion of patients with SLE. Renal lupus, active inflammatory disease and damage are SLE-related factors that drive MetS development while antimalarial agents appear to be protective from early in the disease course.
Published: 2015
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21. Registry of the Spanish network for systemic sclerosis: Survival, prognostic factors, and causes of death

Author: Simeón Aznar, Carmen Pilar, Fonollosa Pla, Vicent, Tolosa Vilella, Carles, Espinosa Garriga, Gerard, Campillo Grau, M., Ramos Casals, Manuel, García Hernández, F.J., Castillo Palma, María Jesús, Sánchez Román, J., Callejas Rubio, José Luis, Ortego Centeno, Norberto, Egurbide Arberas, María Victoria, Trapiella Martínez, Luis, Caminal Montero, L., Sáez Comet, Luis, Velilla Marco, J., Camps García, María Teresa, Ramón Garrido, E . de, Esteban Marcos, E.M., Pallarés Ferreres, Lucio, Navarrete Navarrete, N., Vargas Hitos, José Antonio, Gómez de la Torre, Ricardo, Salvador Cervelló, Gonzalo, Ríos Blanco, Juan José, Vilardell Tarrés, M., Spanish Scleroderma Study Group (SSSG), Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI), Systemic Autoimmune Diseases Group (GEAS), Spanish Scleroderma Study Group (SSSG), Spanish Society of Internal Medicine, Spain, [Simeón-Aznar,CP, Fonollosa-Plá,V, Vilardell-Tarrés,M] Department of Internal Medicine, Hospital Valld’Hebron. [Tolosa-Vilella,C] Department of Internal Medicine, Hospital Parc Taulí, Sabadell. [Espinosa-Garriga,G, Campillo-Grau,M] Department of Autoimmune Diseases, Hospital Clinic. [Campillo-Grau,M] Laboratori of Computacional Medicine, Bioestatistics Unit, Universitat Autònoma de Barcelona, Bellaterra, Barcelona. [García-Hernández,FJ, Castillo-Palma,MJ, Sánchez-Román,J] Unit of Connective Tissue Diseases, Department of Internal Medicine, Hospital Virgen del Rocio, Sevilla. [Callejas-Rubio,JL, Ortego-Centeno,N] Unit of Autoimmune Systemic Diseases, Department of Internal Medicine, Hospital Clínico San Cecilio, Granada. [Egurbide-Arberas,MV] Department of Internal Medicine, Hospital de Cruces, Galdakano, Bilbao. [Trapiellla-Martínez,L] Department of Internal Medicine, Hospital de Cabueñes, Gijón. [Caminal-Montero,L] Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo. [Sáez-Comet,L, Velilla-Marco,J] Department of Internal Medicine, Hospital Miguel Servet, Zaragoza. [Camps-García,MT, de Ramón-Garrido,E] Department of Internal Medicine, Hospital Carlos Haya, Málaga. [Esteban-Marcos,EM, Pallarés-Ferreres,L]Department of Internal Medicine, Hospital Son Espases, Palma de Mallorca. [Navarrete-Navarrete,N, Vargas-Hitos,JA] Department of Internal Medicine, Hospital Virgen de las Nieves, Granada. [Gómez de la Torre,R] Department of Internal Medicine, Hospital San Agustín, Avilés. [Salvador-Cervello,G] Department of Internal Medicine, Hospital La Fe, Valencia. [Rios-Blanco,JJ] Department of Internal Medicine, Hospital La Paz, Madrid, and Universitat de Barcelona
Subjects: Male, Multivariate analysis, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Models, Statistical::Proportional Hazards Models [Medical Subject Headings], sistema de registros, Diseases::Respiratory Tract Diseases::Lung Diseases::Hypertension, Pulmonary [Medical Subject Headings], Autoimmune diseases, humanos, Scleroderma Renal Crisis, España, Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::Ulcer [Medical Subject Headings], Scleroderma, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Probability::Risk::Risk Factors [Medical Subject Headings], Diseases::Respiratory Tract Diseases::Lung Diseases::Lung Diseases, Interstitial [Medical Subject Headings], Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic [Medical Subject Headings], Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans [Medical Subject Headings], Úlcera, Risk Factors, Cause of Death, Registries, Masculino, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Morbidity::Prevalence [Medical Subject Headings], Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic::Scleroderma, Limited [Medical Subject Headings], mediana edad, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Mortality::Cause of Death [Medical Subject Headings], Cause of death, Esclerodermia limitada, anciano, Esclerodermia difusa, Malalties autoimmunitàries, Interstitial lung disease, Pronóstico, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Questionnaires [Medical Subject Headings], General Medicine, adulto, Middle Aged, Modelos de riesgos proporcionales, Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic::Scleroderma, Limited::CREST Syndrome [Medical Subject Headings], Humanos, Encuestas y cuestionarios, Female, Factores de riesgo, Adult, medicine.medical_specialty, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Analysis of Variance::Multivariate Analysis [Medical Subject Headings], Check Tags::Male [Medical Subject Headings], causas de muerte, Internal medicine, Progresión de la enfermedad, Análisis multivariante, medicine, factores de riesgo, Tasa de supervivencia, Humans, Espanya, Survival rate, Aged, Retrospective Studies, Geographical Locations::Geographic Locations::Europe::Spain [Medical Subject Headings], Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Scleroderma, Systemic::Scleroderma, Diffuse [Medical Subject Headings], Scleroderma, Systemic, Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::Disease Attributes::Disease Progression [Medical Subject Headings], Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Mortality::Survival Rate [Medical Subject Headings], business.industry, estudios retrospectivos, Retrospective cohort study, medicine.disease, Pulmonary hypertension, Surgery, Causas de muerte, Scleroderma (Disease), Enfermedades pulmonares Intersticiales, Spain, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Prognosis [Medical Subject Headings], Esclerodèrmia, business, Síndrome CREST, Prevalencia, Hipertensión pulmonar
Abstract: Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan-Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, P
Published: 2015

22. Factors influencing dry mouth in patients with primary Sjögren syndrome: usefulness of the ESSPRI index

Author: Gandía, Myriam, Morales-Espinoza, Enma MarianelaM., Martín-González, Rosa Ma., Retamozo, María Soledad, Kostov, Belchin, Belenguer-Prieto, Rafael, Buss, David, Caballero Borrego, Miguel, Bové, Albert, Gueitasi, Hoda, Brito-Zerón, Pilar, Sisó Almirall, Antoni, Soto-Cárdenas, Maria José, Ramos Casals, Manuel, and Universitat de Barcelona
Subjects: Diabetis, Malalties autoimmunitàries, Autoimmune diseases, Diabetes, Pain, Anxiety, humanities, stomatognathic diseases, Ansietat, Mental depression, Estudi de casos, Sjogren's syndrome, Síndrome de Sjögren, Case studies, Dolor, Depressió psíquica
Abstract: To evaluate health-related quality of life in a large series of primary SS patients using the recently-proposed ESSPRI questionnaire and to evaluate the relationship between the intensity of oral dryness and other signs and symptoms frequently found in these patients. METHODS: We evaluated 90 primary SS patients seen consecutively; all fulfilled the current classification criteria. All patients completed the ESSPRI questionnaire. We compared the mean values of the ESSPRI-dry mouth item with other ESSPRI items related to sicca features, general symptoms, quality of life, quality of sleep, psychological and psychiatric features, extraglandular involvement, laboratory features and immunological markers and cardiovascular risk factors. Multivariate regression analysis with a backwards stepwise selection method was performed to identify those variables that were independently associated with dry mouth. RESULTS: Mean intensity of oral dryness measured by the corresponding ESSPRI item was 7.17±0.23. Oral dryness correlated with age both at diagnosis and at study inclusion (p=0.013), but not with gender or with time of disease evolution. No significant correlation was found with the SF-36, HAQ and FIQ questionnaires. We found a significant correlation between the intensity of oral dryness and the quality of sleep (p=0.001), anxiety and depression measured by the GH28 (p=0.004 and 0.024, respectively), and a statistically-significant trend for anxiety and depression measured by the HADS (p=0.08 and 0.07, respectively). No significant correlation was found with the main extraglandular and immunological features; however, a significant correlation between oral dryness and hypertension (p=0.019), type II diabetes mellitus (p=0.005) and hypercholesterolemia (p=0.011) was found. Multivariate regression analysis shows that fatigue measured by ESSPRI (p=0.049), sleep quality (p=0.008) and hypercholesterolemia (p=0.008) were independently associated with dry mouth. CONCLUSION: We report on the usefulness of the ESSPRI index in evaluating HRQOL associated with oral dryness in primary SS patients. Oral dryness correlated with age and the other sicca symptoms measured by ESSPRI, but not with the main systemic and immunological SS features. In contrast, oral dryness was strongly correlated with fatigue, pain, psychological distress, poor sleep and vascular risk factors. A multidisciplinary therapeutic approach may be the best way of minimizing oral dryness and its consequences in primary SS patients
Published: 2014

23. Hepatitis G virus infection in primary Sjögren's syndrome: analysis in a series of 100 patients

Author: Font Franco, Josep, 1953-2006, Tàssies Penella, María Dolores, García Carrasco, Mario, Ramos Casals, Manuel, Cervera i Segura, Ricard, 1960, Reverter Calatayud, Juan Carlos, Sánchez Tapias, José M. (José María), Mazzara Aguirrezabal, Roberto, and Ingelmo Morín, Miguel
Subjects: Hepatitis G, Estudi de casos, Epidemiology, Síndrome de Sjögren, Sjögren's syndrome, Case studies, Epidemiologia
Abstract: OBJECTIVE To determine the prevalence and clinical significance of hepatitis G virus (HGV) infection in a large cohort of patients with primary Sjögren¿s syndrome (SS). PATIENTS AND METHODS The study included 100 consecutive patients (92 female and eight male), with a mean age of 62 years (range 31¿80) that were prospectively visited in our unit. All patients fulfilled the European Community criteria for SS and underwent a complete history, physical examination, as well as biochemical and immunological evaluation for liver disease. Two hundred volunteer blood donors were also studied. The presence of HGV-RNA was investigated in the serum of all patients and donors. Aditionally, HBsAg and antibodies to hepatitis C virus were determined. RESULTS Four patients (4%) and six volunteer blood donors (3%) presented HGV-RNA sequences in serum. HGV infection was associated with biochemical signs of liver involvement in two (50%) patients. When compared with primary SS patients without HGV infection, no significant differences were found in terms of clinical or immunological features. HCV coinfection occurs in one (25%) of the four patients with HGV infection. CONCLUSION The prevalence of HGV infection in patients with primary SS is low in the geographical area of the study and HCV coinfection is very uncommon. HGV infection alone does not seen to be an important cause of chronic liver injury in the patients with primary SS in this area.
Published: 2014

24. Validation of the classification criteria for cryoglobulinaemic vasculitis

Author: Quartuccio, Luca Isola, Miriam Corazza, Laura Ramos-Casals, Manuel Retamozo, Soledad Ragab, Gaafar Mohamed Zoheir, Mostafa Naguib El-Menyawi, Manal Abdel-Moneim Salem, Mohamed Nabil Sansonno, Domenico Ferraccioli, Gianfranco Gremese, Elisa Tzioufas, Athanasios Voulgarelis, Michael and Vassilopoulos, Dimitris Scarpato, Salvatore Pipitone, Nicolo and Salvarani, Carlo Guillevin, Loic Terrier, Benjamin Cacoub, Patrice Filippini, Davide Saccardo, Francesco Gabrielli, Armando Fraticelli, Paolo Sebastiani, Marco Tomsic, Matija and Tavoni, Antonio Mazzaro, Cesare Pioltelli, Pietro and Nishimoto, Norihiro Scaini, Patrizia Zignego, Anna Linda and Ferri, Clodoveo Monti, Giuseppe Pietrogrande, Maurizio and Bombardieri, Stefano Galli, Massimo De Vita, Salvatore
Abstract: Objective. The aim of this study was to validate the classification criteria for cryoglobulinaemic vasculitis (CV). Methods. Twenty-three centres were involved. New patients with CV (group A) and controls, i.e. subjects with serum cryoglobulins but lacking CV based on the gold standard of clinical judgment (group B) and subjects without cryoglobulins but with clinical features that can be observed in the course of CV (group C), were studied. Positivity of serum cryoglobulins was necessary for CV classification. Sensitivity and specificity of the criteria were calculated by comparing group A vs group B. The group A vs group C comparison was done to demonstrate the possible diagnostic utility of the criteria. Results. The study included 268 patients in group A, 182 controls in group B and 193 controls in group C (small vessel vasculitis, 51.8%). The questionnaire (at least 2/3 positive answers) showed 89.0% sensitivity and 93.4% specificity; the clinical item (at least 3/4 clinical involvement) showed 75.7% sensitivity and 89.0% specificity and the laboratory item (at least 2/3 laboratory data) showed 80.2% sensitivity and 62.4% specificity. The sensitivity and specificity of the classification criteria (at least 2/3 positive items) were 89.9% and 93.5%, respectively. The comparison of group A with group C demonstrated the clinical utility of the criteria in differentiating CV from CV mimickers. Conclusion. Classification criteria for CV were validated in a second, large, international study confirming good sensitivity and specificity in a complex systemic disease.
Published: 2014

25. Cardiovascular Risk in Systemic Autoimmune Diseases: Epigenetic Mechanisms of Immune Regulatory Functions

Author: López-Pedrera, Chary, Pérez-Sánchez, Carlos, Ramos-Casals, Manuel, Santos-Gonzalez, Monica, Rodriguez-Ariza, Antonio, and José Cuadrado, Ma
Subjects: Article Subject
Abstract: Autoimmune diseases (AIDs) have been associated with accelerated atherosclerosis (AT) leading to increased cardio- and cerebrovascular disease risk. Traditional risk factors, as well as systemic inflammation mediators, including cytokines, chemokines, proteases, autoantibodies, adhesion receptors, and others, have been implicated in the development of these vascular pathologies. Yet, the characteristics of vasculopathies may significantly differ depending on the underlying disease. In recent years, many new genes and signalling pathways involved in autoimmunity with often overlapping patterns between different disease entities have been further detected. Epigenetics, the control of gene packaging and expression independent of alterations in the DNA sequence, is providing new directions linking genetics and environmental factors. Epigenetic regulatory mechanisms comprise DNA methylation, histone modifications, and microRNA activity, all of which act upon gene and protein expression levels. Recent findings have contributed to our understanding of how epigenetic modifications could influence AID development, not only showing differences between AID patients and healthy controls, but also showing how one disease differs from another and even how the expression of key proteins involved in the development of each disease is regulated.
Published: 2012
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26. Seizure disorders in systemic lupus erythematosus results from an international, prospective, inception cohort study

Author: Khamashta, Munther A, Fessler, Barri J, Petri, M, Kalunian, Kenneth C, Mackay, Meggan, Gladman, DD, Zoma, Asad A, Nived, Ola, Ramos-Casals, Manuel, Dooley, MA, Isenberg, David A, Merrill, Joan T, Su, Li, Bruce, Ian N, Romero-Diaz, Juanita, van Vollenhoven, RF, Hanly, John G, Aranow, Cynthia, Ginzler, EM, Alarcón, Graciela S, Bae, Sang-Cheol, Ramsey-Goldman, Rosalind, Fortin, Paul R, Steinsson, Kristjan, Rahman, Anisur, Gordon, Caroline, Wallace, Daniel J, Urowitz, Murray B, Sturfelt, Gunnar K, Sanchez-Guerrero, Jorge, Clarke, Ann E, and Manzi, Susan
Subjects: immune system diseases, skin and connective tissue diseases
Abstract: To describe the frequency, attribution, outcome and predictors of seizures in SLE
Published: 2012
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27. Treatment Approaches in Primary Sjogren Syndrome Reply

Author: Ramos-Casals, Manuel Tzioufas, Athanasios G. Stone, John H.
Published: 2010

28. Sjogren's Syndrome

Author: Ramos-Casals, Manuel Daniels, Troy E. Fox, Robert I. and Whitcher, John P. Fragoulis, George E. Skopouli, Fotini N. and Moutsopoulos, Haralampos M.
Published: 2009

29. Rates of, and risk factors for, severe infections in patients with systemic autoimmune diseases receiving biological agents off-label

Author: Diaz-Lagares, Candido, Perez-Alvarez, Roberto, Garcia-Hernandez, Francisco J., Ayala-Gutierrez, Maria M., Luis Callejas, Jose, Martinez-Berriotxoa, Agustin, Rascon, Javier, Caminal-Montero, Luis, Selva-O'Callaghan, Albert, Oristrell, Joaquim, Hidalgo, Carmen, Gomez-de-la-Torre, Ricardo, Saez, Luis, Canora-Lebrato, Jesus, Camps, Maria-Teresa, Ortego-Centeno, Norberto, Castillo-Palma, Maria-Jesus, Ramos-Casals, Manuel, and BIOGEAS Study Grp
Subjects: Male, modelos de riesgos proporcionales, humanos, enfermedades autoinmunes, adolescente, Kaplan-Meier Estimate, vasculitis, Etanercept, rituximab, Infection rate, systemic lupus erythematosus, Risk Factors, adalimumab, Immunology and Allergy, Sjogren syndrome, Young adult, mediana edad, Aged, 80 and over, anciano, Middle Aged, adulto, adulto joven, Female, productos biológicos, Rituximab, prescripción en indicaciones no aprobadas, Infection, Vasculitis, Research Article, medicine.drug, Adult, estimación de Kaplan-Meier, medicine.medical_specialty, Adolescent, Immunology, macromolecular substances, Infections, Autoimmune Diseases, Young Adult, Rheumatology, Internal medicine, Adalimumab, medicine, Humans, factores de riesgo, Aged, Proportional Hazards Models, Biological Products, business.industry, Off-Label Use, infección, medicine.disease, Sjögren syndrome, Infliximab, Observational study, infliximab, business, etanercept
Abstract: Introduction: The purpose of this observational study was to analyze the rates, characteristics and associated risk factors of severe infections in patients with systemic autoimmune diseases (SAD) who were treated off-label with biological agents in daily practice. Methods: The BIOGEAS registry is an ongoing Spanish prospective cohort study investigating the long-term safety and efficacy of the off-label use of biological agents in adult patients with severe, refractory SAD. Severe infections were defined according to previous studies as those that required intravenous treatment or that led to hospitalization or death. Patients contributed person-years of follow-up for the period in which they were treated with biological agents. Results: A total of 344 patients with SAD treated with biological agents off-label were included in the Registry until July 2010. The first biological therapies included rituximab in 264 (77%) patients, infliximab in 37 (11%), etanercept in 21 (6%), adalimumab in 19 (5%), and 'other' agents in 3 (1%). Forty-five severe infections occurred in 37 patients after a mean follow-up of 26.76 months. These infections resulted in four deaths. The crude rate of severe infections was 90.9 events/1000 person-years (112.5 for rituximab, 76.9 for infliximab, 66.9 for adalimumab and 30.5 for etanercept respectively). In patients treated with more than two courses of rituximab, the crude rate of severe infection was 226.4 events/1000 person-years. A pathogen was identified in 24 (53%) severe infections. The most common sites of severe infection were the lower respiratory tract (39%), bacteremia/sepsis (20%) and the urinary tract (16%). There were no significant differences relating to gender, SAD, agent, other previous therapies, number of previous immunosuppressive agents received or other therapies administered concomitantly. Cox regression analysis showed that age (P = 0.015) was independently associated with an increased risk of severe infection. Survival curves showed a lower survival rate in patients with severe infections (log-rank and Breslow tests < 0.001). Conclusions: The rates of severe infections in SAD patients with severe, refractory disease treated depended on the biological agent used, with the highest rates being observed for rituximab and the lowest for etanercept. The rate of infection was especially high in patients receiving three or more courses of rituximab. In patients with severe infections, survival was significantly reduced. Older age was the only significant predictive factor of severe infection., The BIOGEAS Study group has received educational grants from Roche and Abbott supporting the design and maintenance of the webpage [35]. All authors have declared no conflicts of interest. None has received grants from these laboratories or conducted clinical trials with rituximab or etanercept as principal investigators or received honoraria as an Advisory Board member for Roche and Abbott. The financial support of Roche and Abbott is exclusively limited to maintaining the BIOGEAS webpage.
Published: 2011
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30. Characterization and differentiation of autoimmune versus viral liver involvement in patients with Sjögren's syndrome

Author: Ramos-Casals, Manuel, Sanchez-Tapias, Jose-Maria, Albert Pares, Forns, Xavier, Brito-Zeron, Pilar, Nardi, Norma, Vazquez, Pilar, Velez, Desiree, Arias, Isabel, Bove, Albert, Plaza, Joan, Rodes, Juan, and Font, Josep

31. 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

32. Outcomes After Hydroxychloroquine Reduction or Discontinuation in a Multinational Inception Cohort of Systemic Lupus

Author: Almeida-Brasil, Celline, Hanly, John, Urowitz, Murray, Clarke, Ann, Ramsey-Goldman, Rosalind, Gordon, Caroline, Petri, Michelle, Ginzler, Ellen M., Wallace, Daniel J., Bae, Sang-Cheol, Romero-Diaz, Juanita, Dooley, Mary Ann, Peschken, Christine A., Isenberg, David, Rahman, Anisur, Manzi, Susan, Jacobsen, Soren, Lim, S. Sam, Vollenhoven, Ronald, Nived, Ola, Jonsen, Andreas, Kamen, Diane, Aranow, Cynthia, Guillermo Ruiz-Irastorza, Sanchez-Guerrero, Jorge, Gladman, Dafna, Fortin, Paul, Alarcon, Graciela, Merrill, Joan, Kalunian, Kenneth, Ramos-Casals, Manuel, Steinsson, Kristjan, Zoma, Asad, Askanase, Anca, Khamashta, Munther, Bruce, Ian, Inanc, Murat, and Bernatsky, Sasha

33. Cerebrovascular Events in Systemic Lupus Erythematosus: Results From an International Inception Cohort Study

Author: Hanly, John G, Li, Qiuju, Su, Li, Urowitz, Murray B, Gordon, Caroline, Bae, Sang-Cheol, Romero-Diaz, Juanita, Sanchez-Guerrero, Jorge, Bernatsky, Sasha, Clarke, Ann E, Wallace, Daniel J, Isenberg, David A, Rahman, Anisur, Merrill, Joan T, Fortin, Paul, Gladman, Dafna D, Bruce, Ian N, Petri, Michelle, Ginzler, Ellen M, Dooley, MA, Steinsson, Kristjan, Ramsey-Goldman, Rosalind, Zoma, Asad A, Manzi, Susan, Nived, Ola, Jonsen, Andreas, Khamashta, Munther A, Alarcón, Graciela S, Chatham, Winn, Van Vollenhoven, Ronald F, Aranow, Cynthia, Mackay, Meggan, Ruiz-Irastorza, Guillermo, Ramos-Casals, Manuel, Lim, S Sam, Inanc, Murat, Kalunian, Kenneth C, Jacobsen, Soren, Peschken, Christine A, Kamen, Diane L, Askanase, Anca, Theriault, Chris, and Farewell, Vernon
Subjects: Adult, Male, Cerebrovascular Disorders, Young Adult, Quality of Life, Humans, Lupus Erythematosus, Systemic, Female, Prospective Studies, Middle Aged, skin and connective tissue diseases, 3. Good health
Abstract: OBJECTIVE: To determine the frequency, characteristics, and outcomes of cerebrovascular events (CerVEs), as well as clinical and autoantibody associations in a multiethnic/racial inception cohort of patients with systemic lupus erythematosus (SLE). METHODS: A total of 1,826 patients were assessed annually for 19 neuropsychiatric (NP) events, including 5 types of CerVEs: 1) stroke, 2) transient ischemia, 3) chronic multifocal ischemia, 4) subarachnoid/intracranial hemorrhage, and 5) sinus thrombosis. Global disease activity (Systemic Lupus Erythematosus Disease [SLE] Activity Index 2000), damage scores (SLE International Collaborating Clinics/American College of Rheumatology Damage Index), and Short Form 36 (SF-36) scores were collected. Time to event, linear and logistic regressions, and multistate models were used as appropriate. RESULTS: CerVEs were the fourth most frequent NP event: 82 of 1,826 patients had 109 events; of these events, 103 were attributed to SLE, and 44 were identified at the time of enrollment. The predominant events were stroke (60 of 109 patients) and transient ischemia (28 of 109 patients). CerVEs were associated with other NP events attributed to SLE, non-SLE-attributed NP events, African ancestry (at US SLICC sites), and increased organ damage scores. Lupus anticoagulant increased the risk of first stroke and sinus thrombosis and transient ischemic attack. Physician assessment indicated resolution or improvement in the majority of patients, but patients reported sustained reduction in SF-36 summary and subscale scores following a CerVE. CONCLUSION: CerVEs, the fourth most frequent NP event in SLE, are usually attributable to lupus. In contrast to good physician-reported outcomes, patients reported a sustained reduction in health-related quality of life following a CerVE.

34. Mood Disorders in Systemic Lupus Erythematousus (SLE): Results from an International, Inception Cohort Study

Author: Hanly, John G., Su, Li, Urowitz, Murray, Romero-Diaz, Juanita, Gordon, Caroline, Bae, Sang-Cheol, Bernatsky, Sasha R., Clarke, Ann E., Wallace, Daniel J., Merrill, Joan T., Isenberg, David A., Rahman, Anisur, Ginzler, Ellen M., Fortin, Paul, Gladman, Dafna D., Sanchez-Guerrero, Jorge, Petri, Michelle A., Bruce, Ian, Dooley, Mary Anne, Ramsey-Goldman, Rosalind, Aranow, Cynthia, Alarcon, Graciela S., Fessler, Barri J., Steinsson, Kristjan, Nived, Ola, Sturfelt, Gunnar K., Manzi, Susan, Khamashta, Munther A., Vollenhoven, Ronald F., Zoma, Asad, Ramos-Casals, Manuel, Guillermo Ruiz-Irastorza, Lim, S. Sam, Stoll, Thomas, Inanc, Murat, Kalunian, Kenneth C., Kamen, Diane L., Maddison, Peter, Peschken, Christine A., Jacobsen, Soren, Askanase, Anca, Buyon, Jill P., Theriault, Chris, Thompson, Kara, and Farewell, Vernon
Subjects: Clinical Sciences, Immunology, Public Health and Health Services, Arthritis & Rheumatology

35. 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

36. Prediction of Organ Damage Accrual in Systemic Lupus Erythematosus Using a Frailty Index

Author: Legge, Alexandra, Kirkland, Susan, Rockwood, Kenneth, Andreou, Pantelis, Bae, Sang-Cheol, Gordon, Caroline, Romero-Diaz, Juanita, Sanchez-Guerrero, Jorge, Wallace, Daniel J., Bernatsky, Sasha, Clarke, Ann E., Merrill, Joan, Ginzler, Ellen M., Fortin, Paul, Gladman, Dafna, Urowitz, Murray, Bruce, Ian, Isenberg, David A., Rahman, Anisur, Alarcon, Graciela, Petri, Michelle, Khamashta, Munther A., Dooley, M. A., Ramsey-Goldman, Rosalind, Manzi, Susan, Steinsson, Kristjan, Zoma, Asad A., Aranow, Cynthia, Mackay, Meggan, Guillermo Ruiz-Irastorza, Lim, S. Sam, Inanc, Murat, Vollenhoven, Ronald F., Jonsen, Andreas, Nived, Ola, Ramos-Casals, Manuel, Kamen, Diane, Kalunian, Kenneth C., Jacobsen, Soren, Peschken, Christine, Askanase, Anca, and Hanly, John G.

37. Clinical associations of the metabolic syndrome in systemic lupus erythematosus: data from an international inception cohort

Author: Romero-Diaz, Juanita, Fortin, Paul R., Kalunian, Kenneth C., Isenberg, David A., Manzi, Susan, Bae, Sang Cheol, Ramsey-Goldman, Rosalind, Inanc, Murat, Sanchez-Guerrero, Jorge, MacKay, Meggan, Lunt, Mark, Dooley, Mary Anne, Van Vollenhoven, Raymond F., Merrill, Joan T., Fessler, Barri J., Kamen, Diane L., Steinsson, Kristjan, Sturfelt, Gunnar K., Hanly, John G., Wallace, Daniel J., Rahman, Anisur, Khamashta, Munther A., Aranow, Cynthia, Clarke, Ann E., Nived, Ola, Bruce, Ian N., Gordon, Caroline, Gladman, Dafna D., Petri, Michelle, Parker, Ben, Lim, Sam, Peschken, Christine A., Alarcón, Graciela S., Ramos-Casals, Manuel, Ruiz-Irastorza, Guillermo, Urowitz, Murray B., Zoma, Asad A., Ginzler, Ellen M., and Bernatsky, Sasha
Subjects: 3. Good health
Abstract: BackgroundThe metabolic syndrome (MetS) may contribute to increased cardiovascular risk in systemic lupus erythematosus (SLE). We aimed to examine the association of demographic factors, lupus phenotype and therapy exposure with the presence of MetS.MethodsThe Systemic Lupus International Collaborating Clinics Registry for Atherosclerosis inception cohort enrolled recently diagnosed (

38. The Initial Presentation of Cerebrovascular Disease Attributed to Lupus Is Most Frequent Early in the Disease Course: Results from an International, Inception Cohort Study

Author: Hanly, John G., Li, Qiuju, Su, Li, Urowitz, Murray, Romero-Diaz, Juanita, Gordon, C., Bae, Sang-Cheol, Bernatsky, Sasha R., Clarke, Ann, Wallace, Daniel J., Merrill, Joan T., Isenberg, David A., Rahman, Anisur, Ginzler, Ellen M., Petri, Michelle, Bruce, Ian N., Dooley, Mary Anne, Fortin, Paul, Gladman, Dafna D., Sanchez-Guerrero, Jorge, Steinsson, Kristjan, Ramsey-Goldman, Rosalind, Khamashta, Munther, Aranow, Cynthia, Alarcon, Graciela S., Fessler, Barri J., Manzi, Susan, Nived, Ola, Sturfelt, Gunnar K., Zoma, Asad, Vollenhoven, Ronald F., Ramos-Casals, Manuel, Guillermo Ruiz-Irastorza, Lim, S. Sam, Kalunian, Kenneth C., Inanc, Murat, Kamen, Diane L., Peschken, Christine A., Jacobsen, Soren, Askanase, Anca, Theriault, Chris, and Farewell, Vernon

39. Mood Disorders in Systemic Lupus Erythematosus: Results From an International Inception Cohort Study

Author: Hanly, John G, Su, Li, Urowitz, Murray B, Romero-Diaz, Juanita, Gordon, Caroline, Bae, Sang-Cheol, Bernatsky, Sasha, Clarke, Ann E, Wallace, Daniel J, Merrill, Joan T, Isenberg, David A, Rahman, Anisur, Ginzler, Ellen M, Petri, Michelle, Bruce, Ian N, Dooley, MA, Fortin, Paul, Gladman, Dafna D, Sanchez-Guerrero, Jorge, Steinsson, Kristjan, Ramsey-Goldman, Rosalind, Khamashta, Munther A, Aranow, Cynthia, Alarcón, Graciela S, Fessler, Barri J, Manzi, Susan, Nived, Ola, Sturfelt, Gunnar K, Zoma, Asad A, Van Vollenhoven, Ronald F, Ramos-Casals, Manuel, Ruiz-Irastorza, Guillermo, Lim, S Sam, Kalunian, Kenneth C, Inanc, Murat, Kamen, Diane L, Peschken, Christine A, Jacobsen, Soren, Askanase, Anca, Theriault, Chris, Thompson, Kara, and Farewell, Vernon
Subjects: Adult, Male, Internationality, Mood Disorders, Incidence, Black People, Hispanic or Latino, Middle Aged, White People, 3. Good health, Cohort Studies, Asian People, Disease Progression, Quality of Life, Humans, Lupus Erythematosus, Systemic, Regression Analysis, Female, Prospective Studies, Autoantibodies
Abstract: OBJECTIVE: To examine the frequency, characteristics, and outcome of mood disorders, as well as clinical and autoantibody associations, in a multiethnic/racial, prospective inception cohort of patients with systemic lupus erythematosus (SLE). METHODS: Patients were assessed annually for mood disorders (4 types, according to the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition) and 18 other neuropsychiatric events. Global disease activity scores (SLE Disease Activity Index 2000 [SLEDAI-2K]), damage scores (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index [SDI]), and Short Form 36 subscales, mental and physical component summary scores were collected. Time to event, linear and ordinal regressions, and multi-state models were used as appropriate. RESULTS: Among the 1,827 patients with SLE, 88.9% were female, and 48.9% were Caucasian. The mean ± SD age of the patients was 35.1 ± 13.3 years, disease duration was 5.6 ± 4.8 months, and the length of followup was 4.7 ± 3.5 years. During the course of the study, 863 (47.2%) of the 1,827 patients had 1,627 neuropsychiatric events. Mood disorders occurred in 232 (12.7%) of 1,827 patients, and 98 (38.3%) of 256 mood disorder events were attributed to SLE. The estimated cumulative incidence of any mood disorder after 10 years was 17.7% (95% confidence interval 15.1, 20.2%). A greater risk of mood disorder was associated with concurrent neuropsychiatric events (P ≤ 0.01), and a lower risk was associated with Asian race/ethnicity (P = 0.01) and treatment with immunosuppressive drugs (P = 0.003). Mood disorders were associated with lower mental health and mental component summary scores but not with the SLEDAI-2K, SDI, or lupus autoantibodies. Among the 232 patients with depression, 168 (72.4%) were treated with antidepressants. One hundred twenty-six (49.2%) of 256 mood disorders resolved in 117 (50.4%) of 232 patients. CONCLUSION: Mood disorders, the second most frequent neuropsychiatric event in patients with SLE, have a negative impact on health-related quality of life and improve over time. The lack of association with global SLE disease activity, cumulative organ damage, and lupus autoantibodies emphasizes the multifactorial etiology of mood disorders and a role for non-lupus-specific therapies.

40. Risk Factors for Cerebrovascular Events in Systemic Lupus Erythematosus: Results from an International, Inception Cohort Study

Author: Hanly, John G., Li, Qiuju, Su, Li, Urowitz, Murray, Romero-Diaz, Juanita, Gordon, Caroline, Bae, Sang-Cheol, Bernatsky, Sasha, Clarke, Ann E., Wallace, Daniel J., Merrill, Joan T., Isenberg, David A., Rahman, Anisur, Ginzler, Ellen M., Fortin, Paul R., Gladman, D., Sanchez-Guerrero, Jorge, Petri, Michelle, Bruce, Ian N., Dooley, Mary Anne, Ramsey-Goldman, Rosalind, Aranow, Cynthia, Alarcon, Graciela S., Steinsson, Kristjan, Sturfelt, Gunnar K., Nived, Ola, Manzi, Susan, Khamashta, M., Vollenhoven, Ronald F., Zoma, Asad, Guillermo Ruiz-Irastorza, Lim, S. Sam, Inanc, Murat, Kalunian, Kenneth C., Kamen, Diane L., Peschken, Christine A., Jacobsen, Soren, Askanase, Anca, Theriault, Chris, Farewell, Vernon, and Ramos-Casals, Manuel

41. Hepatitis G virus infection in primary Sjögren's syndrome: analysis in a series of 100 patients

Author: Font Franco, Josep, 1953-2006, Tàssies Penella, María Dolores, García Carrasco, Mario, Ramos Casals, Manuel, Cervera i Segura, Ricard, 1960, Reverter Calatayud, Juan Carlos, Sánchez Tapias, José M. (José María), Mazzara Aguirrezabal, Roberto, Ingelmo Morín, Miguel, and Universitat de Barcelona
Subjects: Hepatitis G, Estudi de casos, Epidemiology, Síndrome de Sjögren, virus diseases, Sjögren's syndrome, Case studies, Epidemiologia, digestive system diseases
Abstract: OBJECTIVE To determine the prevalence and clinical significance of hepatitis G virus (HGV) infection in a large cohort of patients with primary Sjögren¿s syndrome (SS). PATIENTS AND METHODS The study included 100 consecutive patients (92 female and eight male), with a mean age of 62 years (range 31¿80) that were prospectively visited in our unit. All patients fulfilled the European Community criteria for SS and underwent a complete history, physical examination, as well as biochemical and immunological evaluation for liver disease. Two hundred volunteer blood donors were also studied. The presence of HGV-RNA was investigated in the serum of all patients and donors. Aditionally, HBsAg and antibodies to hepatitis C virus were determined. RESULTS Four patients (4%) and six volunteer blood donors (3%) presented HGV-RNA sequences in serum. HGV infection was associated with biochemical signs of liver involvement in two (50%) patients. When compared with primary SS patients without HGV infection, no significant differences were found in terms of clinical or immunological features. HCV coinfection occurs in one (25%) of the four patients with HGV infection. CONCLUSION The prevalence of HGV infection in patients with primary SS is low in the geographical area of the study and HCV coinfection is very uncommon. HGV infection alone does not seen to be an important cause of chronic liver injury in the patients with primary SS in this area.

42. Systemic Manifestations of Primary Sjogren Syndrome out of the ESSDAI Classification: Prevalence and Clinical Relevance in a Large International, Multi-ethnic Cohort of Patients

Author: Retamozo, Soledad, Acar-Denizli, Nihan, Rasmussen, Astrid, Horvath, Ildiko Fanny, Baldini, Chiara, Priori, Roberta, Sandhya, Pulukool, Hernandez-Molina, Gabriela, Armagan, Berkan, Praprotnik, Sonja, Kvarnstrom, Marika, Gerli, Roberto, Agata Sebastian, Solans-Laque, Roser, Rischmueller, Maureen, Pasoto, Sandra G., Valim, Valeria, Nordmark, Gunnel, Kruize, Aike, Nakamura, Takashi, Hofauer, Benedikt, Giacomelli, Roberto, Trevisani, Virginia Fernandes Moca, Pensec, Valerie Devauchelle, Atzeni, Fabiola, Gheita, Tamer A., Consani-Fernandez, Sandra, Szanto, Antonia, Sivils, Kathy, Gattamelata, Angelina, Danda, Debashish, Kilic, Levent, Bartoloni, Elena, Bombardieri, Stefano, Sanchez-Guerrero, Jorge, Wahren-Herlenius, Marie, Mariette, Xavier, Ramos-Casals, Manuel, and Brito-Zeron, Pilar

43. Sarcoidosis as a Systemic Disease. Clinical and Epidemiological Characterization of Systemic Phenotype in 1521 Patients

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

44. 'Lupus Headache': Results From a Prospective, International, Inception Cohort Study

Author: Hanly, John G., Urowitz, Murray B., O Keeffe, Aidan, Gordon, Caroline, Bae, Sang-Cheol, Sanchez-Guerrero, Jorge, Romero-Diaz, Juanita, Clarke, Ann E., Bernatsky, Sasha, Wallace, Daniel J., Ginzler, E. M., Isenberg, David A., Rahman, Anisur, Merrill, Joan T., Petri, Michelle A., Fortin, Paul R., Gladman, D. D., Fessler, Barri J., Alarcon, Graciela S., Bruce, Ian N., Dooley, Mary Anne, Steinsson, Kristjan, Khamashta, Munther A., Ramsey-Goldman, Rosalind, Manzi, Susan, Sturfelt, Gunnar K., Nived, Ola, Zoma, Asad A., Vollenhoven, R. F., Ramos-Casals, Manuel, Aranow, Cynthia, Mackay, Meggan, Guillermo Ruiz-Irastorza, Kalunian, Kenneth C., Lim, S. Sam, Inanc, Murat, Kamen, Diane L., Peschken, Christine, Jacobsen, Soren, Theriault, Chris, Thompson, Kara, and Farewell, Vernon

45. Outcome of Lupus Nephritis and Impact on Health Related Quality of Life: Results from an International, Prospective, Inception Cohort Study

Author: Hanly, John G., O Keeffe, Aidan, Su, Li, Urowitz, Murray B., Romero-Diaz, Juanita, Gordon, Caroline, Bae, Sang-Cheol, Bernatsky, Sasha R., Clarke, Ann E., Wallace, Daniel J., Merrill, Joan T., Isenberg, David A., Rahman, Anisur, Ginzler, Ellen M., Fortin, Paul, Gladman, Dafna D., Sanchez-Guerrero, Jorge, Petri, Michelle A., Bruce, Ian, Dooley, Mary Anne, Ramsey-Goldman, Rosalind, Aranow, Cynthia, Alarcon, Graciela S., Fessler, Barri, Steinsson, Kristjan, Nived, Ola, Sturfelt, Gunnar, Manzi, Susan, Khamashta, Munther A., Vollenhoven, Ronald F., Zoma, Asad, Ramos-Casals, Manuel, Guillermo Ruiz-Irastorza, Lim, S. Sam, Stoll, Thomas, Inanc, Murat, Kalunian, Kenneth C., Kamen, Diane L., Maddison, Peter, Peschken, Christine A., Jacobsen, Soren, Askanase, Anca, Buyon, Jill P., Theriault, Chris, Thompson, Kara, and Farewell, Vernon

46. 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

47. 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

48. Cancer in an SLE Inception Cohort: Smoking May out-Perform Tumor Markers As a Risk Predictor

Author: Bernatsky, Sasha, Urowitz, Murray, Hanly, John G., Clarke, Ann E., Fritzler, Marvin J., Gordon, Caroline, Romero-Diaz, Juanita, Alarcon, Graciela S., Bae, Sang-Cheol, Petri, Michelle, Merrill, Joan T., Wallace, Daniel J., Fortin, Paul R., Gladman, Dafna D., Isenberg, David A., Rahman, Anisur, Manzi, Susan, Nived, Ola, Sturfelt, Gunnar K., Peschken, Christine A., Sanchez-Guerrero, Jorge, Guillermo Ruiz-Irastorza, Aranow, Cynthia, Vollenhoven, Ronald F., Zoma, Asad, Steinsson, Kristjan, Khamashta, Munther A., Ginzler, Ellen M., Askanase, Anca, Kalunian, Kenneth C., Dooley, Mary Anne, Lim, S. Sam, Kamen, Diane L., Jacobsen, Soren, Ramos-Casals, Manuel, Inanc, Murat, Lee, Jennifer L. F., and Ramsey-Goldman, Rosalind

49. Factors associated with damage accrual in patients with systemic lupus erythematosus: results from the Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort

Author: Bruce, Ian N, O'Keeffe, Aidan G, Farewell, Vern, Hanly, John G, Manzi, Susan, Su, Li, Gladman, Dafna D, Bae, Sang-Cheol, Sanchez-Guerrero, Jorge, Romero-Diaz, Juanita, Gordon, Caroline, Wallace, Daniel J, Clarke, Ann E, Bernatsky, Sasha, Ginzler, Ellen M, Isenberg, David A, Rahman, Anisur, Merrill, Joan T, Alarcón, Graciela S, Fessler, Barri J, Fortin, Paul R, Petri, Michelle, Steinsson, Kristjan, Dooley, Mary Anne, Khamashta, Munther A, Ramsey-Goldman, Rosalind, Zoma, Asad A, Sturfelt, Gunnar K, Nived, Ola, Aranow, Cynthia, Mackay, Meggan, Ramos-Casals, Manuel, Van Vollenhoven, Ronald F, Kalunian, Kenneth C, Ruiz-Irastorza, Guillermo, Lim, Sam, Kamen, Diane L, Peschken, Christine A, Inanc, Murat, and Urowitz, Murray B
Subjects: Inflammation, Adult, Male, Health Status, Kaplan-Meier Estimate, Middle Aged, Systemic Lupus Erythematosus, 3. Good health, Cohort Studies, Young Adult, Outcomes research, Disease Progression, Ethnicity, Quality of Life, Corticosteroids, Humans, Lupus Erythematosus, Systemic, Female, Longitudinal Studies, Prospective Studies, Proportional Hazards Models
Abstract: BACKGROUND AND AIMS: We studied damage accrual and factors determining development and progression of damage in an international cohort of systemic lupus erythematosus (SLE) patients. METHODS: The Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort recruited patients within 15 months of developing four or more 1997 American College of Rheumatology (ACR) criteria for SLE; the SLICC/ACR damage index (SDI) was measured annually. We assessed relative rates of transition using maximum likelihood estimation in a multistate model. The Kaplan-Meier method estimated the probabilities for time to first increase in SDI score and Cox regression analysis was used to assess mortality. RESULTS: We recruited 1722 patients; mean (SD) age 35.0 (13.4) years at cohort entry. Patients with damage at enrolment were more likely to have further worsening of SDI (SDI 0 vs ≥1; p

50. Is ANA-status at Disease Inception Associated with Long-term Damage Accrual and Direct and Indirect Health Care Costs in the Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort?

Author: Choi, May, Barber, Megan, Fritzler, Marvin, Hanly, John G., Urowitz, Murray, St-Pierre, Yvan, Romero-Diaz, Juanita, Gordon, Caroline, Bae, Sang-Cheol, Bernatsky, Sasha, Wallace, Daniel J., Isenberg, David A., Rahman, Anisur, Ginzler, Ellen M., Petri, Michelle, Bruce, Ian, Fortin, Paul, Gladman, Dafna, Sanchez-Guerrero, Jorge, Ramsey-Goldman, Rosalind, Khamashta, Munther A., Aranow, Cynthia, Mackay, Meggan, Alarcon, Graciela, Manzi, Susan, Nived, Ola, Jonsen, Andreas, Zoma, Asad, Vollenhoven, Ronald, Ramos-Casals, Manuel, Guillermo Ruiz-Irastorza, Lim, S. Sam, Kalunian, Kenneth C., Inanc, Murat, Kamen, Diane, Peschken, Christine, Jacobsen, Soren, Askanase, Anca, Farewell, Vernon, and Clarke, Ann E.

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