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1. POS1247 CHARACTERIZATION OF 284 PATIENTS PRESENTING WITH CENTRAL NERVOUS SYSTEM INVOLVEMENT AT DIAGNOSIS OF SJÖGREN DISEASE: RESULTS FROM THE SJÖGREN BIG DATA REGISTRY

Author

Alunno, A., primary, Brito-Zerón, P., additional, Carubbi, F., additional, Ng, W. F., additional, Flores-Chávez, A., additional, Szántó, A., additional, Li, X., additional, Rasmussen, A., additional, Dong, X., additional, Priori, R., additional, Olsson, P., additional, Baldini, C., additional, Seror, R., additional, Bootsma, H., additional, Armagan, B., additional, Kaya, B., additional, Gottenberg, J. E., additional, Praprotnik, S., additional, Suzuki, Y., additional, Quartuccio, L., additional, Leavis, H., additional, Hernandez-Molina, G., additional, Inanc, N., additional, Danda, D., additional, Bartoloni, E., additional, Rischmueller, M., additional, Sebastian, A., additional, Silvério-António, M., additional, Reis de Oliveira, F., additional, Kwok, S. K., additional, Kvarnstrom, M., additional, Solans-Laqué, R., additional, Fernandes Moça Trevisani, V., additional, Galisteo, C., additional, Sene, D., additional, Jurcut, C., additional, Fugmann, C., additional, Hofauer, B., additional, Isenberg, D., additional, Atzeni, F., additional, Shimuzu, T., additional, Valim, V., additional, Pasoto, S., additional, González García, A., additional, Retamozo, S., additional, Cipriani, P., additional, Devauchelle-Pensec, V., additional, Melchor Díaz, S., additional, Gheita, T. A., additional, Morcillo, C., additional, Fonseca-Aizpuru, E., additional, Lopez-Dupla, J. M., additional, Giacomelli, R., additional, Nakamura, H., additional, Vázquez, M., additional, Morel, J., additional, Consani-Fernández, S., additional, Akasbi Montalvo, M., additional, Diaz Cuiza, P. E., additional, De Miguel-Campo, B., additional, Lee, A. Y. S., additional, and Ramos-Casals, M., additional

Published
2024
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2. AB0788 COEXISTENCE OF SARCOIDOSIS AND SPONDYLOARTHRITIS: A RARE AND INTRIGUING ASSOCIATION

Author

Alunno, A., primary, Carubbi, F., additional, Escalante, B., additional, Policarpo-Torres, G., additional, Retamozo, S., additional, García Morillo, S., additional, Robles Marhuenda, Á., additional, Mariani, F. M., additional, Atzeni, F., additional, Ballano, J., additional, Gómez de la Torre, R., additional, Lopez-Dupla, J. M., additional, Ramos-Casals, M., additional, and Brito-Zerón, P., additional

Published
2024
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3. Exposure to air pollution as an environmental determinant of how Sjögren's disease is expressed at diagnosis

Author

Brito-Zeron, P., Flores-Chavez, A., Ng, W. -F, Horvath, I. Fanny, Rasmussen, A., Priori, R., Baldini, C., Armagan, B., Ozkiziltas, B., Praprotnik, S., Suzuki, Y., Quartuccio, L., Hernandez-Molina, G., Abacar, K., Bartoloni, E., Rischmueller, M., Reis-de Oliveira, F., Trevisani, V. Fernandes Moca, Jurcut, C., Fugmann, Cecilia, Carubbi, F., Hofauer, B., Valim, V., Pasoto, S. G., Retamozo, S., Atzeni, F., Fonseca-Aizpuru, E., Lopez-Dupla, M., Giacomelli, R., Nakamura, H., Akasbi, M., Thompson, K., Szanto, A., Farris, A. D., Villa, M., Bombardieri, S., Kilic, L., Tufan, A., Pirkmajer, K. Perdan, Fujisawa, Y., De Vita, S., Inanc, N., Ramos-Casals, M., Brito-Zeron, P., Flores-Chavez, A., Ng, W. -F, Horvath, I. Fanny, Rasmussen, A., Priori, R., Baldini, C., Armagan, B., Ozkiziltas, B., Praprotnik, S., Suzuki, Y., Quartuccio, L., Hernandez-Molina, G., Abacar, K., Bartoloni, E., Rischmueller, M., Reis-de Oliveira, F., Trevisani, V. Fernandes Moca, Jurcut, C., Fugmann, Cecilia, Carubbi, F., Hofauer, B., Valim, V., Pasoto, S. G., Retamozo, S., Atzeni, F., Fonseca-Aizpuru, E., Lopez-Dupla, M., Giacomelli, R., Nakamura, H., Akasbi, M., Thompson, K., Szanto, A., Farris, A. D., Villa, M., Bombardieri, S., Kilic, L., Tufan, A., Pirkmajer, K. Perdan, Fujisawa, Y., De Vita, S., Inanc, N., and Ramos-Casals, M.

Abstract

ObjectiveTo analyse how the potential exposure to air pollutants can influence the key components at the time of diagnosis of Sjogren's phenotype (epidemiological profile, sicca symptoms, and systemic disease). MethodsFor the present study, the following variables were selected for harmonisation and refinement: age, sex, country, fulfilment of 2002/2016 criteria items, dry eyes, dry mouth, and overall ESSDAI score. Air pollution indexes per country were defined according to the OECD (1990-2021), including emission data of nitrogen and sulphur oxides (NO/SO), particulate matter (PM2.5 and 1.0), carbon monoxide (CO) and volatile organic compounds (VOC) calculated per unit of GDP, Kg per 1000 USD.ResultsThe results of the chi-square tests of independence for each air pollutant with the frequency of dry eyes at diagnosis showed that, except for one, all variables exhibited p-values <0.0001. The most pronounced disparities emerged in the dry eye prevalence among individuals inhabiting countries with the highest NO/SO exposure, a surge of 4.61 percentage points compared to other countries, followed by CO (3.59 points), non-methane (3.32 points), PM2.5 (3.30 points), and PM1.0 (1.60 points) exposures. Concerning dry mouth, individuals residing in countries with worse NO/SO exposures exhibited a heightened frequency of dry mouth by 2.05 percentage points (p<0.0001), followed by non-methane exposure (1.21 percentage points increase, p=0.007). Individuals inhabiting countries with the worst NO/SO, CO, and PM2.5 pollution levels had a higher mean global ESSDAI score than those in lower-risk nations (all p-values <0.0001). When systemic disease was stratified according to DAS into low, moderate, and high systemic activity levels, a heightened proportion of individuals manifesting moderate/severe systemic activity was observed in countries with worse exposures to NO/SO, CO, and PM2.5 pollutant levels. ConclusionFor the first time, we suggest that pollution levels could

Published
2023
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4. Influence of exposure to climate-related hazards in the phenotypic expression of primary Sjögren's syndrome

Author

Flores-Chavez, A., Brito-Zeron, P., Ng, W. -f., Szanto, A., Rasmussen, A., Priori, R., Baldini, C., Armagan, B., Oezkiziltas, B., Praprotnik, S., Suzuki, Y., Quartuccio, L., Hernandez-Molina, G., Inanc, N., Bartoloni, E., Rischmueller, M., Oliveira, F. Reis-de, Trevisani, V. Fernandes Moca, Jurcut, C., Nordmark, Gunnel, Carubbi, F., Hofauer, B., Valim, V., Pasoto, S. G., Retamozo, S., Atzeni, F., Fonseca-Aizpuru, E., Lopez-Dupla, M., Giacomelli, R., Nakamura, H., Akasbi, M., Thompson, K., Horvath, I. Fanny, Farris, A. D., Simoncelli, E., Bombardieri, S., Kilic, L., Tufan, A., Pirkmajer, K. Perdan, Fujisawa, Y., De Vita, S., Abacar, K., Ramos-Casals, M., Flores-Chavez, A., Brito-Zeron, P., Ng, W. -f., Szanto, A., Rasmussen, A., Priori, R., Baldini, C., Armagan, B., Oezkiziltas, B., Praprotnik, S., Suzuki, Y., Quartuccio, L., Hernandez-Molina, G., Inanc, N., Bartoloni, E., Rischmueller, M., Oliveira, F. Reis-de, Trevisani, V. Fernandes Moca, Jurcut, C., Nordmark, Gunnel, Carubbi, F., Hofauer, B., Valim, V., Pasoto, S. G., Retamozo, S., Atzeni, F., Fonseca-Aizpuru, E., Lopez-Dupla, M., Giacomelli, R., Nakamura, H., Akasbi, M., Thompson, K., Horvath, I. Fanny, Farris, A. D., Simoncelli, E., Bombardieri, S., Kilic, L., Tufan, A., Pirkmajer, K. Perdan, Fujisawa, Y., De Vita, S., Abacar, K., and Ramos-Casals, M.

Abstract

Objective To analyse how the key components at the time of diagnosis of the Sjogren's phenotype (epidemiological profile, sicca symptoms, and systemic disease) can be influenced by the potential exposure to climate-related natural hazards. Methods For the present study, the following variables were selected for harmonisation and refinement: age, sex, country, fulfilment of 2002/2016 criteria items, dry eyes, dry mouth, and overall ESSDAI score. Climate-related hazards per country were defined according to the OECD and included seven climate-related hazard types: extreme temperature, extreme precipitation, drought, wildfire, wind threats, river flooding, and coastal flooding. Climatic variables were defined as dichotomous variables according to whether each country is ranked among the ten countries with the most significant exposure. Results After applying data-cleaning techniques and excluding people from countries not included in the OECD climate rankings, the database study analysed 16,042 patients from 23 countries. The disease was diagnosed between 1 and 3 years earlier in people living in countries included among the top 10 worst exposed to extreme precipitation, wildfire, wind threats, river flooding, and coastal flooding. A lower frequency of dry eyes was observed in people living in countries exposed to wind threats, river flooding, and coastal flooding, with a level of statistical association being classified as strong (p<0.0001 for the three variables). The frequency of dry mouth was significantly lower in people living in countries exposed to river flooding (p<0.0001) and coastal flooding (p<0.0001). People living in countries included in the worse climate scenarios for extreme temperature (p<0.0001) and river flooding (p<0.0001) showed a higher mean ESSDAI score in comparison with people living in no-risk countries. In contrast, those living in countries exposed to worse climate scenarios for wind threats (p<0.0001) and coastal flooding

Published
2023
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7. Childhood-onset of primary Sjögren’s syndrome: phenotypic characterization at diagnosis of 158 children

Author

Ramos-Casals, M., Acar-Denizli, N., Vissink, A., Brito-Zeron, P., Li, X., Carubbi, F., Priori, R., Toplak, N., Baldini, C., Faugier-Fuentes, E., Kruize, A. A., Mandl, T., Tomiita, M., Gandolfo, S., Hashimoto, K., Hernandez-Molina, G., Hofauer, B., Mendieta-Zeron, S., Rasmussen, A., Sandhya, P., Sene, D., Trevisani, V. F. M., Isenberg, D., Sundberg, E., Pasoto, S. G., Sebastian, A., Suzuki, Y., Retamozo, S., Xu, B., Giacomelli, R., Gattamelata, A., Bizjak, M., Bombardieri, S., Loor-Chavez, R. -E., Hinrichs, A., Olsson, P., Bootsma, H., Lieberman, S. M., Kostov, B., Horvath, I. -F., Szanto, A., Seror, R., Mariette, X., Kvarnstrom, M., Wahren-Herlenius, M., Praprotnik, S., Solans, R., Nordmark, G., Hammenfors, D., Brun, J. G., Gheita, T. A., Atzeni, F., Armagan, B., Kilic, L., Kalyoncu, U., Nakamura, T., Takagi, Y., Consani, S., Solorzano, F. O., Translational Immunology Groningen (TRIGR), Personalized Healthcare Technology (PHT), Universitat Politècnica de Catalunya. Departament d'Estadística i Investigació Operativa, and Universitat Politècnica de Catalunya. ADBD - Anàlisi de Dades Complexes per a les Decisions Empresarials

Subjects
Male, Systemic disease, Anti-nuclear antibody, Epidemiology, Autoimmune diseases, Matemàtiques i estadística::Matemàtica aplicada a les ciències [Àrees temàtiques de la UPC], Disease, Severity of Illness Index, Parotid Gland, Medicine, CLASSIFICATION CRITERIA, Pharmacology (medical), Registries, Age of Onset, biology, 92 Biology and other natural sciences::92B Mathematical biology in general [Classificació AMS], Dry eyes, Phenotype, Sjogren's syndrome, Female, epidemiology, Antibody, medicine.symptom, PAROTITIS, medicine.medical_specialty, Biomatemàtica, Adolescent, 62 Statistics::62D05 Sampling theory, sample surveys [Classificació AMS], Childhood, Paediatrics, Humans, Sjogren's Syndrome, paediatrics, AGE, Rheumatology, Peripheral nerve, Rheumatoid factor, autoimmune diseases, Sampling (Statistics), Primary Sjögren Syndrome, childhood, Biomathematics, Matemàtiques i estadística::Estadística aplicada::Estadística biosanitària [Àrees temàtiques de la UPC], business.industry, CLINICAL-FEATURES, medicine.disease, Dry mouth, Dermatology, stomatognathic diseases, biology.protein, Sjogren’s syndrome, CONSENSUS, business, Mostreig (Estadística), Parotitis
Abstract

Objectives To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS. Methods The Big Data Sjögren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 years according to the fulfilment of the 2002/2016 classification criteria. Results Among the 12 083 patients included in the Sjögren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2 years): 126 (80%) reported dry mouth, 111 (70%) dry eyes, 52 (33%) parotid enlargement, 118/122 (97%) positive minor salivary gland biopsy and 60/64 (94%) abnormal salivary US study, 140/155 (90%) positive ANA, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive RF. The systemic EULAR Sjögren’s syndrome disease activity index (ESSDAI) domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SS showed the highest mean ESSDAI score and the highest frequencies of systemic disease in 5 (constitutional, lymphadenopathy, glandular, cutaneous and haematological) of the 12 ESSDAI domains, and the lowest frequencies in 4 (articular, pulmonary, peripheral nerve and CNS) in comparison with patients with adult-onset disease. Conclusions Childhood-onset primary SS involves around 1% of patients with primary SS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role in modulating the phenotypic expression of the disease.

Published
2021

10. OP0289 SHORT- AND LONG-TERM EFFICACY OF ULTRASOUND-GUIDED NEEDLE FRAGMENTATION OR LAVAGE (BARBOTAGE) IN SYMPTOMATIC SHOULDER CALCIFIC TENDONITIS REFRACTORY TO SUBACROMIAL STEROID INJECTION. PROSPECTIVE LONGITUDINAL STUDY OF 181 PATIENTS.

Author

Casado, E., primary, Garcia-Cirera, S., additional, Retamozo, S., additional, Rusiñol, M., additional, Costa Moya, E., additional, Lluís, L., additional, Calvet, J., additional, and Gratacos-Masmitja, J., additional

Published
2022
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14. Influence of the age at diagnosis in the disease expression of primary Sjögren's syndrome: Analysis of 12,753 patients from the Sjögren Big Data Consortium

Author

Retamozo, S., Acar-Denizli, N., Horváth, I. F., Ng, W. -F, Rasmussen, A., Dong, X., Li, X., Baldini, C., Olsson, P., Priori, R., Seror, R., Gottenberg, J. -E, Kruize, A. A., Hernandez-Molina, G., Vissink, A., Sandhya, P., Armagan, B., Quartuccio, L., Sebastian, A., Praprotnik, S., Bartoloni, E., Kwok, S. -K, Kvarnstrom, M., Rischmueller, M., Soláns-Laqué, R., Sene, D., Pasoto, S. G., Suzuki, Y., Isenberg, D. A., Valim, V., Nordmark, G., Nakamura, H., Virginia Trevisani, Hofauer, B., Sisó-Almirall, A., Giacomelli, R., Devauchelle-Pensec, V., Bombardieri, M., Atzeni, F., Hammenfors, D., Maure, B., Carsons, S. E., Gheita, T., Sánchez-Berná, I., López-Dupla, M., Morel, J., Inanç, N., Fonseca-Aizpuru, E., Morcillo, C., Vollenweider, C., Melchor, S., Vázquez, M., Díaz-Cuiza, E., Consani-Fernández, S., De-Miguel-Campo, B., Szántó, A., Bombardieri, S., Gattamelata, A., Hinrichs, A., Sánchez-Guerrero, J., Danda, D., Kilic, L., Vita, S., Wiland, P., Gerli, R., Park, S. -H, Wahren-Herlenius, M., Bootsma, H., Mariette, X., Ramos-Casals, M., Brito-Zerón, P., Translational Immunology Groningen (TRIGR), and Personalized Healthcare Technology (PHT)

Subjects
immunological markers, MANIFESTATIONS, age, Sjogren's syndrome, ONSET, YOUNG, MANAGEMENT, LYMPHOMA, disease phenotype, CLASSIFICATION CRITERIA, CONSENSUS, PROJECT, SALIVARY FLOW
Abstract

Objective. To analyse how the main components of the disease phenotype (sicca symptoms, diagnostic tests, immunological markers and systemic disease) can be driven by the age at diagnosis of primary Sjogren's syndrome (pSS). Methods. By January 2021, the participant centres had included 12,753 patients from 25 countries that fulfilled the 2002/2016 classification criteria for pSS. The age at diagnosis was defined as the time when the attending physician confirmed fulfilment of the criteria. Patients were clustered according to age at diagnosis. 50 clusters with more than 100 observations (from 27 to 76 years) were used to study the influence of the age at diagnosis in the disease expression. Results. There was a consistent increase in the frequency of oral dryness according to the age at diagnosis, with a frequency of 95% in those diagnosed at the oldest ages. The smooth curves that best fitted a linear model were the frequency of dry mouth (adjusted R-2 0.87) and the frequency of abnormal oral tests (adjusted R-2 0.72). Therefore, for each 1-year increase in the age at diagnosis, the frequency of dry mouth increased by 0.13%, and the frequency of abnormal oral diagnostic tests by 0.11%. There was a consistent year-by-year decrease in the frequency of all autoantibodies and immunological markers except for cryoglobulins. According to the linear models, for each 1-year increase in the age at diagnosis, the frequency of a positive result decreased by 0.57% (for anti-Ro antibodies), 0.47% (for RF) and 0.42% (for anti-La antibodies). The ESSDAI domains which showed a more consistent decrease were glandular and lymph node involvement (for each 1-year increase in the age at diagnosis, the frequency of activity decreased by 0.18%), and constitutional, cutaneous, and haematological involvements (the frequency decreased by 0.09% for each 1-year increase). In contrast, other domains showed an ascending pattern, especially pulmonary involvement (for each 1-year increase in the age at diagnosis, the frequency of activity increased by 0.22%), and peripheral nerve involvement (the frequency increased by 0.09% for each 1-year increase). Conclusion. The influence of the age at diagnosis on the key phenotypic features of pSS is strong, and should be considered critical not only for designing a personalised diagnostic approach, but also to be carefully considered when analysing the results of diagnostic tests and immunological parameters, and when internal organ involvement is suspected at diagnosis.

Published
2021

16. Post-COVID-19 syndrome in patients with primary Sjögren's syndrome after acute SARS-CoV-2 infection

Author

Brito-Zeron, P., Acar-Denizli, N., Romão, V. C., Armagan, B., Seror, R., Carubbi, F., Melchor, S., Priori, R., Valim, V., Retamozo, S., Pasoto, S. G., Fernandes Moça Trevisani, V., Hofauer, B., Szántó, A., Inanc, N., Hernandez-Molina, G., Agata Sebastian, Bartoloni, E., Devauchelle-Pensec, V., Akasbi, M., Giardina, F., Bandeira, M., Sisó-Almirall, A., and Ramos-Casals, M.

Subjects
Sjogren's Syndrome, Post-Acute COVID-19 Syndrome, Rheumatology, SARS-CoV-2, Immunology, Immunology and Allergy, COVID-19, Humans, Fatigue
Abstract

To analyse the frequency and characteristics of post-COVID-19 syndrome in patients with primary Sjögren's syndrome (pSS) affected by acute SARS-CoV-2 infection.By the first week of April 2021, all centres included in the Big Data Sjögren Consortium were contacted asking for patients included in the Registry diagnosed with SARSCoV-2 infection according to the ECDC guidelines. According to the NICE definitions, symptoms related to COVID-19 were classified as acute COVID-19 (signs and symptoms for up to 4 weeks), ongoing symptomatic COVID-19 (presence of signs and symptoms from 4 to 12 weeks) and post-COVID-19 syndrome (signs and symptoms that continue for12 weeks not explained by an alternative diagnosis after a protocolized study).We identified 132 patients who were followed a mean follow-up of 137.8 days (ranging from 5 days to 388 days) after being diagnosed with COVID-19. In the last visit, 75 (57%) patients remained symptomatic: 68 (52%) remained symptomatic for more than 4 weeks fulfilling the NICE definition for ongoing symptomatic post-COVID-19, and 38 (29%) remained symptomatic for more than 12 weeks fulfilling the definition of post-COVID-19 syndrome. More than 40% of pSS patients reported the persistence of four symptoms or more, including anxiety/depression (59%), arthralgias (56%), sleep disorder (44%), fatigue (40%), anosmia (34%) and myalgias (32%). Age-sex adjusted multivariate analysis identified raised LDH levels (OR 10.36), raised CRP levels (OR 7.33), use of hydroxychloroquine (OR 3.51) and antiviral agents (OR 3.38), hospital admission (OR 8.29), mean length of hospital admission (OR 1.1) and requirement of supplemental oxygen (OR 6.94) as factors associated with a higher risk of developing post-COVID-19 syndrome. A sensitivity analysis including hospital admission in the adjusted model confirmed raised CRP levels (OR 8.6, 95% CI 1.33-104.44) and use of hydroxychloroquine (OR 2.52, 95% CI 1.00-6.47) as the key independent factors associated with an enhanced risk of developing post-COVID-19 syndrome.This is the first study that analyses the frequency and characteristics of post-COVID-19 syndrome in patients affected by a systemic autoimmune disease. We found that 57% of patients with pSS affected by COVID-19 remain symptomatic after a mean follow-up of 5 months. The risk of developing post-COVID-19 syndrome in patients who required hospitalisation was 8-times higher than in non-hospitalised patients, with baseline raised CRP levels and the use of hydroxychloroquine being independent risk factors for post-COVID-19.

Published
2021

18. Therapeutic Recommendations for the Management of Older Adult Patients with Sjögren’s Syndrome

Author

Retamozo, S. Baldini, C. Bootsma, H. De Vita, S. Dörner, T. Fisher, B.A. Gottenberg, J.-E. Hernández-Molina, G. Kocher, A. Kostov, B. Kruize, A.A. Mandl, T. Ng, W.-F. Seror, R. Shoenfeld, Y. Sisó-Almirall, A. Tzioufas, A.G. Vissink, A. Vitali, C. Bowman, S.J. Mariette, X. Ramos-Casals, M. Brito-Zerón, P.

Abstract

Primary Sjögren’s syndrome (SjS) is a systemic autoimmune disease most commonly diagnosed in middle-aged women. Although the disease can occur at all ages, it is diagnosed between 30 and 60 years of age in two-thirds of patients. In more than 20% of cases, the people are older than 65 years. In this review, we focus on the therapeutic management of primary SjS in older patients, following the recently published 2020 European League Against Rheumatism (EULAR) recommendations for the management of the disease with topical and systemic therapies. These recommendations are applicable to all patients with primary SjS regardless of age at diagnosis, although the therapeutic management in older patients requires additional considerations. Older patients are more likely to have pulmonary, liver, kidney, or heart-related comorbidities (even cognitive disturbances); caution is required when most drugs are used, including muscarinic agents, systemic corticosteroids and synthetic immunosuppressants. It is also important to monitor the use of eye drops containing steroids due to the increased risk of developing cataracts, a frequent ocular complication in the older population. In contrast, the majority of drugs that can be used topically (pilocarpine rinses, eye drops containing topical non-steroidal anti-inflammatory drugs (NSAIDs) or cyclosporine A, topical dermal formulations of NSAIDs) have shown an acceptable safety profile in older patients, as well as rituximab. A rigorous evaluation of the medical history of older patients is essential when drugs included in the EULAR guidelines are prescribed, with special attention to factors frequently related to ageing, such as polypharmacy, the existence of organ-specific comorbidities, or the enhanced susceptibility to infections. © 2021, The Author(s), under exclusive licence to Springer Nature Switzerland AG part of Springer Nature.

Published
2021

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

Author

Medicina i Cirurgia, Universitat Rovira i Virgili, Brito-Zerón P; Brito-Zerón P; Melchor S; Seror R; Seror R; Priori R; Priori R; Solans R; Solans R; Kostov B; Kostov B; Baldini C; Carubbi F; Carubbi F; Callejas JL; Guisado-Vasco P; Hernández-Molina G; Hernández-Molina G; Pasoto SG; Pasoto SG; Valim V; Valim V; Sisó-Almirall A; Sisó-Almirall A; Mariette X; Carreira P; Ramos-Casals M; Brito-Zerón P; Morcillo C; Flores-Chávez A; Acar-Denizli N; Horvath IF; Szanto A; Tarr T; Mandl T; Olsson P; Li X; Xu B; Baldini C; Bombardieri S; Gottenberg JE; Gandolfo S; De Vita S; Giardina F; Sánchez-Guerrero J; Kruize AA; Hinrichs A; Isenberg D; Rischmueller M; Downie-Doyle S; Kwok SK; Park SH; 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; Consani-Fernández S; Callejas JL; López-Dupla M; Pérez-Alvarez R; Akasbi M; Sánchez I, Medicina i Cirurgia, Universitat Rovira i Virgili, and Brito-Zerón P; Brito-Zerón P; Melchor S; Seror R; Seror R; Priori R; Priori R; Solans R; Solans R; Kostov B; Kostov B; Baldini C; Carubbi F; Carubbi F; Callejas JL; Guisado-Vasco P; Hernández-Molina G; Hernández-Molina G; Pasoto SG; Pasoto SG; Valim V; Valim V; Sisó-Almirall A; Sisó-Almirall A; Mariette X; Carreira P; Ramos-Casals M; Brito-Zerón P; Morcillo C; Flores-Chávez A; Acar-Denizli N; Horvath IF; Szanto A; Tarr T; Mandl T; Olsson P; Li X; Xu B; Baldini C; Bombardieri S; Gottenberg JE; Gandolfo S; De Vita S; Giardina F; Sánchez-Guerrero J; Kruize AA; Hinrichs A; Isenberg D; Rischmueller M; Downie-Doyle S; Kwok SK; Park SH; 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; Consani-Fernández S; Callejas JL; López-Dupla M; Pérez-Alvarez R; Akasbi M; Sánchez I

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 co

Published
2021

20. Influence of the age at diagnosis in the disease expression of primary Sjögren's syndrome. Analysis of 12,753 patients from the Sjögren Big Data Consortium

Author

Universitat Rovira i Virgili, Retamozo S; Acar-Denizli N; Horváth IF; Ng WF; Rasmussen A; Dong X; Li X; Baldini C; Olsson P; Priori R; Seror R; Gottenberg JE; Kruize AA; Hernandez-Molina G; Vissink A; Sandhya P; Armagan B; Quartuccio L; Sebastian A; Praprotnik S; Bartoloni E; Kwok SK; Kvarnstrom M; Rischmueller M; Soláns-Laqué R; Sene D; Pasoto SG; Suzuki Y; Isenberg DA; Valim V; Nordmark G; Nakamura H; Trevisani VFM; Hofauer B; Sisó-Almirall A; Giacomelli R; Devauchelle-Pensec V; Bombardieri M; Atzeni F; Hammenfors D; Maure B; Carsons SE; Gheita T; Sánchez-Berná I; López-Dupla M; Morel J; Inanç N; Fonseca-Aizpuru E; Morcillo C; Vollenweider C; Melchor S; Vázquez M; Díaz-Cuiza E; Consani-Fernández S; De-Miguel-Campo B; Szántó A; Bombardieri S; Gattamelata A; Hinrichs A; Sánchez-Guerrero J; Danda D; Kilic L; De Vita S; Wiland P; Gerli R; Park SH; Wahren-Herlenius M; Bootsma H; Mariette X; Ramos-Casals M; Brito-Zerón P, Universitat Rovira i Virgili, and Retamozo S; Acar-Denizli N; Horváth IF; Ng WF; Rasmussen A; Dong X; Li X; Baldini C; Olsson P; Priori R; Seror R; Gottenberg JE; Kruize AA; Hernandez-Molina G; Vissink A; Sandhya P; Armagan B; Quartuccio L; Sebastian A; Praprotnik S; Bartoloni E; Kwok SK; Kvarnstrom M; Rischmueller M; Soláns-Laqué R; Sene D; Pasoto SG; Suzuki Y; Isenberg DA; Valim V; Nordmark G; Nakamura H; Trevisani VFM; Hofauer B; Sisó-Almirall A; Giacomelli R; Devauchelle-Pensec V; Bombardieri M; Atzeni F; Hammenfors D; Maure B; Carsons SE; Gheita T; Sánchez-Berná I; López-Dupla M; Morel J; Inanç N; Fonseca-Aizpuru E; Morcillo C; Vollenweider C; Melchor S; Vázquez M; Díaz-Cuiza E; Consani-Fernández S; De-Miguel-Campo B; Szántó A; Bombardieri S; Gattamelata A; Hinrichs A; Sánchez-Guerrero J; Danda D; Kilic L; De Vita S; Wiland P; Gerli R; Park SH; Wahren-Herlenius M; Bootsma H; Mariette X; Ramos-Casals M; Brito-Zerón P

Abstract

Objective. To analyse how the main components of the disease phenotype (sicca symptoms, diagnostic tests, immunological markers and systemic disease) can be driven by the age at diagnosis of primary Sjögren's syndrome (pSS). Methods. By January 2021, the participant centres had included 12,753 patients from 25 countries that fulfilled the 2002/2016 classification criteria for pSS. The age at diagnosis was defined as the time when the attending physician confirmed fulfilment of the criteria. Patients were clustered according to age at diagnosis. 50 clusters with more than 100 observations (from 27 to 76 years) were used to study the influence of the age at diagnosis in the disease expression. Results. There was a consistent increase in the frequency of oral dryness according to the age at diagnosis, with a frequency of <90% in patients diagnosed at the youngest ages and >95% in those diagnosed at the oldest ages. The smooth curves that best fitted a linear model were the frequency of dry mouth (adjusted R2 0.87) and the frequency of abnormal oral tests (adjusted R2 0.72). Therefore, for each 1-year increase in the age at diagnosis, the frequency of dry mouth increased by 0.13%, and the frequency of abnormal oral diagnostic tests by 0.11%. There was a consistent year-by-year decrease in the frequency of all autoantibodies and immunological markers except for cryoglobulins. According to the linear models, for each 1-year increase in the age at diagnosis, the frequency of a positive result decreased by 0.57% (for anti-Ro antibodies), 0.47% (for RF) and 0.42% (for anti-La antibodies). The ESSDAI domains which showed a more consistent decrease were glandular and lymph node involvement (for each 1-year increase in the age at diagnosis, the frequency of activity decreased by 0.

Published
2021

21. 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

22. Systemic phenotype related to primary Sjögren's syndrome in 279 patients carrying isolated anti-La/SSB antibodies

Author

Acar-Denizli, N., Horvath, I. -F, Mandl, T., Priori, R., Vissink, A., Hernandez-Molina, G., Armagan, B., Praprotnik, S., Sebastian, A., Bartoloni, E., Rischmueller, M., Pasoto, S. G., Nordmark, Gunnel, Nakamura, H., Fernandes Moca Trevisani, V., Retamozo, S., Carsons, S. E., Maure-Noia, B., Sanchez-Berna, I., Lopez-Dupla, M., Fonseca-Aizpuru, E., Melchor Diaz, S., Vazquez, M., Diaz Cuiza, P. E., de Miguel Campo, B., Ng, W. -F, Rasmussen, A., Dong, X., Li, X., Baldini, C., Seror, R., Gottenberg, J. -E, Kruize, A. A., Sandhya, P., Gandolfo, S., Kwok, S. -K, Kvarnstrom, M., Solans, R., Sene, D., Suzuki, Y., Isenberg, D. A., Valim, V., Hofauer, B., Giacomelli, R., Devauchelle-Pensec, V., Atzeni, F., Gheita, T. A., Morel, J., Izzo, R., Kalyoncu, U., Szanto, A., Olsson, P., Bootsma, H., Ramos-Casals, M., Kostov, B., Brito-Zeron, P., Acar-Denizli, N., Horvath, I. -F, Mandl, T., Priori, R., Vissink, A., Hernandez-Molina, G., Armagan, B., Praprotnik, S., Sebastian, A., Bartoloni, E., Rischmueller, M., Pasoto, S. G., Nordmark, Gunnel, Nakamura, H., Fernandes Moca Trevisani, V., Retamozo, S., Carsons, S. E., Maure-Noia, B., Sanchez-Berna, I., Lopez-Dupla, M., Fonseca-Aizpuru, E., Melchor Diaz, S., Vazquez, M., Diaz Cuiza, P. E., de Miguel Campo, B., Ng, W. -F, Rasmussen, A., Dong, X., Li, X., Baldini, C., Seror, R., Gottenberg, J. -E, Kruize, A. A., Sandhya, P., Gandolfo, S., Kwok, S. -K, Kvarnstrom, M., Solans, R., Sene, D., Suzuki, Y., Isenberg, D. A., Valim, V., Hofauer, B., Giacomelli, R., Devauchelle-Pensec, V., Atzeni, F., Gheita, T. A., Morel, J., Izzo, R., Kalyoncu, U., Szanto, A., Olsson, P., Bootsma, H., Ramos-Casals, M., Kostov, B., and Brito-Zeron, P.

Abstract

Objective: To evaluate the systemic phenotype associated with the presence of isolated anti-La/SSB antibodies in a large international registry of patients with primary Sjogren's syndrome (pSS) fulfilling the 2002 classification criteria. Methods: The Big Data Sjogren Project Consortium is an international, multicentre registry created in 2014. Baseline clinical information from leading centres on clinical research in SS of the 5 continents was collected. Combination patterns of anti-Ro/SSA-La/SSB antibodies at the time of diagnosis defined the following four immunological phenotypes: double positive (combined Ro/SSA and La/SSB,) isolated anti-Ro/SSA, isolated anti-La/SSB, and immunonegative. Results: The cohort included 12,084 patients (11,293 females, mean 52.4 years) with recorded ESSDAI scores available. Among them, 279 (2.3%) had isolated anti-La/SSB antibodies. The mean total ESSDAI score at diagnosis of patients with pSS carrying isolated anti-La/SSB was 6.0, and 80.4% of patients had systemic activity (global ESSDAI score >= 1) at diagnosis. The domains with the highest frequency of active patients were the biological (42.8%), glandular (36.8%) and articular (31.2%) domains. Patients with isolated anti-La/ SSB showed a higher frequency of active patients in all ESSDAI domains but two (articular and peripheral nerve) in comparison with immune-negative patients, and even a higher absolute frequency in six clinical ESSDAI domains in comparison with patients with isolated anti-Ro/SSA. In addition, patients with isolated anti-La/SSB showed a higher frequency of active patients in two ESSDAI domains (pulmonary and glandular) with respect to the most active immunological subset (double-positive antibodies). Meanwhile, systemic activity detected in patients with isolated anti-La/SSB was overwhelmingly low. Even in ESSDAI domains where patients with isolated anti-La/SSB had the highest frequencies of systemic activity (lymphadenopathy and muscular), the percentage

Published
2020

23. EULAR recommendations for the management of Sjogren's syndrome with topical and systemic therapies

Author

Medicina i Cirurgia, Ramos-Casals, M Brito-Zeron, P; Bombardieri, S; Bootsma, H; De Vita, S; Dörner, T; Fisher, BA; Gottenberg, JE; Hernandez-Molina, G; Kocher, A; Kostov, B; Kruize, AA; Mandl, T; Ng, WF; Retamozo, S; Seror, R; Shoenfeld, Y; Siso-Almirall, A; Tzioufas, AG; Vitali, C; Bowman, S; Mariette, X, Medicina i Cirurgia, and Ramos-Casals, M Brito-Zeron, P; Bombardieri, S; Bootsma, H; De Vita, S; Dörner, T; Fisher, BA; Gottenberg, JE; Hernandez-Molina, G; Kocher, A; Kostov, B; Kruize, AA; Mandl, T; Ng, WF; Retamozo, S; Seror, R; Shoenfeld, Y; Siso-Almirall, A; Tzioufas, AG; Vitali, C; Bowman, S; Mariette, X

Abstract

The therapeutic management of Sjögren syndrome (SjS) has not changed substantially in recent decades: treatment decisions remain challenging in clinical practice, without a specific therapeutic target beyond the relief of symptoms as the most important goal. In view of this scenario, the European League Against Rheumatism (EULAR) promoted and supported an international collaborative study (EULAR SS Task Force) aimed at developing the first EULAR evidence and consensus-based recommendations for the management of patients with SjS with topical and systemic medications. The aim was to develop a rational therapeutic approach to SjS patients useful for healthcare professionals, physicians undergoing specialist training, medical students, the pharmaceutical industry and drug regulatory organisations following the 2014 EULAR standardised operating procedures. The Task Force (TF) included specialists in rheumatology, internal medicine, oral health, ophthalmology, gynaecology, dermatology and epidemiology, statisticians, general practitioners, nurses and patient representatives from 30 countries of the 5 continents. Evidence was collected from studies including primary SjS patients fulfilling the 2002/2016 criteria; when no evidence was available, evidence from studies including associated SjS or patients fulfilling previous sets of criteria was considered and extrapolated. The TF endorsed the presentation of general principles for the management of patients with SjS as three overarching, general consensus-based recommendations and 12 specific recommendations that form a logical sequence, starting with the management of the central triplet of symptoms (dryness, fatigue and pain) followed by the management of systemic disease. The recommendations address the use of topical oral (

Published
2020

25. EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies

Author

Ramos-Casals, M., Brito-Zeron, P., Bombardieri, S., Bootsma, H., De Vita, S., Dorner, T., Fisher, B. A., Gottenberg, J. -E., Hernandez-Molina, G., Kocher, A., Kostov, B., Kruize, A. A., Mandl, T., W. -F., Ng, Retamozo, S., Seror, R., Shoenfeld, Y., Siso-Almirall, A., Tzioufas, A. G., Vitali, C., Bowman, S., EULAR-Sjögren Syndrome Task Force Group: Sebastian A, Mariette X., Saraux, A, Vissink, A, Rasmussen, A, Hofauer, B, Armagan, B, Feijoo-Massó, C, Shiboski, Ch, Baldini, C, Vollenweider, C, Sene, D, Hammenfors, D, Isenberg, D, Danda, D, Bartoloni, E, Hospital Clinic i Provincial de Barcelona (SCReN), CELLEX-IDIBAPS Department of Autoimmune Diseases, Barcelona, H. CIMA-Sanitas, Barcelona, University of Pisa - Università di Pisa, University of Groningen [Groningen], Ospedale 'Santa Maria della Misericordia' = University Hospital 'Santa Maria della Misericordia', Charité - UniversitätsMedizin = Charité - University Hospital [Berlin], University of Birmingham [Birmingham], Service de rhumatologie [Strasbourg], CHU Strasbourg-Hôpital de Hautepierre [Strasbourg], Immunopathologie et chimie thérapeutique (ICT), Institut de biologie moléculaire et cellulaire (IBMC), Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS)-Centre National de la Recherche Scientifique (CNRS), Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán - National Institute of Medical Science and Nutrition Salvador Zubiran [Mexico], University Hospital Basel [Basel], Bern University Hospital [Berne] (Inselspital), Transverse group for research in primary care [Barcelona], Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Universitat Politècnica de Catalunya [Barcelona] (UPC), University Medical Center [Utrecht], Skane University Hospital [Malmo], Lund University [Lund], Newcastle University [Newcastle], Newcastle Upon Tyne Hospitals NHS Foundation Trust, Universidad de Córdoba = University of Córdoba [Córdoba], Universidad Nacional de Córdoba [Argentina], Immunologie des Maladies Virales et Autoimmunes (IMVA - U1184), Université Paris-Sud - Paris 11 (UP11)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de la Santé et de la Recherche Médicale (INSERM), AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), The Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Saint Petersburg University (SPBU), CAP Les Corts (CAPSBE), National and Kapodistrian University of Athens (NKUA), Santo Stefano Riabilitazione, University Hospitals Birmingham [Birmingham, Royaume-Uni], Centre National de la Recherche Scientifique (CNRS)-Institut de biologie moléculaire et cellulaire (IBMC), Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS), Rheumatology Unit, Cordoba (Institute University of Biomedical Sciences University of Cordoba (IUCBC), Universitat Politècnica de Catalunya. Departament d'Estadística i Investigació Operativa, and Salvy-Córdoba, Nathalie

Subjects
MESH: Antirheumatic Agents, Autoimmune diseases, MESH: Hydroxychloroquine, Matemàtiques i estadística::Matemàtica aplicada a les ciències [Àrees temàtiques de la UPC], Azathioprine, Administration, Ophthalmic, MESH: Cyclosporine, 0302 clinical medicine, Adrenal Cortex Hormones, MESH: Muscarinic Agonists, Epidemiology, Immunology and Allergy, 030212 general & internal medicine, [SDV.MHEP.RSOA] Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, treatment, Anti-Inflammatory Agents, Non-Steroidal, 92 Biology and other natural sciences::92B Mathematical biology in general [Classificació AMS], [SDV.SP]Life Sciences [q-bio]/Pharmaceutical sciences, MESH: Anti-Inflammatory Agents, Non-Steroidal, MESH: Administration, Ophthalmic, 3. Good health, [SDV.SP] Life Sciences [q-bio]/Pharmaceutical sciences, Sjogren's Syndrome, [SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, Antirheumatic Agents, Cyclosporine, Rituximab, MESH: Immunosuppressive Agents, MESH: Lubricant Eye Drops, sjøgren's syndrome, MESH: Saliva, Artificial, autoimmune diseases, Immunosuppressive Agents, medicine.drug, Hydroxychloroquine, medicine.medical_specialty, Biomatemàtica, Immunology, Muscarinic Agonists, General Biochemistry, Genetics and Molecular Biology, Lubricant Eye Drops, MESH: Adrenal Cortex Hormones, 03 medical and health sciences, Therapeutic approach, Rheumatology, medicine, Humans, Intensive care medicine, Glucocorticoids, Sjøgren's syndrome, Biomathematics, 030203 arthritis & rheumatology, MESH: Humans, business.industry, Abatacept, Saliva, Artificial, Evidence-based medicine, medicine.disease, Belimumab, Treatment, stomatognathic diseases, MESH: Sjogren's Syndrome, MESH: Glucocorticoids, business, Rheumatism
Abstract

International audience; The therapeutic management of Sjögren syndrome (SjS) has not changed substantially in recent decades: treatment decisions remain challenging in clinical practice, without a specific therapeutic target beyond the relief of symptoms as the most important goal. In view of this scenario, the European League Against Rheumatism (EULAR) promoted and supported an international collaborative study (EULAR SS Task Force) aimed at developing the first EULAR evidence and consensus-based recommendations for the management of patients with SjS with topical and systemic medications. The aim was to develop a rational therapeutic approach to SjS patients useful for healthcare professionals, physicians undergoing specialist training, medical students, the pharmaceutical industry and drug regulatory organisations following the 2014 EULAR standardised operating procedures. The Task Force (TF) included specialists in rheumatology, internal medicine, oral health, ophthalmology, gynaecology, dermatology and epidemiology, statisticians, general practitioners, nurses and patient representatives from 30 countries of the 5 continents. Evidence was collected from studies including primary SjS patients fulfilling the 2002/2016 criteria; when no evidence was available, evidence from studies including associated SjS or patients fulfilling previous sets of criteria was considered and extrapolated. The TF endorsed the presentation of general principles for the management of patients with SjS as three overarching, general consensus-based recommendations and 12 specific recommendations that form a logical sequence, starting with the management of the central triplet of symptoms (dryness, fatigue and pain) followed by the management of systemic disease. The recommendations address the use of topical oral (saliva substitutes) and ocular (artificial tear drops, topical non-steroidal anti-inflammatory drugs, topical corticosteroids, topical CyA, serum tear drops) therapies, oral muscarinic agonists (pilocarpine, cevimeline), hydroxychloroquine, oral glucocorticoids, synthetic immunosuppressive agents (cyclophosphamide, azathioprine, methotrexate, leflunomide and mycophenolate), and biological therapies (rituximab, abatacept and belimumab). For each recommendation, levels of evidence (mostly modest) and TF agreement (mostly very high) are provided. The 2019 EULAR recommendations are based on the evidence collected in the last 16 years in the management of primary 2002 SjS patients and on discussions between a large and broadly international TF. The recommendations synthesise current thinking on SjS treatment in a set of overarching principles and recommendations. We hope that the current recommendations will be broadly applied in clinical practice and/or serve as a template for national societies to develop local recommendations.

Published
2019

26. How the age at diagnosis modifies the phenotype of primary Sjögren syndrome: analysis in 11.420 patients (Big data Sjögren project

Author

Retamozo, S, Acar-Denizli, N, Ng, WF, Horvath, I., Rasmussen, A., Seror, R., Li, X, Baldini, C, Gottenberg, J., Sandhya, P, Quartuccio L, R, Priori, Roberta, Hernandez-Molina, G, Armagan, B, Kruize, A, Kwok, S, Kvarnstrom, M, Praprotnik, S, Sene, D, Bartoloni Bocci, E, Solans-Laqué, R, Rischmueller, M, Mandl, T, Suzuki, Y, Isenberg, D, Valim, V, Sebastián, A., Nordmark, G, Bootsma, H, Nakamura, H, Giacomelli, R, Devauchelle-Pensec, V., Hofauer, B, Bombardieri, M, Fernandes Moça Trevisani, V, Hammenfors, D, Pasoto, S, Gheita, T, Atzeni, F, Morel, J., Vollenveider, C, Consani-Fernández, S, Mariette, X., Ramos-Casals, M, Brito-Zerón, P, Michel, Geneviève, INICSA, UNC, CONICED, Cordoba, Mimar Sinan Üniversitesi, Newcastle University (UNITED KINGDOM), University of Debrecen, OMRF Oklahoma, University of Oklahoma (OU), Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Anhui Provincial Hosp, University of Pisa - Università di Pisa, Institut de biologie moléculaire et cellulaire (IBMC), Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS), Department of Clinical Immunology and Rheumatology, Vellore (Christian Medical College & Hospital), Department of Medical Area, University Hospital Santa Maria della Misericordia, Udine, Ospedale 'Santa Maria della Misericordia' = University Hospital 'Santa Maria della Misericordia', Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome], INCMNSS, Mexico, Hacettepe University = Hacettepe Üniversitesi, University Medical Center [Utrecht], Catholic University of Korea, Karolinska Institutet [Stockholm], University Medical Centre Ljubljana [Ljubljana, Slovenia] (UMCL), Université Paris Diderot - Paris 7 (UPD7), Università degli Studi di Perugia (UNIPG), Vall d'Hebron University Hospital [Barcelona], The University of Western Australia (UWA), Lund University, Malmö University Hospital, Kanazawa University (KU), University College of London [London] (UCL), Federal University of Espírito Santo, Wroclaw Medical University, Uppsala Universitet [Uppsala], University Medical Center Groningen [Groningen] (UMCG), Nagasaki University, 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), Technische Universität Munchen - Université Technique de Munich [Munich, Allemagne] (TUM), 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, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), German Hospital, Buenos Aires, Facultad de Ciencias (UDELAR), Hosp Maciel montevideo, Service de Rhumatologie [CHU Bicêtre], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Clinical Institute of Neuroscience, Hospital Clinic, University of Barcelona, IDIBAPS, CIBERSAM, Barcelona, Catalonia, Spain., and H. CIMA-Sanitas, Barcelona

Subjects
[SDV.IMM] Life Sciences [q-bio]/Immunology, [SDV.IMM]Life Sciences [q-bio]/Immunology, ComputingMilieux_MISCELLANEOUS
Abstract

International audience

Published
2019

27. LYMPHOMA ARISING AT THE TIME OF DIAGNOSIS OF PRIMARY SJOGREN SYNDROME: A HIGHLY-ACTIVE SYSTEMIC SUBSET OF THE DISEASE

Author

Retamozo, S, Acar-Denizli, N, Ng, Wf, Szanto, A, Rasmussen, A., Seror, R., Li, X, Baldini, C, Gottenberg, J., Sandhya, P, Quartuccio, L, Priori, R, Hernandez-Molina, G, Armagan, B, Kruize, A, Kwok, S, Kvarnstrom, M, Praprotnik, S, Sene, D, Solans-Laque, R, Rischmueller, M, Mandl, T, Suzuki, Y, Isenberg, D, Valim, V, Sebastián, A., Nordmark, G, Bootsma, H, Nakamura, H, Giacomelli, R, Devauchelle-Pensec, V., Hofauer, B, Bombardieri, M, Fernandes Mocatrevisani, V, Hammenfors, D, Pasoto, S, Gheita, T, Atzeni, F, Morel, J., Vollenveider, C, Consani-Fernandez, S, Mariette, X., Ramos-Casals, M, Brito-Zeron, P, Bartoloni Bocci, E, INICSA, UNC, CONICED, Cordoba, Mimar Sinan Üniversitesi, Newcastle University (UNITED KINGDOM), University of Debrecen, OMRF Oklahoma, University of Oklahoma (OU), INSERM UMR972, SFR André Lwoff, Université Paris Sud, APHP Paul Brousse, Anhui Provincial Hosp, University of Pisa - Università di Pisa, Institut de biologie moléculaire et cellulaire (IBMC), Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS), Christian Medical College & Hospital, Azienda Ospedaliera Universitaria Santa Maria della Misericordia, Udine, Italy., Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome], INCMNSS, Mexico, Hacettepe University = Hacettepe Üniversitesi, University Medical Center [Utrecht], Catholic University of Korea, Karolinska Institutet [Stockholm], University Medical Centre Ljubljana [Ljubljana, Slovenia] (UMCL), Université Paris Diderot - Paris 7 (UPD7), Vall d'Hebron University Hospital [Barcelona], The University of Western Australia (UWA), Kanazawa University (KU), University College of London [London] (UCL), Federal University of Espírito Santo, Wroclaw Medical University, Uppsala Universitet [Uppsala], University Medical Center Groningen [Groningen] (UMCG), Nagasaki University, University of L'Aquila [Italy] (UNIVAQ), 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), CHRU Brest - Service de Rhumatologie (CHU - BREST - Rhumato), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Technische Universität Munchen - Université Technique de Munich [Munich, Allemagne] (TUM), Queen Mary University of London (QMUL), Federal University of Sao Paulo (Unifesp), Haukeland University Hospital, University of Bergen (UiB), Hospital das Clinicas, Cairo University, Univ Messina, Dipartimento Sci Matemat & Informat Sci Fis & Sci, Viale Ferdinando Stagno dAlcontres 31, I-98166 Messina S Agata, Italy, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), German Hospital, Buenos Aires, Hospital Maciel, Immunologie antivirale systémique et cérébrale, Université Paris-Sud - Paris 11 (UP11)-IFR93-Institut National de la Santé et de la Recherche Médicale (INSERM), Clinical Institute of Neuroscience, Hospital Clinic, University of Barcelona, IDIBAPS, CIBERSAM, Barcelona, Catalonia, Spain., H. CIMA-Sanitas, Barcelona, Università degli Studi di Perugia (UNIPG), 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), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Brestois Santé Agro Matière (IBSAM), and Michel, Geneviève

Subjects
[SDV.IMM] Life Sciences [q-bio]/Immunology, [SDV.IMM]Life Sciences [q-bio]/Immunology, ComputingMilieux_MISCELLANEOUS
Abstract

International audience

Published
2019

28. Efficacy and safety of topical and systemic medications: A systematic literature review informing the EULAR recommendations for the management of Sjögren's syndrome

Author

Brito-Zerón, P. Retamozo, S. Kostov, B. Baldini, C. Bootsma, H. De Vita, S. Dörner, T. Gottenberg, J.-E. Kruize, A.A. Mandl, T. Ng, W.-F. Seror, R. Tzioufas, A.G. Vitali, C. Bowman, S. Mariette, X. Ramos-Casals, M.

Abstract

Objective To evaluate current evidence on the efficacy and safety of topical and systemic medications in patients with primary Sjögren syndrome (SjS) to inform European League Against Rheumatism treatment recommendations. Methods The MEDLINE, EMBASE and Cochrane databases were searched for case-control/prospective cohort studies, randomised controlled trials (RCTs) and systematic reviews. Results Current evidence in primary SjS patients fulfilling the 2002 criteria is based on the data from 9 RCTs, 18 prospective cohort studies and 5 case-control studies. Two Cochrane systematic literature reviews (SLRs) have reported that topical treatments for dry mouth and dry eye are safe and effective. Ocular cyclosporine A was safe and effective in two RCTs including 1039 patients with dry eye syndrome. Two Cochrane SLRs on serum tear drops and plugs showed inconsistency in possible benefits, both for symptoms and objective measures. Five RCTs reported significant improvements in oral dryness and salivary flow rates for pilocarpine and cevimeline. An RCT showed no significant placebo-differences for hydroxychloroquine 400 mg/day for the primary outcome (visual analogue scale (VAS) composite of dryness, fatigue and pain). We identified seven RCTs carried out in primary SjS patients. RCTs using infliximab, anakinra and baminercept found no placebo-differences for the primary outcomes. The two largest RCTs randomised 255 patients to receive rituximab or placebo and reported no significant results in the primary outcome (VAS composite), while prospective studies suggested efficacy in systemic disease. Conclusion The current evidence supporting the use of the main topical therapeutic options of primary SjS is solid, while limited data from RCTs are available to guide systemic therapies. © 2019 Author(s).

Published
2019

29. Systemic manifestations of primary Sjogren's syndrome out of the ESSDAI classification : prevalence and clinical relevance in a large international, multi-ethnic cohort of patients

Author

Retamozo, S., Acar-Denizli, N., Rasmussen, A., Horvath, I. F., Baldini, C., Priori, R., Sandhya, P., Hernandez-Molina, G., Armagan, B., Praprotnik, S., Kvarnstrom, M., Gerli, R., Sebastian, A., Solans, R., Rischmueller, M., Pasoto, S. G., Valim, V., Nordmark, Gunnel, Kruize, A. A., Nakamura, H., Hofauer, B., Giacomelli, R., Fernandes Moca Trevisani, V., Devauchelle-Pensec, V., Atzeni, F., Gheita, T. A., Consani-Fernandez, S., Szanto, A., Sivils, K., Gattamelata, A., Danda, D., Kilic, L., Bartoloni, E., Bombardieri, S., Sanchez-Guerrero, J., Wahren-Herlenius, M., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Retamozo, S., Acar-Denizli, N., Rasmussen, A., Horvath, I. F., Baldini, C., Priori, R., Sandhya, P., Hernandez-Molina, G., Armagan, B., Praprotnik, S., Kvarnstrom, M., Gerli, R., Sebastian, A., Solans, R., Rischmueller, M., Pasoto, S. G., Valim, V., Nordmark, Gunnel, Kruize, A. A., Nakamura, H., Hofauer, B., Giacomelli, R., Fernandes Moca Trevisani, V., Devauchelle-Pensec, V., Atzeni, F., Gheita, T. A., Consani-Fernandez, S., Szanto, A., Sivils, K., Gattamelata, A., Danda, D., Kilic, L., Bartoloni, E., Bombardieri, S., Sanchez-Guerrero, J., Wahren-Herlenius, M., Mariette, X., Ramos-Casals, M., and Brito-Zeron, P.

Abstract

Objectives: To analyse the frequency and characterise the systemic presentation of primary Sjogren's syndrome (SS) out of the ESSDAI classification in a large international, multi-ethnic cohort of patients. Methods: The Big Data Sjogren Project Consortium is an international, multicentre registry based on world-wide data-sharing and cooperative merging of pre-existing clinical SS databases from leading centres in clinical research in SS from the five continents. A list of 26 organ-by-organ systemic features not currently included in the ESSDAI classification was defined according to previous studies; these features were retrospectively recorded. Results: Information about non-ESSDAI features was available in 6331 patients [5,917 female, mean age at diagnosis 52 years, mainly White (86.3%)]. A total of 1641 (26%) patients had at least one of the ESSDAI systemic features. Cardiovascular manifestations were the most frequent organ-specific group of non-ESSDAI features reported in our patients (17% of the total cohort), with Raynaud's phenomenon being reported in 15%. Patients with systemic disease due to non-ESSDAI features had a lower frequency of dry mouth (90.7% vs. 94.1%, p<0.001) and positive minor salivary gland biopsy (86.7% vs. 89%, p=0.033), a higher frequency of anti-Ro/SSA (74.7% vs. 68.7%, p<0.001), anti-La/SSB antibodies (44.5% vs. 40.4%, p=0.004), ANA (82.7% vs. 79.5%, p=0.006), low C3 levels (17.4% vs. 9.7%, p<0.001), low C4 levels (14.4% vs. 9.6%, p<0.001), and positive serum cryoglobulins (8.6% vs. 5.5%, p=0.001). Systemic activity measured by the ESSDAI, clinESSDAI and DAS was higher in patients with systemic disease out of the ESSDAI in comparison with those without these features (p<0.001 for all comparisons). Conclusions: More than a quarter of patients with primary SS may have systemic manifestations not currently included in the ESSDAI classification, with a wide variety of cardiovascular, digestive, pulmonary, neurological, ocular

Published
2019

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

Author

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

Published
2018

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

Author

Brito-Zerón, P., Acar-Denizli, N., Ng, Wf, Zeher, M., Rasmussen, A., Mandl, T., Seror, R., Li, X., Baldini, C., Gottenberg, Je, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, Aa, Kwok, Sk, Marika Kvarnström, Praprotnik, S., Sène, D., Bartoloni, E., Solans, R., Rischmueller, M., Suzuki, Y., Isenberg, Da, Valim, V., Wiland, P., Nordmark, G., Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensec, V., Knopf, A., Bombardieri, M., Trevisani, Vf, Hammenfors, D., Pasoto, Sg, Retamozo, S., Gheita, Ta, Atzeni, F., Morel, J., Vollenveider, C., Horvath, If, Sivils, Kl, Olsson, P., Vita, S., Sánchez-Guerrero, J., Kilic, L., Wahren-Herlenius, M., Mariette, X., Ramos-Casals, M., Sjögren Big Data Consortium, Hôpital Bicêtre, Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Service de rhumatologie [Strasbourg], CHU Strasbourg-Hôpital de Hautepierre [Strasbourg], Hôpital Lariboisière, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Lariboisière-Fernand-Widal [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), CHRU Brest - Service de Rhumatologie (CHU - BREST - Rhumato), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Département de Rhumatologie[Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Lapeyronie, Institut de Génétique Moléculaire de Montpellier (IGMM), Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Hôpital Bicêtre-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris-Sud - Paris 11 (UP11), Herrada, Anthony, and Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS)

Subjects
Adult, Male, [SDV.MHEP.RSOA] Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, Complement C4, Complement C3, Middle Aged, Aged, Antibodies, Antinuclear, Autoantibodies, Biomarkers, Cryoglobulins, Female, Humans, Phenotype, Prognosis, Registries, Rheumatoid Factor, Sjogren's Syndrome, Antibodies, [SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, Antinuclear
Abstract

International audience; OBJECTIVES:To evaluate the influence of the main immunological markers on the disease phenotype at diagnosis in a large international cohort of patients with primary Sjögren's syndrome (SjS).METHODS:The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. As a first step, baseline clinical information from leading centres on clinical research in SjS of the 5 continents was collected. The centres shared a harmonised data architecture and conducted cooperative online efforts in order to refine collected data under the coordination of a big data statistical team. Inclusion criteria were the fulfillment of the 2002 classification criteria. Immunological tests were carried out using standard commercial assays.RESULTS:By January 2018, the participant centres had included 10,500 valid patients from 22 countries. The cohort included 9,806 (93%) women and 694 (7%) men, with a mean age at diagnosis of primary SjS of 53 years, mainly White (78%) and included from European countries (71%). The frequency of positive immunological markers at diagnosis was 79.3% for ANA, 73.2% for anti-Ro, 48.6% for RF, 45.1% for anti- La, 13.4% for low C3 levels, 14.5% for low C4 levels and 7.3% for cryoglobulins. Positive autoantibodies (ANA, Ro, La) correlated with a positive result in salivary gland biopsy, while hypocomplementaemia and especially cryoglo-bulinaemia correlated with systemic activity (mean ESSDAI score of 17.7 for cryoglobulins, 11.3 for low C3 and 9.2 for low C4, in comparison with 3.8 for negative markers). The immunological markers with a great number of statistically-significant associations (p

Published
2018

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

Author

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

Published
2018

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

Author

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

Published
2018

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

Author

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

Published
2018

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

Author

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

Published
2018
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37. Influence of epidemiology and ethnicity on systemic expression of primary Sjögren syndrome in 9974 patients

Author

Retamozo, S., Acar-Denizli, N., Ng, W. Fai, Zeher, M., Rasmussen, A., Seror, R., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, A. A., Kwok, S. -K, Wahren-Herlenius, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Rischmueller, M., Mandl, T., Suzuki, Y., Isenberg, D., Valim, V., Wiland, P., Nordmark, Gunnel, Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensee, V., Hofauer, B., Bombardieri, M., Fernandes Moca Trevisani, V., Hammenfors, D., Pasoto, S. G., Gheita, T. A., Atzeni, F., Morel, J., Vollenveider, C., Brito-Zeron, P., Ramos-Casals, M., Retamozo, S., Acar-Denizli, N., Ng, W. Fai, Zeher, M., Rasmussen, A., Seror, R., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, A. A., Kwok, S. -K, Wahren-Herlenius, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Rischmueller, M., Mandl, T., Suzuki, Y., Isenberg, D., Valim, V., Wiland, P., Nordmark, Gunnel, Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensee, V., Hofauer, B., Bombardieri, M., Fernandes Moca Trevisani, V., Hammenfors, D., Pasoto, S. G., Gheita, T. A., Atzeni, F., Morel, J., Vollenveider, C., Brito-Zeron, P., and Ramos-Casals, M.

Published
2018
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38. Baseline ESSDAI/DAS scores in 8061 patients with primary sjÖgren syndrome: characterization of systemic disease

Author

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

Published
2017

40. Standardisation of labial salivary gland histopathology in clinical trials in primary Sjögren's syndrome

Author

Fisher, B.A. Jonsson, R. Daniels, T. Bombardieri, M. Brown, R.M. Morgan, P. Bombardieri, S. Ng, W.-F. Tzioufas, A.G. Vitali, C. Shirlaw, P. Haacke, E. Costa, S. Bootsma, H. Devauchelle-Pensec, V. Radstake, T.R. Mariette, X. Richards, A. Stack, R. Bowman, S.J. Barone, F. De Albuquerque, R.P.P. Allen, R. Astorri, E. Baldini, C. Bhabra, R. Brito-Zerón, P. Chengot, P. Fedele, S. Kruize, A.A. Omdal, R. Porter, S. Potts, J. Poveda-Gallego, A. Price, E. Priori, R. Ramos-Casals, M. Retamozo, S. Suchak, K. Sutcliffe, N. Syed, Z. Tappuni, A.R. Theander, E. Wahren-Herlenius, M. Triantafyllou, A. Vissink, A. De Vita, S. Yap, C. Osayi, K. Sjogren's histopathology workshop group (appendix) from ESSENTIAL (EULAR Sjogren's syndrome study group)

Abstract

Labial salivary gland (LSG) biopsy is used in the classification of primary Sjögren's syndrome (PSS) and in patient stratification in clinical trials. It may also function as a biomarker. The acquisition of tissue and histological interpretation is variable and needs to be standardised for use in clinical trials. A modified European League Against Rheumatism consensus guideline development strategy was used. The steering committee of the ad hoc working group identified key outstanding points of variability in LSG acquisition and analysis. A 2-day workshop was held to develop consensus where possible and identify points where further discussion/data was needed. These points were reviewed by a subgroup of experts on PSS histopathology and then circulated via an online survey to 50 stakeholder experts consisting of rheumatologists, histopathologists and oral medicine specialists, to assess level of agreement (0-10 scale) and comments. Criteria for agreement were a mean score ≥6/10 and 75% of respondents scoring ≥6/10. Thirty-nine (78%) experts responded and 16 points met criteria for agreement. These points are focused on tissue requirements, identification of the characteristic focal lymphocytic sialadenitis, calculation of the focus score, identification of germinal centres, assessment of the area of leucocyte infiltration, reporting standards and use of prestudy samples for clinical trials. We provide standardised consensus guidance for the use of labial salivary gland histopathology in the classification of PSS and in clinical trials and identify areas where further research is required to achieve evidence-based consensus. © 2016 BMJ Publishing Group Limited.

Published
2017

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

Author

Retamozo, S., primary, Brito-Zerón, P., additional, Pérez-Alvarez, R., additional, Kostov, B., additional, Feijoo Masso, C., additional, Fraile, G., additional, Gómez-de-la-Torre, R., additional, De-Escalante, B., additional, López-Dupla, M., additional, Alguacil, A., additional, Chara-Cervantes, J., additional, Pérez-Conesa, M., additional, Rascón, J., additional, Garcia-Morillo, J.S., additional, Perez-Guerrero, P., additional, Fonseca, E., additional, Akasbi, M., additional, Bonet, M., additional, Callejas, J.L., additional, De-la-Red, G., additional, Calvo, E., additional, Soler, C., additional, Peral-Gutiérrez, E., additional, Gómez-Cerezo, J.F., additional, Cruz-Caparrós, G., additional, Rodríguez-Fernández, S., additional, Pinilla, B., additional, Gato, A., additional, Rolo, A., additional, Morcillo, C., additional, Robles, A., additional, Ojeda, I., additional, Vives, M.J., additional, de-Miguel, B., additional, Penadés, M., additional, De-Vicente, M., additional, Bosch, X., additional, Pérez-de-Lis, M., additional, González-García, A., additional, Yllera, C., additional, Gracia-Tello, B., additional, Perez-Gonzalez, A., additional, Pedrosa, M., additional, Tolosa, C., additional, Pallarés, L., additional, and Ramos-Casals, M., additional

Published
2018
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42. SAT0457 SjÖgren big data project, the first example of data sharing in autoimmune diseases: analysis of 10475 worldwide patients

Author

Retamozo, S., primary, Acar-Denizli, N., additional, Fai Ng, W., additional, Zeher, M., additional, Rasmussen, A., additional, Mandl, T., additional, Seror, R., additional, Li, X., additional, Baldini, C., additional, Gottenberg, J.-E., additional, Danda, D., additional, Quartuccio, L., additional, Minniti, A., additional, Hernandez-Molina, G., additional, Kalyoncu, U., additional, Kruize, A.A., additional, Kwok, S.-K., additional, Wahren-Herlenius, M., additional, Praprotnik, S., additional, Sene, D., additional, Bartoloni, E., additional, Solans, R., additional, Rischmueller, M., additional, Suzuki, Y., additional, Isenberg, D., additional, Valim, V., additional, Wiland, P., additional, Nordmark, G., additional, Fraile, G., additional, Bootsma, H., additional, Nakamura, T., additional, Giacomelli, R., additional, Devauchelle-Pensec, V., additional, Hofauer, B., additional, Bombardieri, M., additional, Fernandes Moça Trevisani, V., additional, Hammenfors, D., additional, Pasoto, S.G., additional, Gheita, T.A., additional, Atzeni, F., additional, Morel, J., additional, Vollenveider, C., additional, Brito-Zerón, P., additional, and Ramos-Casals, M., additional

Published
2018
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45. Analysis Of 9302 Patients From The Big Data International Primary Sjogren Syndrome Cohort : Clinical Presentation At Diagnosis Of European Vs Non-European Patients

Author

Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Priori, R., Quartuccio, L., Hernandez-Molina, G., Kruize, A., Park, S. -H, Kvarnstrom, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Suzuki, Y., Isenberg, D., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Sebastian, A., Vissink, A., Nakamura, T., Valim, V., Giacomelli, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Trevisani, V., Hammenfors, D., Carsons, S. E., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Priori, R., Quartuccio, L., Hernandez-Molina, G., Kruize, A., Park, S. -H, Kvarnstrom, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Suzuki, Y., Isenberg, D., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Sebastian, A., Vissink, A., Nakamura, T., Valim, V., Giacomelli, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Trevisani, V., Hammenfors, D., Carsons, S. E., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., and Ramos-Casals, M.

Published
2017
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46. Baseline Essdai/ Das Scores In 8061 Patients With Primary Sjögren Syndrome : Characterization Of Systemic Disease

Author

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

Published
2017
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47. Predicting Survival In 6240 Patients With Primary Sjögren' Syndrome (Big Data Sjögren Project)

Author

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

Published
2017
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48. Early diagnosis of primary Sjögrens syndrome: EULAR-SS task force clinical recommendations

Author

Brito-Zerón, P. Theander, E. Baldini, C. Seror, R. Retamozo, S. Quartuccio, L. Bootsma, H. Bowman, S.J. Dörner, T. Gottenberg, J.-E. Mariette, X. Bombardieri, S. De Vita, S. Mandl, T. Ng, W.-F. Kruize, A.A. Tzioufas, A. Vitali, C. Buyon, J. Izmirly, P. Fox, R. Ramos-Casals, M.

Subjects
stomatognathic diseases
Abstract

Sjögrens 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. © 2015 Taylor & Francis.

Published
2016

49. THU0619 Prevalence of pneumococcal vaccination in rheumatologic patients with community acquired pneumonia. biobadasar registry

Author

Gόmez, G, primary, Brigante, A, additional, Benitez, A, additional, Cerda, O, additional, Retamozo, S, additional, Gandino, I, additional, Quinteros, A, additional, Exeni, I, additional, Barrios, B, additional, Astesana, P, additional, Andia, C Sanchez, additional, Collado, MV, additional, Granel, A, additional, Cappuccio, AM, additional, Quintana, R, additional, Mussano, E, additional, Smichowski, A, additional, Sota, M de la, additional, Kirmayr, K, additional, Velozo, E, additional, Larroude, MS, additional, Bertoli, A, additional, Agüero, S, additional, Battagliotti, C, additional, Souza, S Soaures de, additional, Cavillon, E, additional, Bohr, A, additional, Rillo, O, additional, Carlevaris, L, additional, Bedoya, E, additional, Kerzberg, E, additional, Kisluk, B, additional, Petkovic, I, additional, Pereira, D, additional, Barreira, JC, additional, Somma, L, additional, Costi, C, additional, Melo, F, additional, Virasoro, B, additional, Paira, S, additional, Perez, L Roa, additional, Casado, G, additional, and Celina, DLV María, additional

Published
2017
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