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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. POS1246 CHARACTERIZATION OF 785 PATIENTS WITH PULMONARY MANIFESTATIONS OF SJÖGREN DISEASE: RESULTS FROM THE BIG DATA SJÖGREN REGISTRY

Author

Alunno, A., primary, Carubbi, F., additional, Flores-Chávez, A., additional, Szántó, A., additional, Sebastian, A., additional, Rischmueller, M., additional, Ng, W. F., additional, Pasoto, S., additional, Kaya, B., additional, Solans-Laqué, R., additional, Rasmussen, A., additional, Hernandez-Molina, G., additional, Reis de Oliveira, F., additional, Suzuki, Y., additional, Bandeira, M., additional, Quartuccio, L., additional, Melchor Díaz, S., additional, Priori, R., additional, Devauchelle-Pensec, V., additional, Bartoloni, E., additional, Inanc, N., additional, Vissink, A., additional, Lopez-Dupla, J. M., additional, Valim, V., additional, Álvarez Troncoso, J., additional, Policarpo-Torres, G., additional, Baldini, C., additional, Perdan-Pirkmajer, K., additional, Vázquez, M., additional, González, A., additional, Hofauer, B., additional, Atzeni, F., additional, Fonseca-Aizpuru, E., additional, Akasbi Montalvo, M., additional, Nakamura, H., additional, Ramos-Casals, M., additional, and Brito-Zerón, P., additional

Published
2024
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3. 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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4. Cardiovascular events prior to or early after diagnosis of systemic lupus erythematosus in the systemic lupus international collaborating clinics cohort

Author

Urowitz, MB, Gladman, DD, Anderson, NM, Su, J, Romero-Diaz, J, Bae, SC, Fortin, PR, Sanchez-Guerrero, J, Clarke, A, Bernatsky, S, Gordon, C, Hanly, JG, Wallace, DJ, Isenberg, D, Rahman, A, Merrill, J, Ginzler, E, Alarcón, GS, Fessler, BF, Petri, M, Bruce, IN, Khamashta, M, Aranow, C, Dooley, M, Manzi, S, Ramsey-Goldman, R, Sturfelt, G, Nived, O, Steinsson, K, Zoma, A, Ruiz-Irastorza, G, Lim, S, Kalunian, KC, Ỉnanç, M, van Vollenhoven, R, Ramos-Casals, M, Kamen, DL, Jacobsen, S, Peschken, C, Askanase, A, and Stoll, T

Subjects
Reproductive Medicine, Biomedical and Clinical Sciences, Prevention, Atherosclerosis, Lupus, Cardiovascular, Autoimmune Disease, Clinical Research, Evaluation of treatments and therapeutic interventions, 4.1 Discovery and preclinical testing of markers and technologies, Detection, screening and diagnosis, 6.1 Pharmaceuticals, Inflammatory and immune system, Cardiovascular Disease, Inflammation, Systemic Lupus Erythematosus, Clinical sciences, Immunology
Abstract

ObjectiveTo describe the frequency of myocardial infarction (MI) prior to the diagnosis of systemic lupus erythematosus (SLE) and within the first 2 years of follow-up.MethodsThe systemic lupus international collaborating clinics (SLICC) atherosclerosis inception cohort enters patients within 15 months of SLE diagnosis. MIs were reported and attributed on a specialised vascular event form. MIs were confirmed by one or more of the following: abnormal ECG, typical or atypical symptoms with ECG abnormalities and elevated enzymes (≥2 times upper limit of normal), or abnormal stress test, echocardiogram, nuclear scan or angiogram. Descriptive statistics were used.Results31 of 1848 patients who entered the cohort had an MI. Of those, 23 patients had an MI prior to SLE diagnosis or within the first 2 years of disease. Of the 23 patients studied, 60.9% were female, 78.3% were Caucasian, 8.7% black, 8.7% Hispanic and 4.3% other. The mean age at SLE diagnosis was 52.5±15.0 years. Of the 23 MIs that occurred, 16 MIs occurred at a mean of 6.1±7.0 years prior to diagnosis and 7 occurred within the first 2 years of follow-up. Risk factors associated with early MI in univariate analysis are male sex, Caucasian, older age at diagnosis, hypertension, hypercholesterolaemia, family history of MI and smoking. In multivariate analysis only age (OR=1.06 95% CI 1.03 to 1.09), hypertension (OR=5.01, 95% CI 1.38 to 18.23), hypercholesterolaemia (OR=4.43, 95% CI 1.51 to 12.99) and smoking (OR=7.50, 95% CI 2.38 to 23.57) remained significant risk factors.ConclusionsIn some patients with lupus, MI may develop even before the diagnosis of SLE or shortly thereafter, suggesting that there may be a link between autoimmune inflammation and atherosclerosis.

Published
2016

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

Author

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

Published
2019
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7. 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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8. 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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9. IgG4-Related Disease

Author

Brito-Zerón, P., primary, Bosch, X., additional, Gandía, M., additional, Soto Cárdenas, M.-J., additional, Ramos-Casals, M., additional, and Stone, J.H., additional

Published
2017
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15. 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
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16. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögrenʼs syndrome: A consensus and data-driven methodology involving three international patient cohorts

Author

Shiboski, Caroline H, Shiboski, Stephen C, Seror, Raphaèle, Criswell, Lindsey A, Labetoulle, Marc, Lietman, Thomas M, Rasmussen, Astrid, Scofield, Hal, Vitali, Claudio, Bowman, Simon J, Mariette, Xavier, Heidenreich, AM, Lanfranchi, H, Vollenweider, C, Schiødt, M., Devauchelle, V., Gottenberg, JE, Saraux, A, Pincemin, Maggy, Dörner, T, Tzoufias, A, Baldini, C, Bombardieri, S, De Vita, S, Kitagawa, K, Sumida, T, Umehara, H, Bootsma, H, Kruize, AA, Radstake, TR, Vissink, A, Jonsson, R, Ramos-Casals, M, Theander, E, Challacombe, S, Fisher, B, Kirkham, B, Larkin, G, Ng, F, Rauz, S, Akpek, E, Atkinson, J, Baer, AN, Carsons, S, Carteron, N, Daniels, T, Fox, B, Greenspan, J, Illei, G, Nelson, D, Parke, A, Pillemer, S, Segal, B, Sivils, K, Clair, EW St, Stone, D, Vivino, F, Wu, A, and Hammitt, Kathy

Published
2017
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17. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögrenʼs Syndrome: A Consensus and Data‐Driven Methodology Involving Three International Patient Cohorts

Author

Shiboski, Caroline H., Shiboski, Stephen C., Seror, Raphaèle, Criswell, Lindsey A., Labetoulle, Marc, Lietman, Thomas M., Rasmussen, Astrid, Scofield, Hal, Vitali, Claudio, Bowman, Simon J., Mariette, Xavier, Heidenreich, A. M., Lanfranchi, H., Vollenweider, C, Schiødt, M., Devauchelle, V, Gottenberg, J. E, Saraux, A, Pincemin, Maggy, Dörner, T., Tzoufias, A, Baldini, C, Bombardieri, S, De Vita, S., Kitagawa, K, Sumida, T, Umehara, H, Bootsma, H, Kruize, A. A., Radstake, T. R., Vissink, A, Jonsson, R, Ramos‐Casals, M., Theander, E, Challacombe, S, Fisher, B, Kirkham, B, Larkin, G, Ng, F, Rauz, S, Akpek, E, Atkinson, J, Baer, A. N., Carsons, S, Carteron, N, Daniels, T., Fox, B, Greenspan, J, Illei, G, Nelson, D, Parke, A, Pillemer, S, Segal, B, Sivils, K, St.Clair, E. W., Stone, D, Vivino, F, and Wu, A.

Published
2017
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18. Addressing the clinical unmet needs in primary Sjögren's Syndrome through the sharing, harmonization and federated analysis of 21 European cohorts

Author

Pezoulas, V.C. Goules, A. Kalatzis, F. Chatzis, L. Kourou, K.D. Venetsanopoulou, A. Exarchos, T.P. Gandolfo, S. Votis, K. Zampeli, E. Burmeister, J. May, T. Marcelino Pérez, M. Lishchuk, I. Chondrogiannis, T. Andronikou, V. Varvarigou, T. Filipovic, N. Tsiknakis, M. Baldini, C. Bombardieri, M. Bootsma, H. Bowman, S.J. Shahnawaz Soyfoo, M. Parisis, D. Delporte, C. Devauchelle-Pensec, V. Pers, J.-O. Dörner, T. Bartoloni, E. Gerli, R. Giacomelli, R. Jonsson, R. Ng, W.-F. Priori, R. Ramos-Casals, M. Sivils, K. Skopouli, F. Torsten, W. A. G. van Roon, J. Xavier, M. De Vita, S. Tzioufas, A.G. Fotiadis, D.I.

Abstract

For many decades, the clinical unmet needs of primary Sjögren's Syndrome (pSS) have been left unresolved due to the rareness of the disease and the complexity of the underlying pathogenic mechanisms, including the pSS-associated lymphomagenesis process. Here, we present the HarmonicSS cloud-computing exemplar which offers beyond the state-of-the-art data analytics services to address the pSS clinical unmet needs, including the development of lymphoma classification models and the identification of biomarkers for lymphomagenesis. The users of the platform have been able to successfully interlink, curate, and harmonize 21 regional, national, and international European cohorts of 7,551 pSS patients with respect to the ethical and legal issues for data sharing. Federated AI algorithms were trained across the harmonized databases, with reduced execution time complexity, yielding robust lymphoma classification models with 85% accuracy, 81.25% sensitivity, 85.4% specificity along with 5 biomarkers for lymphoma development. To our knowledge, this is the first GDPR compliant platform that provides federated AI services to address the pSS clinical unmet needs. © 2022 The Author(s)

Published
2022

19. 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

21. Mise à jour des recommandations EULAR sur l’utilisation des thérapies immunomodulatrices dans la prise en charge de la Covid-19

Author

Najm, A., primary, Alunno, A., additional, Machado, P., additional, Bertheussen, H., additional, Burmester, G.R., additional, Carubbi, F., additional, De Marco, G., additional, Giacomelli, R., additional, Hermine, O., additional, Isaacs, J.D., additional, Koné-Paut, I., additional, Magro-Checa, C., additional, McInnes, I.B., additional, Meroni, P.L., additional, Quartuccio, L., additional, Athimalaipet, V.R., additional, Ramos-Casals, M., additional, Rodríguez-Carrio, J., additional, Schultze-Koops, H., additional, Stamm, T., additional, Tas, S.W., additional, Terrier, B., additional, McGonagle, D., additional, and Mariette, X., additional

Published
2021
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22. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease

Author

Wallace, Zachary S, Naden, Ray P, Chari, Suresh, Choi, Hyon K, Della-Torre, Emanuel, Dicaire, Jean-Francois, Hart, Phillip A, Inoue, Dai, Kawano, Mitsuhiro, Khosroshahi, Arezou, Lanzillotta, Marco, Okazaki, Kazuichi, Perugino, Cory A, Sharma, Amita, Saeki, Takako, Schleinitz, Nicolas, Takahashi, Naoki, Umehara, Hisanori, Zen, Yoh, Stone, Collaborators: Akamizu T, John H., Akiyama, M, Barra, L, Bateman, A, Blockmans, D, Brito-Zeron, P, Campochiaro, C, Carruthers, M, Chiba, T, Cornell, L, Culver, E, Darabian, S, Deshpande, V, Dong, L, Ebbo, M, Fernández-Codina, A, Ferry, Ja, Fragkoulis, G, Frost, F, Frulloni, Luca, Hernandez-Molina, G, Ji, H, Keat, K, Kamisawa, T, Kawa, S, Kobayashi, H, Kodama, Y, Kubo, S, Kubota, K, Leng, H, Lerch, Mm, Liu, Y, Liu, Z, Löhr, M, Martin-Nares, E, Martinez-Valle, F, Marvisi, C, Masaki, Y, Matsui, S, Mizushima, I, Nakamura, S, Nordeide, J, Notohara, K, Paira, S, Popovic, J, Ramos-Casals, M, Rosenbaum, J, Ryu, J, Sato, Y, Sekiguchi, H, Sokol, Ev, Stone, Jr, Sun, W, Takahashi, H, Takahira, M, Tanaka, Y, Vaglio, A, Villamil, A, Wada, Y, Webster, G, Yamada, K, Yamamoto, M, Yi, J, Yi, Y, Zamboni, G, Zhang, W., Wallace, Z, Naden, Rp, Chari, S, Choi, Hk, DELLA TORRE, E, Dicaire, Jf, Hart, Pa, Inoue, D, Kawano, M, Khosroshahi, A, Lanzillotta, M, Okazaki, K, Perugino, Ca, Sharma, A, Saeki, T, Schleinitz, N, Takahashi, N, Umehara, H, Zen, Y, Stone, Jh, and Members of the ACR/EULAR IgG4-RD Classification Criteria Working, Group.

Subjects
rheumatoid arthritis, Adult, Male, 0301 basic medicine, medicine.medical_specialty, Immunology, Sensitivity and Specificity, General Biochemistry, Genetics and Molecular Biology, Diagnosis, Differential, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Internal medicine, parasitic diseases, Epidemiology, Humans, Immunology and Allergy, Medicine, Pathological, Sjøgren's syndrome, Aged, 030203 arthritis & rheumatology, business.industry, Middle Aged, inflammation, medicine.disease, Test (assessment), 030104 developmental biology, Rheumatoid arthritis, Radiological weapon, Female, Immunoglobulin G4-Related Disease, business, Rheumatism, Decision analysis
Abstract

IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serological, radiological and pathological data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises; existing literature; derivation and validation cohorts of 1879 subjects (1086 cases, 793 mimickers); and multicriterion decision analysis to identify, weight and test potential classification criteria. Two independent validation cohorts were included. A three-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least one of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serological, radiological and pathological items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, eight weighted inclusion criteria domains, addressing clinical findings, serological results, radiological assessments and pathological interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% CI 97.2% to 99.8%) and a sensitivity of 85.5% (95% CI 81.9% to 88.5%). In the second, the specificity was 97.8% (95% CI 93.7% to 99.2%) and the sensitivity was 82.0% (95% CI 77.0% to 86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. ACR/EULAR classification criteria for IgG4-RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiological and basic science investigations.

Published
2019
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24. 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

25. Characterization and Outcomes of SARS-CoV-2 Infection in Patients with Sarcoidosis

Author

Brito-Zerón, P., Gracia-Tello, B., Robles, A., Alguacil, A., Bonet, M., De-Escalante, B., Noblejas-Mosso, A., Gómez-de-la-Torre, R., Akasbi, M., Pérez-de-Lis, M., Pérez-Alvarez, R., Ramos-Casals, M., and Registry, on behalf of the SarcoGEAS-SEMI

Subjects
Adult, Male, medicine.medical_specialty, SARS-Cov-2, Comorbidity, comorbidities, Microbiology, survival, Article, law.invention, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, law, Virology, Internal medicine, Diabetes mellitus, medicine, Humans, 030212 general & internal medicine, Registries, sarcoidosis, Cardiopulmonary disease, Aged, 030203 arthritis & rheumatology, Aged, 80 and over, business.industry, COVID-19, Odds ratio, Middle Aged, medicine.disease, Intensive care unit, QR1-502, Hospitalization, hospital admission, Infectious Diseases, Treatment Outcome, Cohort, Female, Sarcoidosis, France, business, Cohort study
Abstract

To analyze the clinical characteristics and outcomes of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in patients with sarcoidosis from a large multicenter cohort from Southern Europe and to identify the risk factors associated with a more complicated infection. We searched for patients with sarcoidosis presenting with SARS-CoV-2 infection (defined according to the European Centre for Disease Prevention and Control guidelines) among those included in the SarcoGEAS Registry, a nationwide, multicenter registry of patients fulfilling the American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and Other Granulomatous Disorders 1999 classification criteria for sarcoidosis. A 2:1 age-sex-matched subset of patients with sarcoidosis without SARS-CoV-2 infection was selected as control population. Forty-five patients with SARS-CoV-2 infection were identified (28 women, mean age 55 years). Thirty-six patients presented a symptomatic SARS-CoV-2 infection and 14 were hospitalized (12 required supplemental oxygen, 2 intensive care unit admission and 1 mechanical ventilation). Four patients died due to progressive respiratory failure. Patients who required hospital admission had an older mean age (64.9 vs. 51.0 years, p = 0.006), a higher frequency of baseline comorbidities including cardiovascular disease (64% vs. 23%, p = 0.016), diabetes mellitus (43% vs. 13%, p = 0.049) and chronic liver/kidney diseases (36% vs. 0%, p = 0.002) and presented more frequently fever (79% vs. 35%, p = 0.011) and dyspnea (50% vs. 3%, p = 0.001) in comparison with patients managed at home. Age- and sex-adjusted multivariate analysis identified the age at diagnosis of SARS-Cov-2 infection as the only independent variable associated with hospitalization (adjusted odds ratio 1.18, 95% conficence interval 1.04–1.35). A baseline moderate/severe pulmonary impairment in function tests was associated with a higher rate of hospitalization but the difference was not statistically significant (50% vs. 23%, p = 0.219). A close monitoring of SARS-CoV-2 infection in elderly patients with sarcoidosis, especially in those with baseline cardiopulmonary diseases and chronic liver or renal failure, is recommended. The low frequency of severe pulmonary involvement in patients with sarcoidosis from Southern Europe may explain the weak prognostic role of baseline lung impairment in our study, in contrast to studies from other geographical areas.

Published
2021
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27. 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

28. 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

29. Long Covid-19: Proposed Primary Care Clinical Guidelines for Diagnosis and Disease Management

Author

Medicina i Cirurgia, Universitat Rovira i Virgili, Sisó-Almirall A; Brito-Zerón P; Conangla Ferrín L; Kostov B; Moragas Moreno A; Mestres J; Sellarès J; Galindo G; Morera R; Basora J; Trilla A; Ramos-Casals M; On Behalf Of The CAMFiC Long Covid-Study Group, Medicina i Cirurgia, Universitat Rovira i Virgili, and Sisó-Almirall A; Brito-Zerón P; Conangla Ferrín L; Kostov B; Moragas Moreno A; Mestres J; Sellarès J; Galindo G; Morera R; Basora J; Trilla A; Ramos-Casals M; On Behalf Of The CAMFiC Long Covid-Study Group

Abstract

Long COVID-19 may be defined as patients who, four weeks after the diagnosis of SARS-Cov-2 infection, continue to have signs and symptoms not explainable by other causes. The estimated frequency is around 10% and signs and symptoms may last for months. The main long-term manifestations observed in other coronaviruses (Severe Acute Respiratory Syndrome (SARS), Middle East respiratory syndrome (MERS)) are very similar to and have clear clinical parallels with SARS-CoV-2: mainly respiratory, musculoskeletal, and neuropsychiatric. The growing number of patients worldwide will have an impact on health systems. Therefore, the main objective of these clinical practice guidelines is to identify patients with signs and symptoms of long COVID-19 in primary care through a protocolized diagnostic process that studies possible etiologies and establishes an accurate differential diagnosis. The guidelines have been developed pragmatically by compiling the few studies published so far on long COVID-19, editorials and expert opinions, press releases, and the authors' clinical experience. Patients with long COVID-19 should be managed using structured primary care visits based on the time from diagnosis of SARS-CoV-2 infection. Based on the current limited evidence, disease management of long COVID-19 signs and symptoms will require a holistic, longitudinal follow up in primary care, multidisciplinary rehabilitation services, and the empowerment of affected patient groups.

Published
2021

30. 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

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

Author

Brito-Zerón, Pilar, Melchor, Sheila, Seror, Raphaèle, Priori, Roberta, Solans, Roser, Kostov, Belchin, Baldini, Chiara, Carubbi, Francesco, Callejas, Jose Luis, Guisado-Vasco, Pablo, Hernández-Molina, Gabriela, Pasoto, Sandra G, Valim, Valeria, Sisó-Almirall, Antoni, Mariette, Xavier, Carreira, Patricia, Ramos-Casals, Manuel, Brito-Zerón, P, Morcillo, C, Flores-Chávez, A, Ramos-Casals, M, Acar-Denizli, N, Horvath, I F, Szanto, A, Tarr, T, Seror, R, Mariette, X, Mandl, T, Olsson, P, Li, X, Xu, B, Baldini, C, Bombardieri, S, Gottenberg, J E, Gandolfo, S, De Vita, S, Priori, R, Giardina, F, Hernandez-Molina, G, Sánchez-Guerrero, J, Kruize, A A, Hinrichs, A, Valim, V, Isenberg, D, Solans, R, Rischmueller, M, Downie-Doyle, S, Kwok, S-K, Park, S-H, Nordmark, G, Suzuki, Y, Kawano, M, Giacomelli, R, Devauchelle-Pensec, V, Saraux, A, Hofauer, B, Knopf, A, Bootsma, H, Vissink, A, Morel, J, Vollenveider, C, Atzeni, F, Retamozo, S, Moça Trevisano, V, Armagan, B, Kilic, L, Kalyoncu, U, Pasoto, S G, Kostov, B, Sisó-Almirall, A, Consani-Fernández, S, Carubbi, F, Callejas, J L, López-Dupla, M, Pérez-Alvarez, R, Akasbi, M, Guisado-Vasco, P, Sánchez, I, Hospital Universitario 12 de Octubre [Madrid], EULAR standing committee of People with Arthritis/Rheumatism in Europe (PARE), H. CIMA-Sanitas, Barcelona, CELLEX-IDIBAPS Department of Autoimmune Diseases, Barcelona, Instituto Mexicano del Seguro Social [Mexico City, Mexico] (IMSS), Universidad de Colima [Mexico], Hospital Clinic [Barcelona, Spain], İstanbul Üniversitesi, İstanbul, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary, MTA-Debreceni Egyetem, Immunologie des maladies virales, auto-immunes, hématologiques et bactériennes (IMVA-HB), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay, Center for Immunology of Viral Infections and Autoimmune Diseases, Le Kremlin Bicêtre, Skåne University Hospital [Malmö, Suède], Stockholm University, Wuhan University [China], Pisa University Hospital, Nuclear Medicine Unit, Humanitas Gavazzeni, Bergamo, Italy, University Hospitals, Strasbourg, Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome] (UNIROMA), Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán - National Institute of Medical Science and Nutrition Salvador Zubiran [Mexico], Federal University of Espírito Santo, University Hospital Vall d’Hebròn [Barcelona, Spain], Lymphocytes B, Autoimmunité et Immunothérapies (LBAI), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-LabEX IGO Immunothérapie Grand Ouest, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Institut de Génétique Moléculaire de Montpellier (IGMM), Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Institut d'Investigaciones Biomèdiques August Pi i Sunye (IDIBAPS), Università degli studi di Palermo - University of Palermo, Hospitales Universitarios de Granada/Universidad de Granada, Hospital Universitario Quironsalud, Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome], Lymphocyte B et Auto-immunité (LBAI), Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Rheumatology Unit, Department of Internal Medicine, University of Palermo, Palermo, Italy, and Michel, Geneviève

Subjects
Male, medicine.medical_specialty, Multivariate analysis, [SDV.IMM] Life Sciences [q-bio]/Immunology, Coronavirus disease 2019 (COVID-19), Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Comorbidity, Laboratory abnormality, comorbidities, outcomes, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Risk Factors, Internal medicine, medicine, Humans, In patient, Pharmacology (medical), 030212 general & internal medicine, Registries, Risk factor, Primary Sjögren Syndrome, AcademicSubjects/MED00360, ComputingMilieux_MISCELLANEOUS, 030203 arthritis & rheumatology, business.industry, SARS-CoV-2, COVID-19, Primary SS, SARS-Cov-2, Middle Aged, medicine.disease, 3. Good health, Hospitalization, Sjogren's Syndrome, [SDV.IMM]Life Sciences [q-bio]/Immunology, Original Article, Female, business
Abstract

Objective To analyse the prognosis and outcomes of SARS-CoV-2 infection in patients with primary SS. Methods We searched for patients with primary SS presenting with SARS-CoV-2 infection (defined following and according to the European Centre for Disease Prevention and Control guidelines) among those included in the Big Data Sjögren Registry, an international, multicentre registry of patients diagnosed according to the 2002/2016 classification criteria. Results A total of 51 patients were included in the study (46 women, mean age at diagnosis of infection of 60 years). According to the number of patients with primary SS evaluated in the Registry (n = 8211), the estimated frequency of SARS-CoV-2 infection was 0.62% (95% CI 0.44, 0.80). All but two presented with symptoms suggestive of COVID-19, including fever (82%), cough (57%), dyspnoea (39%), fatigue/myalgias (27%) and diarrhoea (24%), and the most frequent abnormalities included raised lactate dehydrogenase (LDH) (88%), CRP (81%) and D-dimer (82%) values, and lymphopenia (70%). Infection was managed at home in 26 (51%) cases and 25 (49%) required hospitalization (five required admission to ICU, four died). Compared with patients managed at home, those requiring hospitalization had higher odds of having lymphopenia as laboratory abnormality (adjusted OR 21.22, 95% CI 2.39, 524.09). Patients with comorbidities had an older age (adjusted OR 1.05, 95% CI 1.00, 1.11) and showed a risk for hospital admission six times higher than those without (adjusted OR 6.01, 95% CI 1.72, 23.51) in the multivariate analysis. Conclusion Baseline comorbidities were a key risk factor for a more complicated COVID-19 in patients with primary SS, with higher rates of hospitalization and poor outcomes in comparison with patients without comorbidities.

Published
2020
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34. 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

35. 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

38. Autoantibodies as biomarkers for the prediction of neuropsychiatric events in systemic lupus erythematosus

Author

Hanly, J G, Urowitz, M B, Su, L, Bae, S-C, Gordon, C, Clarke, A, Bernatsky, S, Vasudevan, A, Isenberg, D, Rahman, A, Wallace, D J, Fortin, P R, Gladman, D, Romero-Dirz, J, Sanchez-Guerrero, J, Dooley, M A, Bruce, I, Steinsson, K, Khamashta, M, Manzi, S, Ramsey-Goldman, R, Sturfelt, G, Nived, O, van Vollenhoven, R, Ramos-Casals, M, Aranow, C, Mackay, M, Kalunian, K, Alarcón, G S, Fessler, B J, Ruiz-Irastorza, G, Petri, M, Lim, S, Kamen, D, Peschken, C, Farewell, V, Thompson, K, Theriault, C, and Merrill, J T

Published
2011
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39. SF-36 summary and subscale scores are reliable outcomes of neuropsychiatric events in systemic lupus erythematosus

Author

Hanly, J G, Urowitz, M B, Jackson, D, Bae, S C, Gordon, C, Wallace, D J, Clarke, A, Bernatsky, S, Vasudevan, A, Isenberg, D, Rahman, A, Sanchez-Guerrero, J, Romero-Diaz, J, Merrill, J T, Fortin, P R, Gladman, D D, Bruce, I N, Steinsson, K, Khamashta, M, Alarcón, G S, Fessler, B, Petri, M, Manzi, S, Nived, O, Sturfelt, G, Ramsey-Goldman, R, Dooley, M A, Aranow, C, Van Vollenhoven, R, Ramos-Casals, M, Zoma, A, Kalunian, K, and Farewell, V

Published
2011
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42. Prospective analysis of neuropsychiatric events in an international disease inception cohort of patients with systemic lupus erythematosus

Author

Hanly, J G, Urowitz, M B, Su, L, Bae, S C, Gordon, C, Wallace, D J, Clarke, A, Bernatsky, S, Isenberg, D, Rahman, A, Alarcón, G S, Gladman, D D, Fortin, P R, Sanchez-Guerrero, J, Romero-Diaz, J, Merrill, J T, Ginzler, E, Bruce, I N, Steinsson, K, Khamashta, M, Petri, M, Manzi, S, Dooley, M A, Ramsey-Goldman, R, Van Vollenhoven, R, Nived, O, Sturfelt, G, Aranow, C, Kalunian, K, Ramos-Casals, M, Zoma, A, Douglas, J, Thompson, K, and Farewell, V

Published
2010
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45. Extrahepatic manifestations associated with Chronic Hepatitis C Virus Infection

Author

Flores-Chávez, A., Carrion, J.A., Forns, X., and Ramos-Casals, M.

Subjects
hepacivirus, Revision, España, enfermedades autoinmunes, lcsh:Medicine, lymphoma, prisons, Autoimmune Diseases, Central Nervous System Diseases, antiviral agents, Humans, autoinmune diseases, linfoma, lcsh:Public aspects of medicine, lcsh:R, antivirales, lcsh:RA1-1270, Hepatitis C, Chronic, Hematologic Diseases, Fatty Liver, prisiones, Cardiovascular Diseases, Spain, diabetes mellitus, Medicine, hepatitis C, Public aspects of medicine, RA1-1270
Abstract

Summary Chronic hepatitis C virus (HCV) infection has been associated with both organ-specific and systemic autoimmune diseases, with cryoglobulinemia being the most frequent associated disease. Experimental, virologic, and clinical evidence have demon-strated a close association between HCV infection and some systemic autoimmune diseases, especially Sjögren’s syndrome, but also rheumatoid arthritis and lupus. A higher prevalence of hematological processes has also been described in patients with HCV infection, including cytopenias and lymphoproliferative disorders (B-cell lymphoma). In addition, patients with chronic HCV infection have a higher frequency of other extrahepatic manifestations including endocrine, metabolic and cardiovascular disorders that may worse the prognosis of patients, along with neuropsychiatric manifestations and general symptoms that have a significant influence on the quality of life of the patient. Direct-acting antiviral therapies (DAAs) that have recently begun to be used are providing the opportunity to effectively cure chronic HCV infection and reduce the burden of both hepatic and extrahepatic complications.

Published
2017

48. 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
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49. 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

50. 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

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