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1. 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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2. 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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3. 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

4. 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

6. 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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7. Defining disease activity states and clinically meaningful improvement in primary Sjögren's syndrome with EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI)

Author

Seror, R. Bootsma, H. Saraux, A. Bowman, S.J. Theander, E. Brun, J.G. Baron, G. Le Guern, V. Devauchelle-Pensec, V. Ramos-Casals, M. Valim, V. Dörner, T. Tzioufas, A. Gottenberg, J.-E. Laqué, R.S. Mandl, T. Hachulla, E. Sivils, K.L. Ng, W.-F. Fauchais, A.-L. Bombardieri, S. Priori, R. Bartoloni, E. Goeb, V. Praprotnik, S. Sumida, T. Nishiyama, S. Caporali, R. Kruize, A.A. Vollenweider, C. Ravaud, P. Meiners, P. Brito-Zerón, P. Vitali, C. Mariette, X. Gerli, R. Kallenberg, C. De Vita, S. Demoulins, F. Montecucco, C. Tomsic, M. Scofield, H. Valesini, G. EULAR Sjogren's Task Force

Abstract

Objectives To define disease activity levels, minimal clinically important improvement (MCII) and patientacceptable symptom state (PASS) with the primary Sjögren's syndrome (SS) disease activity indexes: European League Against Rheumatism (EULAR) SS disease activity index (ESSDAI) and EULAR SS patientreported index (ESSPRI). Methods For 790 patients from two large prospective cohorts, ESSDAI, physician evaluation of disease activity, ESSPRI and patients' satisfaction with their current health status were recorded. Receiver operating characteristic curve analyses and anchoring methods were used to estimate disease activity levels of ESSDAI and the PASS of ESSPRI. At follow-up visit, patients and physicians assessed, respectively, whether symptoms and disease activity have improved or not. An anchoring method based on this evaluation was used to estimate MCII of ESSDAI and ESSPRI. Results Low-activity (ESSDAI

Published
2016

8. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts

Author

MS Reumatologie/Immunologie/Infectie, Infection & Immunity, Translationele immunologie, 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., Wu, A., MS Reumatologie/Immunologie/Infectie, Infection & Immunity, Translationele immunologie, 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

9. Validation of EULAR primary Sjögren's syndrome disease activity (ESSDAI) and Patient indexes (ESSPRI)

Author

Seror, R. Theander, E. Brun, J.G. Ramos-Casals, M. Valim, V. Dörner, T. Bootsma, H. Tzioufas, A. Solans-Laqué, R. Mandl, T. Gottenberg, J.-E. Hachulla, E. Sivils, K.L. Ng, W.-F. Fauchais, A.-L. Bombardieri, S. Valesini, G. Bartoloni, E. Saraux, A. Tomsic, M. Sumida, T. Nishiyama, S. Caporali, R. Kruize, A.A. Vollenweider, C. Ravaud, P. Vitali, C. Mariette, X. Bowman, S.J.

Abstract

Objectives: To validate the two recently developed disease activity indexes for assessment of primary Sjögren's syndrome (SS): the European League Against Rheumatism (EULAR) SS Patient Reported Index (ESSPRI) and the EULAR SS Disease Activity Index (ESSDAI). Methods: A prospective international 6-month duration validation study was conducted in 15 countries. At each visit, physicians completed ESSDAI, SS disease activity index (SSDAI), Sjögren's Systemic Clinical Activity Index (SCAI) and physician global assessment (PhGA); and patients completed ESSPRI, Sicca Symptoms Inventory (SSI), Profile of Fatigue and Discomfort (PROFAD) and patient global assessment (PGA). Psychometric properties (construct validity, responsiveness and reliability) were evaluated and compared between scores. Results: Of the 395 patients included, 145 (37%) and 251 (64%) had currently active or current or past systemic manifestations, respectively. EULAR scores had higher correlation with the gold standard than other scores (ESSDAI with PhGA: r=0.59; ESSRPI with PGA: r=0.70). Correlations between patient and systemic scores were very low (ranging from 0.07 to 0.29). All systemic scores had similar large responsiveness in improved patients. Responsiveness of patient scores was low but was significantly higher for ESSPRI compared with SSI and PROFAD. Reliability was very good for all scores. Conclusions: ESSDAI and ESSPRI had good construct validity. All scores were reliable. Systemic scores had a large sensitivity to change in patients whose disease activity improves. Patient scores had a small sensitivity to change, however, significantly better for ESSPRI. Systemic and patient scores poorly correlated, suggesting that they are 2 complementary components that should be both evaluated, but separately.

Published
2015

10. Defining disease activity states and clinically meaningful improvement in primary Sjögren's syndrome with EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI)

Author

Seror, R, Bootsma, H, Saraux, A, Bowman, S, Theander, E, Brun, Jg, Baron, G, Le Guern, V, Devauchelle Pensec, V, Ramos Casals, M, Valim, V, Dörner, T, Tzioufas, A, Gottenberg, Je, Laqué, Rs, Mandl, T, Hachulla, E, Sivils, Kl, Wf, Ng, Fauchais, A, Bombardieri, S, Priori, R, BARTOLONI BOCCI, Elena, Goeb, V, Praprotnik, S, Sumida, T, Nishiyama, S, Caporali, R, Kruize, Aa, Vollenweider, C, Ravaud, P, Meiners, P, Brito Zerón, P, Vitali, C, Mariette, X, Gerli, Roberto, Kallenberg, C, De Vita, S, Demoulins, F, Montecucco, C, Tomsic, M, Scofield, H, Valesini, G., Service de rhumatologie, AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Immunologie et Pathologie (EA2216), Université de Brest (UBO)-IFR148, Centre d'Investigation Clinique (CIC - Brest), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM), Department of Rheumatology, Lund University [Lund], Education Discours Apprentissages (EDA - EA 4071), Université Paris Descartes - Paris 5 (UPD5), Klinik für Dermatologie, Venerologie und Allergologie, Department of Pathophysiology, Medical School, University of Athens, Immuno-Rhumatologie Moléculaire, Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Lille, Droit et Santé, University of Northumbria at Newcastle [United Kingdom], Service de Médecine interne A et polyclinique médicale [CHU Limoges], CHU Limoges, Service de rhumatologie [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), University Medical Centre, University of Tsukuba, Equipe 5 : METHODS - Méthodes de l’évaluation thérapeutique des maladies chroniques (CRESS - U1153), Université Paris Descartes - Paris 5 (UPD5)-Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Department of Internal Medicine and section of Rheumatology, Villamarina Hospital, 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), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Strasbourg (UNISTRA), Service de rhumatologie [CHU Rouen], Université de Tsukuba = University of Tsukuba, Université Paris Diderot - Paris 7 (UPD7)-Université Sorbonne Paris Cité (USPC)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA), Université Paris-Sud - Paris 11 (UP11)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA), Translational Immunology Groningen (TRIGR), Immunologie et Pathologie ( EA2216 ), Université de Brest ( UBO ) -IFR148, Centre d'Investigation Clinique ( CIC - Brest ), Université de Brest ( UBO ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Education Discours Apprentissages ( EDA - EA 4071 ), Université Paris Descartes - Paris 5 ( UPD5 ), Université de Strasbourg ( UNISTRA ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), CHU Rouen-Université de Rouen Normandie ( UNIROUEN ), Normandie Université ( NU ) -Normandie Université ( NU ), Equipe 5 : METHODS - Méthodes de l’évaluation thérapeutique des maladies chroniques ( CRESS - U1153 ), Université Paris Descartes - Paris 5 ( UPD5 ) -Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité ( CRESS (U1153 / UMR_A 1125) ), Institut National de la Recherche Agronomique ( INRA ) -Université Sorbonne Paris Cité ( USPC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Institut National de la Recherche Agronomique ( INRA ) -Université Sorbonne Paris Cité ( USPC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Immunologie des Maladies Virales et Autoimmunes ( IMVA - U1184 ), Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université Paris-Sud - Paris 11 ( UP11 ) -Commissariat à l'énergie atomique et aux énergies alternatives ( CEA ), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Sorbonne Paris Cité (USPC), and Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Sud - Paris 11 (UP11)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)

Subjects
0301 basic medicine, Genetics and Molecular Biology (all), Male, [SDV]Life Sciences [q-bio], Health Status, Rheumatology, Immunology, Biochemistry, Genetics and Molecular Biology (all), Immunology and Allergy, Biochemistry, Severity of Illness Index, RESPONSIVENESS, DOUBLE-BLIND, 0302 clinical medicine, EUROPEAN LEAGUE, Epidemiology, Prospective Studies, SYSTEMIC-LUPUS-ERYTHEMATOSUS, 10. No inequality, Prospective cohort study, Non-U.S. Gov't, Research Support, Non-U.S. Gov't, RANDOMIZED CONTROLLED-TRIAL, Middle Aged, 3. Good health, Sjogren's Syndrome, [SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, Patient Satisfaction, Rheumatoid arthritis, patient-reported indexes (ESSPRI), Disease Progression, Female, Symptom Assessment, medicine.medical_specialty, BELIMUMAB, Research Support, General Biochemistry, Genetics and Molecular Biology, [ SDV.MHEP.RSOA ] Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, 03 medical and health sciences, Diagnostic Self Evaluation, Patient satisfaction, Internal medicine, [ SDV.MHEP ] Life Sciences [q-bio]/Human health and pathology, Severity of illness, medicine, Journal Article, Humans, disease activity states, Aged, 030203 arthritis & rheumatology, Outcomes research, Patient perspective, Sjøgren's Syndrome, ROC Curve, [ SDV ] Life Sciences [q-bio], EULAR primary Sjogren's syndrome disease activity, business.industry, RITUXIMAB TREATMENT, medicine.disease, EFFICACY, RHEUMATOID-ARTHRITIS, Clinical trial, 030104 developmental biology, Physical therapy, business, ACCEPTABLE SYMPTOM STATE, Rheumatism, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

International audience; OBJECTIVES: To define disease activity levels, minimal clinically important improvement (MCII) and patient-acceptable symptom state (PASS) with the primary Sjogren's syndrome (SS) disease activity indexes: European League Against Rheumatism (EULAR) SS disease activity index (ESSDAI) and EULAR SS patient-reported index (ESSPRI). METHODS: For 790 patients from two large prospective cohorts, ESSDAI, physician evaluation of disease activity, ESSPRI and patients' satisfaction with their current health status were recorded. Receiver operating characteristic curve analyses and anchoring methods were used to estimate disease activity levels of ESSDAI and the PASS of ESSPRI. At follow-up visit, patients and physicians assessed, respectively, whether symptoms and disease activity have improved or not. An anchoring method based on this evaluation was used to estimate MCII of ESSDAI and ESSPRI. RESULTS: Low-activity (ESSDAI/=14) levels were defined. MCII of ESSDAI was defined as an improvement of at least three points. The PASS estimate was defined as an ESSPRI/=5) and define response to treatment as an improvement of ESSDAI at least three points. For addressing patient-reported outcomes, inclusion of patients with unsatisfactory symptom state (ESSPRI>/=5) and defining response as an improvement of ESSPRI at least one point or 15% seems reasonable.

Published
2014
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11. Defining disease activity states and clinically meaningful improvement in primary Sjögren's syndrome with EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI). in combination wiIh methotrexate

Author

Gseror, R, Bootsma, H, Saraux, A, Bowman, Sj, Theander, E, Brun, Jg, Baron, G, Le Guern, V, Devauchelle Pensec, V, Ramos Casals, M, Valim, V, Dörner, T, Tzioufas, A, Gottenberg, Je, Solans Laqué, R, Mandl, T, Hachulla, E, Sivils, Kl, Ng, Wf, Fauchais, Al, Bombardieri, Stefano, Priori, R, Bartoloni, E, Goeb, V, Praprotnik, S, Sumida, T, Nishiyama, S, Caporali, R, Kruize, Aa, Vollenweider, C, Ravaud, P, Meiners, P, Brito Zerón, P, Vitali, C, and Mariette, X.

Published
2014

12. American College of Rheumatology Classification Criteria for Sjögren’s Syndrome: A Data-Driven, Expert Consensus Approach in the SICCA Cohort

Author

Shiboski, SC, Shiboski, CH, Criswell, LA, Baer, AN, Challacombe, S, Lanfranchi, H, Schiødt, M, Umehara, H, Vivino, F, Zhao, Y, Dong, Y, Greenspan, D, Heidenreich, AM, Helin, P, Kirkham, B, Kitagawa, K, Larkin, G, Li, M, Lietman, T, Lindegaard, J, McNamara, N, Sack, K, Shirlaw, P, Sugai, S, Vollenweider, C, Whitcher, J, Wu, A, Zhang, S, Zhang, W, Greenspan, JS, and Daniels, TE

Subjects
Adult, Aged, 80 and over, Male, Biopsy, Reproducibility of Results, Middle Aged, Sensitivity and Specificity, Article, Salivary Glands, Sialadenitis, United States, Phenotype, Sjogren's Syndrome, Rheumatoid Factor, Antibodies, Antinuclear, Humans, Female, Societies, Medical, Aged
Abstract

We propose new classification criteria for Sjögren's syndrome (SS), which are needed considering the emergence of biologic agents as potential treatments and their associated comorbidity. These criteria target individuals with signs/symptoms suggestive of SS.Criteria are based on expert opinion elicited using the nominal group technique and analyses of data from the Sjögren's International Collaborative Clinical Alliance. Preliminary criteria validation included comparisons with classifications based on the American–European Consensus Group (AECG) criteria, a model-based “gold standard”obtained from latent class analysis (LCA) of data from a range of diagnostic tests, and a comparison with cases and controls collected from sources external to the population used for criteria development.Validation results indicate high levels of sensitivity and specificity for the criteria. Case definition requires at least 2 of the following 3: 1) positive serum anti-SSA and/or anti-SSB or (positive rheumatoid factor and antinuclear antibody titer1:320), 2) ocular staining score3, or 3) presence of focal lymphocytic sialadenitis with a focus score1 focus/4 mm2 in labial salivary gland biopsy samples. Observed agreement with the AECG criteria is high when these are applied using all objective tests. However, AECG classification based on allowable substitutions of symptoms for objective tests results in poor agreement with the proposed and LCA-derived classifications.These classification criteria developed from registry data collected using standardized measures are based on objective tests. Validation indicates improved classification performance relative to existing alternatives, making them more suitable for application in situations where misclassification may present health risks.

Published
2012

13. EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI): Development of a consensus patient index for primary Sjögren's syndrome

Author

Seror, R. Ravaud, P. Mariette, X. Bootsma, H. Theander, E. Hansen, A. Ramos-Casals, M. Dörner, T. Bombardieri, S. Hachulla, E. Brun, J.G. Kruize, A.A. Praprotnik, S. Tomsic, M. Gottenberg, J.-E. Devauchelle, V. Devita, S. Vollenweider, C. Mandl, T. Tzioufas, A. Carsons, S. Saraux, A. Sutcliffe, N. Vitali, C. Bowman, S.J.

Abstract

Objectives To develop a score for assessment of patients' symptoms in primary SjÖgren's syndrome (SS): the EULAR SS Patient Reported Index (ESSPRI). Methods Dryness, pain, somatic and mental fatigue were identified as the main symptoms of patients with primary SS, in studies developing the Profile of Fatigue and Discomfort (PROFAD) and Sicca Symptoms Inventory (SSI). It was suspected that a single 0-10 numerical scale for each domain was sufficient to assess these symptoms. These four scales were gathered to form the ESSPRI. 230 patients, from 12 countries completed the ESSPRI, SSI and PROFAD questionnaires and a 0-10 patient global assessment (PGA). Correlations between each symptom and PGA were obtained. Multiple regression modelling, using PGA as 'gold standard' was used to select domains and estimate their weights. Results PGA had good correlation with dryness, limb pain, fatigue and mental fatigue (r=0.49-0.59, all p

Published
2011

14. OP0089 Big Data Sjögren Project (Eular-SS Task Force International Network): Characterization at Diagnosis of 5027 Patients with Primary Sjögren Syndrome

Author

Brito Zeron, P., primary, Kostov, B.A., additional, Zeher, M., additional, Theander, E., additional, Gottenberg, J.-E., additional, Baldini, C., additional, Quartuccio, L., additional, Priori, R., additional, Kvarnstrom, M., additional, Kruize, A., additional, Hernández Molina, G., additional, Praprotnik, S., additional, Isenberg, D., additional, Bartoloni, E., additional, Rasmussen, A., additional, Solans, R., additional, Valim, V., additional, Giacomelli, R., additional, Carsons, S., additional, Hammenfors, D., additional, Vollenweider, C., additional, Atzeni, F., additional, Mandl, T., additional, De Vita, S., additional, Wahren-Herlenius, M., additional, Sanchez-Guerrero, J., additional, Gerli, R., additional, Sivils, K., additional, Mowa, S., additional, Brun, J.G., additional, Mariette, X., additional, and Ramos-Casals, M., additional

Published
2015
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15. FRI0419 Big Data Sjogren Project (Eular-SS Task Force International Network): Systemic Involvement at Diagnosis Evaluated by the Essdai in 3314 Patients with Primary Sjögren Syndrome

Author

Brito Zeron, P., primary, Kostov, B.A., additional, Seror, R., additional, Baldini, C., additional, Quartuccio, L., additional, Kvarnstrom, M., additional, Kruize, A., additional, Hernández Molina, G., additional, Praprotnik, S., additional, Bartoloni, E., additional, Solans, R., additional, Theander, E., additional, Valim, V., additional, Priori, R., additional, Zeher, M., additional, Isenberg, D., additional, Rasmussen, A., additional, Giacomelli, R., additional, Carsons, S., additional, Hammenfors, D., additional, Vollenweider, C., additional, Atzeni, F., additional, Mandl, T., additional, De Vita, S., additional, Wahren-Herlenius, M., additional, Sanchez-Guerrero, J., additional, Gerli, R., additional, Sivils, K., additional, Mowa, S., additional, Brun, J.G., additional, Mariette, X., additional, and Ramos-Casals, M., additional

Published
2015
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17. FRI0303 Validation of eular primary sjögren’s syndrome disease activity and patient indexes

Author

Seror, R., primary, Theander, E., additional, Brun, J., additional, Ramos-Casals, M., additional, valim, V., additional, Dörner, T., additional, Mariette, X., additional, bootsma, H., additional, Tzioufas, A., additional, Solans Laqué, R., additional, Mandl, T., additional, Gottenberg, J.-E., additional, Hachulla, E., additional, Ng, W.-F., additional, Bombardieri, S., additional, Gerli, R., additional, sumida, T., additional, saraux, A., additional, tomsic, M., additional, caporali, R., additional, Priori, R., additional, Moser, K., additional, Kruize, A. A., additional, Vollenweider, C., additional, Ravaud, P., additional, Praprotnik, S., additional, Scofield, H., additional, Valesini, G., additional, Montecucco, C., additional, Fauchais, A. L., additional, Vitali, C., additional, and Bowman, S., additional

Published
2013
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19. Multipoles of the SLS storage ring: manufacturing and magnetic measurements

Author

Antokhin, E.I., primary, Batrakov, A.M., additional, Churkin, I.N., additional, Demenev, V.V., additional, Dodonov, A.B., additional, Golubenko, O.B., additional, Korchuganov, V.N., additional, Levichev, E.B., additional, Mikhailov, S.F., additional, Ogurtsov, A.B., additional, Philipchenko, A.V., additional, Rivkin, L., additional, Rouvinski, E.S., additional, Rouvinski, S.I., additional, Schreiner, K.K., additional, Sernenov, E.P., additional, Singatulin, S.R., additional, Skorobogatov, D.A., additional, Steshov, A.G., additional, Sukhanov, A.V., additional, Ushakov, V.A., additional, Vollenweider, C., additional, and Zichy, J.A., additional

Published
2002
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20. EULAR Sjogren's Syndrome Patient Reported Index (ESSPRI): development of a consensus patient index for primary Sjogren's syndrome.

Author

Seror R, Ravaud P, Mariette X, Bootsma H, Theander E, Hansen A, Ramos-Casals M, Dörner T, Bombardieri S, Hachulla E, Brun JG, Kruize AA, Praprotnik S, Tomsic M, Gottenberg JE, Devauchelle V, Devita S, Vollenweider C, Mandl T, and Tzioufas A

Abstract

Objectives: To develop a score for assessment of patients' symptoms in primary Sjögren's syndrome (SS): the EULAR SS Patient Reported Index (ESSPRI).Methods: Dryness, pain, somatic and mental fatigue were identified as the main symptoms of patients with primary SS, in studies developing the Profile of Fatigue and Discomfort (PROFAD) and Sicca Symptoms Inventory (SSI). It was suspected that a single 0-10 numerical scale for each domain was sufficient to assess these symptoms. These four scales were gathered to form the ESSPRI. 230 patients, from 12 countries completed the ESSPRI, SSI and PROFAD questionnaires and a 0-10 patient global assessment (PGA). Correlations between each symptom and PGA were obtained. Multiple regression modelling, using PGA as 'gold standard' was used to select domains and estimate their weights.Results: PGA had good correlation with dryness, limb pain, fatigue and mental fatigue (r=0.49-0.59, all p<0.0001), but correlated less well with individual dryness features. In multivariate analysis, dryness, limb pain and fatigue, but not mental fatigue, were significantly associated with PGA; weights derived from the regression were identical for these three domains. Thus, ESSPRI was redefined as the mean of the three scales: dryness, limb pain and fatigue. Lastly, ESSPRI significantly correlated with PGA (r=0.70), PROFAD (r=0.73) and SSI (r=0.66).Conclusion: ESSPRI is a very simple index designed to measure patients' symptoms in primary SS. It has good construct validity and is well correlated with SSI and PROFAD. ESSPRI should now be validated for use as an outcome measure in clinical trials. [ABSTRACT FROM AUTHOR]

Published
2011
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21. American College of Rheumatology classification criteria for Sjögren's syndrome: A data‐driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance Cohort

Author

Shiboski, S. C., Shiboski, C. H., Criswell, L. A., Baer, A. N., Challacombe, S., Lanfranchi, H., Schiødt, M., Umehara, H., Vivino, F., Zhao, Y., Dong, Y., Greenspan, D., Heidenreich, A. M., Helin, P., Kirkham, B., Kitagawa, K., Larkin, G., Li, M., Lietman, T., Lindegaard, J., McNamara, N., Sack, K., Shirlaw, P., Sugai, S., Vollenweider, C., Whitcher, J., Wu, A., Zhang, S., Zhang, W., Greenspan, J. S., and Daniels, T. E.

Published
2012
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24. Influence of the age at diagnosis in the disease expression of primary Sjögren syndrome. Analysis of 12,753 patients from the Sjögren Big Data Consortium.

Author

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, Fernandes Moça Trevisani V, 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, and Brito-Zerón P

Subjects
Big Data, Humans, Sjogren's Syndrome diagnosis, Sjogren's Syndrome epidemiology
Abstract

Objectives: 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 0.18%), 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)., Conclusions: 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
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25. Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjögren's syndrome.

Author

Brito-Zerón P, Acar-Denizli N, Ng WF, Horváth IF, Rasmussen A, Seror R, Li X, Baldini C, Gottenberg JE, Danda D, Quartuccio L, Priori R, Hernandez-Molina G, Armagan B, Kruize AA, Kwok SK, Kvarnstrom M, Praprotnik S, Sene D, Gerli R, Solans R, Rischmueller M, Mandl T, Suzuki Y, Isenberg D, Valim V, Wiland P, Nordmark G, Fraile G, Bootsma H, Nakamura H, Giacomelli R, Devauchelle-Pensec V, Hofauer B, Bombardieri M, Trevisani VFM, Hammenfors D, Pasoto SG, Retamozo S, Gheita TA, Atzeni F, Morel J, Vollenweider C, Zeher M, Sivils K, Xu B, Bombardieri S, Sandhya P, De Vita S, Minniti A, Sánchez-Guerrero J, Kilic L, van der Heijden E, Park SH, Wahren-Herlenius M, Mariette X, and Ramos-Casals M

Subjects
Black or African American statistics & numerical data, Asian People statistics & numerical data, Cohort Studies, Female, Hispanic or Latino statistics & numerical data, Humans, Information Dissemination, Male, Middle Aged, Phenotype, Registries, Severity of Illness Index, Sjogren's Syndrome ethnology, White People statistics & numerical data, Ethnicity statistics & numerical data, Racial Groups statistics & numerical data, Sjogren's Syndrome epidemiology
Abstract

Objective: To characterize the systemic phenotype of primary Sjögren's syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores., Methods: The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren's syndrome from the five continents., Results: The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P < 0.001) compared with females, as did patients diagnosed at <35 years (6.7 vs 5.6 in patients diagnosed at >65 years, P < 0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P < 0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P < 0.001)., Conclusion: The systemic phenotype of primary Sjögren's syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis., (© The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Published
2020
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26. Defining disease activity states and clinically meaningful improvement in primary Sjögren's syndrome with EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI).

Author

Seror R, Bootsma H, Saraux A, Bowman SJ, Theander E, Brun JG, Baron G, Le Guern V, Devauchelle-Pensec V, Ramos-Casals M, Valim V, Dörner T, Tzioufas A, Gottenberg JE, Solans Laqué R, Mandl T, Hachulla E, Sivils KL, Ng WF, Fauchais AL, Bombardieri S, Priori R, Bartoloni E, Goeb V, Praprotnik S, Sumida T, Nishiyama S, Caporali R, Kruize AA, Vollenweider C, Ravaud P, Meiners P, Brito-Zerón P, Vitali C, and Mariette X

Subjects
Aged, Diagnostic Self Evaluation, Disease Progression, Female, Humans, Male, Middle Aged, Patient Satisfaction, Prospective Studies, ROC Curve, Sjogren's Syndrome psychology, Symptom Assessment psychology, Health Status, Severity of Illness Index, Sjogren's Syndrome diagnosis, Symptom Assessment methods
Abstract

Objectives: To define disease activity levels, minimal clinically important improvement (MCII) and patient-acceptable symptom state (PASS) with the primary Sjögren's syndrome (SS) disease activity indexes: European League Against Rheumatism (EULAR) SS disease activity index (ESSDAI) and EULAR SS patient-reported index (ESSPRI)., Methods: For 790 patients from two large prospective cohorts, ESSDAI, physician evaluation of disease activity, ESSPRI and patients' satisfaction with their current health status were recorded. Receiver operating characteristic curve analyses and anchoring methods were used to estimate disease activity levels of ESSDAI and the PASS of ESSPRI. At follow-up visit, patients and physicians assessed, respectively, whether symptoms and disease activity have improved or not. An anchoring method based on this evaluation was used to estimate MCII of ESSDAI and ESSPRI., Results: Low-activity (ESSDAI<5), moderate-activity (5≤ESSDAI≤13) and high-activity (ESSDAI≥14) levels were defined. MCII of ESSDAI was defined as an improvement of at least three points. The PASS estimate was defined as an ESSPRI<5 points and MCII as a decrease of at least one point or 15%., Conclusions: This study determined disease activity levels, PASS and MCII of ESSDAI and ESSPRI. These results will help designing future clinical trials in SS. For evaluating systemic complications, the proposal is to include patients with moderate activity (ESSDAI≥5) and define response to treatment as an improvement of ESSDAI at least three points. For addressing patient-reported outcomes, inclusion of patients with unsatisfactory symptom state (ESSPRI≥5) and defining response as an improvement of ESSPRI at least one point or 15% seems reasonable., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

Published
2016
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27. Validation of EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient indexes (ESSPRI).

Author

Seror R, Theander E, Brun JG, Ramos-Casals M, Valim V, Dörner T, Bootsma H, Tzioufas A, Solans-Laqué R, Mandl T, Gottenberg JE, Hachulla E, Sivils KL, Ng WF, Fauchais AL, Bombardieri S, Valesini G, Bartoloni E, Saraux A, Tomsic M, Sumida T, Nishiyama S, Caporali R, Kruize AA, Vollenweider C, Ravaud P, Vitali C, Mariette X, and Bowman SJ

Subjects
Adult, Aged, Europe, Fatigue diagnosis, Fatigue etiology, Female, Humans, Male, Middle Aged, Pain diagnosis, Pain etiology, Prospective Studies, Reproducibility of Results, Severity of Illness Index, Sjogren's Syndrome complications, Sjogren's Syndrome diagnosis, Xerophthalmia diagnosis, Xerophthalmia etiology, Xerostomia diagnosis, Xerostomia etiology, Fatigue physiopathology, Pain physiopathology, Self Report, Sjogren's Syndrome physiopathology, Xerophthalmia physiopathology, Xerostomia physiopathology
Abstract

Objectives: To validate the two recently developed disease activity indexes for assessment of primary Sjögren's syndrome (SS): the European League Against Rheumatism (EULAR) SS Patient Reported Index (ESSPRI) and the EULAR SS Disease Activity Index (ESSDAI)., Methods: A prospective international 6-month duration validation study was conducted in 15 countries. At each visit, physicians completed ESSDAI, SS disease activity index (SSDAI), Sjögren's Systemic Clinical Activity Index (SCAI) and physician global assessment (PhGA); and patients completed ESSPRI, Sicca Symptoms Inventory (SSI), Profile of Fatigue and Discomfort (PROFAD) and patient global assessment (PGA). Psychometric properties (construct validity, responsiveness and reliability) were evaluated and compared between scores., Results: Of the 395 patients included, 145 (37%) and 251 (64%) had currently active or current or past systemic manifestations, respectively. EULAR scores had higher correlation with the gold standard than other scores (ESSDAI with PhGA: r=0.59; ESSRPI with PGA: r=0.70). Correlations between patient and systemic scores were very low (ranging from 0.07 to 0.29). All systemic scores had similar large responsiveness in improved patients. Responsiveness of patient scores was low but was significantly higher for ESSPRI compared with SSI and PROFAD. Reliability was very good for all scores., Conclusions: ESSDAI and ESSPRI had good construct validity. All scores were reliable. Systemic scores had a large sensitivity to change in patients whose disease activity improves. Patient scores had a small sensitivity to change, however, significantly better for ESSPRI. Systemic and patient scores poorly correlated, suggesting that they are 2 complementary components that should be both evaluated, but separately., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Published
2015
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28. EULAR Sjögren's syndrome disease activity index (ESSDAI): a user guide.

Author

Seror R, Bowman SJ, Brito-Zeron P, Theander E, Bootsma H, Tzioufas A, Gottenberg JE, Ramos-Casals M, Dörner T, Ravaud P, Vitali C, Mariette X, Asmussen K, Jacobsen S, Bartoloni E, Gerli R, Bijlsma JW, Kruize AA, Bombardieri S, Bookman A, Kallenberg C, Meiners P, Brun JG, Jonsson R, Caporali R, Carsons S, De Vita S, Del Papa N, Devauchelle V, Saraux A, Fauchais AL, Sibilia J, Hachulla E, Illei G, Isenberg D, Jones A, Manoussakis M, Mandl T, Jacobsson L, Demoulins F, Montecucco C, Ng WF, Nishiyama S, Omdal R, Parke A, Praprotnik S, Tomsic M, Price E, Scofield H, L Sivils K, Smolen J, Laqué RS, Steinfeld S, Sutcliffe N, Sumida T, Valesini G, Valim V, Vivino FB, and Vollenweider C

Abstract

The EULAR Sjögren's syndrome (SS) disease activity index (ESSDAI) is a systemic disease activity index that was designed to measure disease activity in patients with primary SS. With the growing use of the ESSDAI, some domains appear to be more challenging to rate than others. The ESSDAI is now in use as a gold standard to measure disease activity in clinical studies, and as an outcome measure, even a primary outcome measure, in current randomised clinical trials. Therefore, ensuring an accurate and reproducible rating of each domain, by providing a more detailed definition of each domain, has emerged as an urgent need. The purpose of the present article is to provide a user guide for the ESSDAI. This guide provides definitions and precisions on the rating of each domain. It also includes some minor improvement of the score to integrate advance in knowledge of disease manifestations. This user guide may help clinicians to use the ESSDAI, and increase the reliability of rating and consequently of the ability to detect true changes over time. This better appraisal of ESSDAI items, along with the recent definition of disease activity levels and minimal clinically important change, will improve the assessment of patients with primary SS and facilitate the demonstration of effectiveness of treatment for patients with primary SS.

Published
2015
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