ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 876

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

Soledad Retamozo1,2,3, Pilar Brito-Zerón3,4, Margit Zeher5, Kathy L. Sivils6, Raphaele Seror7, Thomas Mandl8, Xiaomei Li9, Chiara Baldini10, Jacques-Eric Gottenberg11, Debashish Danda12, Roberta Priori13, Luca Quartuccio14, Gabriela Hernandez-Molina15, Aike A. Kruize16, Seung-Ki Kwok17, Marie Wahren-Herlenius18, Sonja Praprotnik19, Damien Sene20, Roberto Gerli21, Roser Solans22, Yasunori Suzuki23, David A. Isenberg24, Maureen Rischmueller25, Gunnel Nordmark26, Guadalupe Fraile27, Piotr Wiland28, Hendrika Bootsma29, Takashi Nakamura30, Valeria Valim31, Roberto Giacomelli32, Valérie Devauchelle-Pensec33, Benedikt Hofauer34, Michele Bombardieri35, Virginia Fernandes Moça Trevisani36, Daniel S. Hammenfors37, Steven E. Carsons38, Sandra Gofinet Pasoto39, Jacques Morel40, Tamer Gheita41, Fabiola Atzeni42, Cristina F. Vollenweider43, Belchin Kostov44, Xavier Mariette45 and Manuel Ramos-Casals46, 1Rheumatology Unit, Hospital Privado Universitario de Córdoba, Institute University of Biomedical Sciences University of Córdoba (IUCBC), Cordoba, Argentina, 2Instituto de Investigaciones en Ciencias de la Salud, Universidad Nacional de Córdoba, Consejo Nacional de Investigaciones Científicas y Técnicas (INICSA-UNC-CONICET), Cordoba, Argentina, 3Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Barcelona, Spain, 4Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona., Bacelona, Spain, 5Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary., Debrecen, Hungary, 6Arthritis and Clinical Immunology Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, 7Center for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique – Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicêtre, Université Paris Sud, INSERM, Paris, France, Paris, France, 8Department of Rheumatology, Skåne University Hospital, Malmö, Sweden, Lund, Sweden, 9Department of Rheumatology and Immunology, Anhui Medical University Affiliated Provincial Hospital, China, Hefei, Anhui, China, 10Internal Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy, 11Department of Rheumatology, Strasbourg University Hospital, Université de Strasbourg, CNRS, Strasbourg, France, Strasbourg, France, 12Clinical Immunology & Rheumatology, Christian Medical College, Vellore, India, Vellore, India, 13UO Complessa Reumatologia, Policlinico Umberto I Università Sapienza di Roma, Rome, Italy, 14Rheumatology Clinic, DSMB, University of Udine, Udine, Italy, Udine, Italy, 15Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición SZ, mexico city, Mexico, 16Rheumatology & Clinical Immunology, University Medical Center Utrecht, Utrecht, Netherlands, 17[email protected], Division of Rheumatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea, Republic of (South), 18Unit of Experimental Rheumatology, Department of Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, Stockholm, Sweden, 19Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia, 20Service de Médecine Interne 2, Hôpital Lariboisière, Université Paris VII, Assistance Publique-Hôpitaux de Paris, 2, Paris, France, Paris, France, 21University and Azienda Ospedaliera of Perugia, Perugia, Italy, 22Autoimmune Systemic Diseases Unit, Department of Internal Medicine, Hospital Vall d'Hebron, Autonomous University of Barcelona, Spain, Barcelona, Spain, 23Division of Rheumatology, Kanazawa University Graduate School of Medicine, Ishikawa, Japan, Kanazawa, Japan, 24Centre for Rheumatology Research, Division of Medicine, University College London, London, United Kingdom, 25Rheumatology, The Queen Elizabeth Hospital, South Australia, Adelaide, Australia, 26Department of Medical Sciences, Section of Rheumatology, Uppsala University, Uppsala, Sweden, Uppsala, Sweden, 27Autoimmune Diseases Department, Hospital Ramón y Cajal, Madrid, Spain, 28Department and Clinic of Rheumatology and Internal Medicine, Medical University, Wroclaw, Poland, 29Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands, 30Department of Radiology and Cancer Biology, Nagasaki University School of Dentistry, Nagasaki, Japan, 31Rheumatology, Department of Medicine, Universidade Federal do Espírito Santo, Vitória, Brazil, Vitória, Brazil, 32University of L'Aquila, L'Aquila, Italy, 33Department of Rheumatology, Brest University Hospital, Brest, France, 34Hals-Nasen-Ohrenklinik und Poliklinik, Technische Universität München, München, Germany, München, Germany, 35Centre for Experimental Medicine and Rheumatology, William Harvey Research Institute, Queen Mary University of London, UK, London, United Kingdom, 36UNIFESP, Sao Paulo, Brazil, San Paulo, Brazil, 37Department of Rheumatology, Haukeland University Hospital, University of Bergen, Bergen, Norway, 38NYU Winthrop University Hospital, Department of Medicine, Mineola, NY, 39Internal Medicine, Division of Rheumatology - Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, São Paulo, Brazil, 40Department of Rheumatology, Teaching hospital and University of Montpellier, France, Montpellier, France, 41Rheumatology, Rheumatology Department, Faculty of Medicine, Cairo University, Egypt, Cairo, Egypt, 42Rheumatology Unit, ASST Fatebenefratelli - Sacco, L. Sacco University Hospital, Milano, Italy, 43Rheumatology, German Hospital, Buenos Aires, Argentina, Buenos Aires, Argentina, 44Primary Care Research Group, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Primary Care Centre Les Corts, CAPSBE, Barcelona, Spain, 45Université Paris-Sud, AP-HP, Hôpitaux Universitaires Paris-Sud, Paris, France, 46Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Spain, Barcelona, Spain

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: , ,

Session Information

Date: Sunday, November 5, 2017

Title: Sjögren's Syndrome I: Clinical Assessment and Trial Outcomes

Session Type: ACR Concurrent Abstract Session

Session Time: 2:30PM-4:00PM

Background/Purpose: To analyse whether epidemiologic factors (such as gender or age at diagnosis of the disease) are associated with particular disease expressions and define some specific subsets in patients with primary Sjögren syndrome (SS).

Methods: The Big Data Sjögren project is an international, multicentre registry formed in 2014 to take a “high-definition” picture of the main features of primary SS at diagnosis by merging international SS databases using a Data-Sharing methodological approach. By January 2017, the database included 9302 consecutive patients recruited from 21 countries of the five continents. The main features at diagnosis (time of criteria fulfilment) or at recruitment were collected and analysed.

Results: Of the 9032 patients, 8680 (93%) were women and 622 (7%) were men with a mean age at diagnosis of primary SS of 50 years; 76% were Caucasian. The frequency of fulfilment of the 2002 criteria was: 92% for dry eye, 93% for dry mouth, 88% for positive salivary gland biopsy, 93% for positive ocular tests, 85% for positive oral tests and 71% for positive Ro/La autoantibodies. Other immunological tests included positive ANA (81%), RF (49%), low C4 levels (13%), low C3 levels (14%) and cryoglobulins (7%). Men with primary SS presented a higher frequency of White ethnicity (83% vs 76% in women, p < 0.001) and rheumatoid factor (54% vs 49%, p = 0.017), and a lower frequency of dry eyes (89% vs 92%, p = 0.011) and dry mouth (90% vs 94%, p = 0.026) in the multivariate model analysis. Patients with a younger onset (< 35 years) showed a lower frequency of White ethnicity (69% vs 77% in aged > 35 yrs, p < 0.001), dry eyes (86% vs 93%, p < 0.001) and positive ocular tests (81% vs 85%, p = 0.001), and a higher frequency of anti-Ro/La autoantibodies (84% vs 70%, p < 0.001), ANA (89% vs 80%, p < 0.001), RF (62% vs 47%, p < 0.001) and low C3 levels (19% vs 13%, p < 0.001) in the multivariate model analysis. Patients with an elderly onset (> 70 years) showed a higher frequency of White ethnicity (85% vs 75% in aged < 70 yrs, p < 0.001), positive oral tests (82% vs 76%, p = 0.003) and a lower frequency of anti-Ro/La autoantibodies (62% vs 72%, p < 0.001) and low C3 levels (9% vs 14%, p < 0.001) in the multivariate model analysis.

Conclusion: In the largest reported cohort of primary SS patients diagnosed homogeneously around the world according to the 2002 AE criteria, we found that primary SS is a disease that can be presented heterogeneously at diagnosis, depending on specific epidemiologic features such as gender, age and ethnicity.

Disclosure: S. Retamozo, None; P. Brito-Zerón, None; M. Zeher, None; K. L. Sivils, None; R. Seror, None; T. Mandl, None; X. Li, None; C. Baldini, None; J. E. Gottenberg, None; D. Danda, None; R. Priori, None; L. Quartuccio, None; G. Hernandez-Molina, None; A. A. Kruize, None; S. K. Kwok, None; M. Wahren-Herlenius, None; S. Praprotnik, None; D. Sene, None; R. Gerli, None; R. Solans, None; Y. Suzuki, None; D. A. Isenberg, EMD Serono, Inc, 5; M. Rischmueller, None; G. Nordmark, None; G. Fraile, None; P. Wiland, Celltrion Inc., 2; H. Bootsma, None; T. Nakamura, None; V. Valim, None; R. Giacomelli, None; V. Devauchelle-Pensec, Roche-Chugai provided me tocilizumab for the SEMAPHORER study, 2; B. Hofauer, None; M. Bombardieri, GSK, Amgen/MedImmune and UCB, 5; V. Fernandes Moça Trevisani, None; D. S. Hammenfors, None; S. E. Carsons, None; S. G. Pasoto, None; J. Morel, None; T. Gheita, None; F. Atzeni, None; C. F. Vollenweider, None; B. Kostov, None; X. Mariette, None; M. Ramos-Casals, None.

To cite this abstract in AMA style:

Retamozo S, Brito-Zerón P, Zeher M, Sivils KL, Seror R, Mandl T, Li X, Baldini C, Gottenberg JE, Danda D, Priori R, Quartuccio L, Hernandez-Molina G, Kruize AA, Kwok SK, Wahren-Herlenius M, Praprotnik S, Sene D, Gerli R, Solans R, Suzuki Y, Isenberg DA, Rischmueller M, Nordmark G, Fraile G, Wiland P, Bootsma H, Nakamura T, Valim V, Giacomelli R, Devauchelle-Pensec V, Hofauer B, Bombardieri M, Fernandes Moça Trevisani V, Hammenfors DS, Carsons SE, Pasoto SG, Morel J, Gheita T, Atzeni F, Vollenweider CF, Kostov B, Mariette X, Ramos-Casals M. Epidemiologic Subsets Drive a Differentiated Clinical and Immunological Presentation of Primary Sjögren Syndrome: Analysis of 9302 Patients from the Big Data International Sjögren Cohort [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/epidemiologic-subsets-drive-a-differentiated-clinical-and-immunological-presentation-of-primary-sjogren-syndrome-analysis-of-9302-patients-from-the-big-data-international-sjogren-cohort/. Accessed .

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