Big Data International Primary Sjögren Syndrome Registry: Baseline Characterization and Diagnostic Approach in 6047 Patients Fulfilling the 2002 AE Criteria

Pilar Brito-Zerón¹, Soledad Retamozo^1,2, Margit Zeher³, Astrid Rasmussen⁴, Elke Theander⁵, Jacques Gottenberg⁶, Chiara Baldini⁷, Luca Quartuccio⁸, Roberta Priori⁹, Valeria Valim¹⁰, Marika Kvarnström¹¹, Aike Kruize¹², Gabriela Hernandez-Molina¹³, Elena Bartoloni-Bocci¹⁴, Sonja Praprotnik¹⁵, David A. Isenberg¹⁶, Gunnel Nordmark¹⁷, Michele Bombardieri¹⁸, Yasunori Suzuki¹⁹, Roser Solans²⁰, Roberto Giacomelli²¹, Daniel S. Hammenfors²², Steven E. Carsons²³, Hendrika Boostma²⁴, Cristina F. Vollenweider²⁵, Fabiola Atzeni²⁶, Kathy Sivils²⁷, Thomas Mandl²⁸, Salvatore De Vita²⁹, Marie Wahren-Herlenius¹¹, Mitsuhiro Kawano³⁰, Roberto Gerli³¹, Arjan Vissink³², Johan G. Brun³³, Virginia Trevisani³⁴, Jorge Sánchez-Guerrero³⁵, Xavier Mariette³⁶, Manuel Ramos-Casals¹ and on behalf of the EULAR-SS Task Force, ¹Department of Autoimmune Diseases, CELLEX-IDIBAPS, Hospital Clínic, Barcelona, Barcelona, Spain, ²Rheumatology Unit, Hospital Privado Centro Médico de Córdoba, Córdoba, Argentina, ³University of Debrecen, Debrecen, Hungary, ⁴Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, ⁵Skåne University Hospital Malmö, Lund University, Malmo, Sweden, ⁶Hôpitaux Universitaires de Strasbourg, Strasbourg, France, ⁷Rheumatology Unit, University of Pisa, Italy, Pisa, Italy, ⁸Clinic of Rheumatology, Department of Medical and Biological Sciences (DSMB), Santa Maria della Misericordia Hospital, University of Udine, Udine, Italy, ⁹Rheumatology Unit, Sapienza University of Rome, Rome, Italy, ¹⁰Rheumatology, Universidade Federal do Espírito Santo, Vitória, Brazil, ¹¹Department of medicine, Rheumatology unit, Karolinska Institutet, Stockholm, Sweden, ¹²Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ¹³Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico city, Mexico, ¹⁴Department of Medicine, Rheumatology Unit, University of Perugia, Perugia, Italy, ¹⁵Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia, ¹⁶Rayne Institute, Centre for Rheumatology Research, UCL Division of Medicine, London, United Kingdom, ¹⁷Medical Sciences, Uppsala University, Uppsala, Sweden, ¹⁸Experimental Medicine and Rheumatology, Queen Mary University of London, London, United Kingdom, ¹⁹Kanazawa University Graduate School of Medicine, Kanazawa, Japan, ²⁰Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, ²¹University of L'Aquila, L'Aquila, Italy, ²²Department of Rheumatology, Haukeland University Hospital, Bergen, Norway, ²³Rheumatology, Allergy and Immunology, Winthrop University Hospital, Mineola, NY, ²⁴University of Groningen, University Medical Center Groningen, Groningen, Netherlands, ²⁵Rheumatology, German Hospital, Buenos Aires, Argentina, ²⁶Rheumatology Unit, L. Sacco University Hospital of Milan, Milan, Italy, ²⁷Oklahoma Medical Research Foundation, Oklahoma City, OK, ²⁸Dept of Rheumatology, Skåne University Hospital Malmö, Lund University, Malmo, Sweden, ²⁹Santa Maria della Misericordia Hospital, University of Udine, Udine, Italy, ³⁰Division of Rheumatology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan, ³¹University of Perugia, Perugia, Italy, ³²Department of Oral and Maxillofacial Surgery, University of Groningen, University Medical Center Groningen, Groningen, Netherlands, ³³Haukeland University Hospital, Bergen, Norway, ³⁴Health Evidence Based, Universidade Federal de São Paulo, Sâo Paulo, Brazil, ³⁵Rheumatology, Mount Sinai Hospital and University Health Network, Toronto Canada, Toronto, ON, Canada, ³⁶Université Paris-Sud, AP-HP, Hôpitaux Universitaires Paris-Sud, Paris, France

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: big data and diagnostic criteria, Sjogren's syndrome

Session Information

Date: Sunday, November 8, 2015

Title: Sjögren's Syndrome Poster I: Clinical Insights into Sjögren's Syndrome

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose:

To analyse the epidemiological, clinical and immunological characteristics at diagnosis of the largest international cohort of patients diagnosed with primary Sjögren syndrome (SS) according to the 2002 AE classification criteria.

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. International experts participating in the EULAR-SS Task Force were invited to participate. By June 2015, the database included 6120 consecutive patients recruited from 14 countries (Europe, America and Japan), of whom 6047 fulfilled the 2002 classification criteria for primary SS (some diagnostic tests are pending in the remaining patients). The main features at diagnosis (time of criteria fulfilment) or at recruitment were collected and analysed.

Results:

Of the 6047 patients, 5648 (93.4%) were women and 399 (6.6%) were men (female:male ratio, 14:1), with a mean age at diagnosis of primary SS of 49.96 years (range, 5-97), of which 79% were Caucasian and 81% from European countries. The frequency of fulfilment of the 2002 criteria was: 94.1% for dry eye, 93.6% for dry mouth, 88.3% for positive salivary gland biopsy, 86% for positive ocular tests, 79.1% for positive oral tests and 72.2% for positive Ro/La autoantibodies. With respect to the criteria fulfilled, 10.6% fulfilled 3 criteria, 30.6% 4 criteria, 40.7% 5 criteria and 18% all 6 criteria. The percentage of diagnostic tests performed varied from 70% to nearly 100%: Ro/La autoantibodies were tested in 99.5% of patients, ocular diagnostic tests (Schirmer’s test and/or corneal stainings) in 89.7%, oral tests in 81.3% and salivary gland biopsy in 71.8% of patients. The four diagnostic tests included in the 2002 AE criteria were made (complete diagnostic approach) in 3459 (57%) patients, while in the remaining 2588 (43%), only two or three tests were made (incomplete diagnostic approach). A complete diagnostic approach was more frequent in patients from non-European countries (73% vs 55%, p<0.001), Ro/La negative patients (79% vs 50%, p<0.001), ANA-negative patients (75% vs 57%, p<0.001), RF-negative patients (66% vs 55%, p<0.001) and patients with normal C3/C4 values (74% vs 59%, p<0.001).

Conclusion:

In the largest cohort of primary SS patients diagnosed homogeneously according to the 2002 AE criteria, a full diagnostic evaluation was made in less than 60% of patients. In addition, the diagnostic approach was heterogeneous and varied according to the geographical area and the baseline immunological profile; in contrast, gender and age did not influence the number of diagnostic tests made to achieve fulfilment of the criteria.

Disclosure: P. Brito-Zerón, None; S. Retamozo, None; M. Zeher, None; A. Rasmussen, None; E. Theander, Roche, 5; J. Gottenberg, None; C. Baldini, None; L. Quartuccio, None; R. Priori, None; V. Valim, None; M. Kvarnström, None; A. Kruize, None; G. Hernandez-Molina, None; E. Bartoloni-Bocci, None; S. Praprotnik, None; D. A. Isenberg, Bristol-Myers Squibb, 2; G. Nordmark, None; M. Bombardieri, None; Y. Suzuki, None; R. Solans, None; R. Giacomelli, None; D. S. Hammenfors, None; S. E. Carsons, None; H. Boostma, None; C. F. Vollenweider, None; F. Atzeni, None; K. Sivils, None; T. Mandl, None; S. De Vita, None; M. Wahren-Herlenius, None; M. Kawano, None; R. Gerli, None; A. Vissink, None; J. G. Brun, None; V. Trevisani, None; J. Sánchez-Guerrero, None; X. Mariette, None; M. Ramos-Casals, Bristol-Myers Squibb, 2.

To cite this abstract in AMA style:

Brito-Zerón P, Retamozo S, Zeher M, Rasmussen A, Theander E, Gottenberg J, Baldini C, Quartuccio L, Priori R, Valim V, Kvarnström M, Kruize A, Hernandez-Molina G, Bartoloni-Bocci E, Praprotnik S, Isenberg DA, Nordmark G, Bombardieri M, Suzuki Y, Solans R, Giacomelli R, Hammenfors DS, Carsons SE, Boostma H, Vollenweider CF, Atzeni F, Sivils K, Mandl T, De Vita S, Wahren-Herlenius M, Kawano M, Gerli R, Vissink A, Brun JG, Trevisani V, Sánchez-Guerrero J, Mariette X, Ramos-Casals M. Big Data International Primary Sjögren Syndrome Registry: Baseline Characterization and Diagnostic Approach in 6047 Patients Fulfilling the 2002 AE Criteria [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/big-data-international-primary-sjogren-syndrome-registry-baseline-characterization-and-diagnostic-approach-in-6047-patients-fulfilling-the-2002-ae-criteria/. Accessed .

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