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

Lookup NU author(s): Professor Fai NgORCiD


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© Copyright CLINICAL AND EXPERIMENTAL RHEUMATOLOGY 2018. Objective. To evaluate the influence of the main immunological markers on the disease phenotype at diagnosis in a large international cohort of patients with primary Sjögren's syndrome (SjS). Methods. The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. As a first step, baseline clinical information from leading centres on clinical research in SjS of the 5 continents was collected. The centres shared a harmonised data architecture and conducted cooperative online efforts in order to refine collected data under the coordination of a big data statistical team. Inclusion criteria were the fulfillment of the 2002 classification criteria. Immunological tests were carried out using standard commercial assays. Results. By January 2018, the participant centres had included 10,500 valid patients from 22 countries. The cohort included 9,806 (93%) women and 694 (7%) men, with a mean age at diagnosis of primary SjS of 53 years, mainly White (78%) and included from European countries (71%). The frequency of positive immunological markers at diagnosis was 79.3% for ANA, 73.2% for anti-Ro, 48.6% for RF, 45.1% for anti- La, 13.4% for low C3 levels, 14.5% for low C4 levels and 7.3% for cryoglobulins. Positive autoantibodies (ANA, Ro, La) correlated with a positive result in salivary gland biopsy, while hypocomplementaemia and especially cryoglobulinaemia correlated with systemic activity (mean ESSDAI score of 17.7 for cryoglobulins, 11.3 for low C3 and 9.2 for low C4, in comparison with 3.8 for negative markers). The immunological markers with a great number of statistically-significant associations (p<0.001) in the organ-by-organ ESSDAI evaluation were cryoglobulins (9 domains), low C3 (8 domains), anti-La (7 domains) and low C4 (6 domains). Conclusion. We confirm the strong influence of immunological markers on the phenotype of primary SjS at diagnosis in the largest multi-ethnic international cohort ever analysed, with a greater influence for cryoglobulinaemic- related markers in comparison with Ro/La autoantibodies and ANA. Immunological patterns play a central role in the phenotypic expression of the disease already at the time of diagnosis, and may guide physicians to design a specific personalised management during the follow-up of patients with primary SjS.

Publication metadata

Author(s): Brito-Zeron P, Acar-Denizli N, Ng WF, Zeher M, Rasmussen A, Mandl T, Seror R, Li X, Baldini C, Gottenberg J-E, Danda D, Quartuccio L, Priori R, Hernandez-Molina G, Armagan B, Kruize AA, Kwok S-K, Kvarnstrom M, Praprotnik S, Sene D, Bartoloni E, Solans R, Rischmueller M, Suzuki Y, Isenberg DA, Valim V, Wiland P, Nordmark G, Fraile G, Bootsma H, Nakamura T, Giacomelli R, Devauchelle-Pensec V, Knopf A, Bombardieri M, Trevisani V-F, Hammenfors D, Pasoto SG, Retamozo S, Gheita TA, Atzeni F, Morel J, Vollenveider C, Horvath I-F, Sivils KL, Olsson P, De Vita S, Sanchez-Guerrero J, Kilic L, Wahren-Herlenius M, Mariette X, Ramos-Casals M, Brito-Zeron P, Morcillo C, Brito-Zeron P, Flores-Chavez A, Ramos-Casals M, Acar-Denizli N, Ng WF, Zeher M, Horvath I-F, Rasmussen A, Sivils K, Scofield H, Seror R, Mariette X, Mandl T, Olsson P, Li X, Baldini C, Gottenberg JE, Danda D, Sandhya P, Quartuccio L, Corazza L, De Vita S, Priori R, Hernandez-Molina G, Sanchez-Guerrero J, Kruize AA, Van Der Heijden E, Valim V, Kvarnstrom M, Wahren-Herlenius M, Sene D, Gerli R, Bartoloni E, Praprotnik S, Isenberg DA, Solans R, Rischmueller M, Downie-Doyle S, Kwok S-K, Park S-H, Nordmark G, Suzuki Y, Kawano M, Giacomelli R, Carubbi F, Devauchelle-Pensec V, Saraux A, Bombardieri M, Astorri E, Hofauer B, Knopf A, Bootsma H, Vissink A, Brun JG, Hammenfors D, Fraile G, Carsons SE, Gheita TA, Khalil HM, Morel J, Vollenveider C, Atzeni F, Retamozo S, Moca Trevisano V, Armagan B, Kilic L, Nakamura T, Sebastian A, Wiland P, Pasoto S, Kostov B, Siso-Almirall A, Sibilia J, Miceli-Richard C, Nocturne G, Benessiano J, Dieude P, Dubost J-J, Fauchais A-L, Goeb V, Hachulla E, Hatron PY, Larroche C, Le Guern V, Puechal X, Morel J, Perdriger A, Rist S, Vittecoq O, Ravaud P, Diaz-Lopez B, Feijoo C, Pallares L, Lopez-Dupla M, Perez-Alvarez R, Ripoll M, Pinilla B, Akasbi M, Maure B, Fonseca E, Canora J, De La Red G, Chamorro AJ, Jimenez-Heredia I, Fanlo P, Guisado-Vasco P, Zamora M

Publication type: Article

Publication status: Published

Journal: Clinical and Experimental Rheumatology

Year: 2018

Volume: 36

Pages: S102-S111

Online publication date: 14/08/2018

Acceptance date: 17/05/2018

ISSN (print): 0392-856X

ISSN (electronic): 1593-098X

Publisher: Clinical and Experimental Rheumatology S.A.S.


PubMed id: 30156539