Influence of epidemiology and ethnicity on systemic expression of primary sjÖgren syndrome in 9974 patients
S Retamozo1,2,3, N. Acar-Denizli4, W. Fai Ng5, M. Zeher6, A. Rasmussen7, R. Seror8, X. Li9, C. Baldini10, J.-E. Gottenberg11, D. Danda12, L. Quartuccio13, R. Priori14, G. Hernandez-Molina15, B. Armagan16, A.A. Kruize17, S.-K. Kwok18, M. Wahren-Herlenius19, S. Praprotnik20, D. Sene21, E. Bartoloni22, R. Solans23, M. Rischmueller24, T. Mandl25, Y. Suzuki26, D. Isenberg27, V. Valim28, P. Wiland29, G. Nordmark30, G. Fraile31, H. Bootsma32, T. Nakamura33, R. Giacomelli34, V. Devauchelle-Pensec35, B. Hofauer36, M. Bombardieri37, V. Fernandes Moça Trevisani38, D. Hammenfors39, S.G. Pasoto40, T.A. Gheita41, F. Atzeni42, J. Morel43, C. Vollenveider44, P. Brito-Zerón45, M. Ramos-Casals1,45,1, on behalf of the Sjogren Big Data Consortium
1H. Clinic, IDIBAPS, Barcelona, Spain 2H. Privado Cordoba, IUCBC 3INICSA, UNC-CONICET, Cordoba, Argentina 4Mimar Sinan Univ, Istanbul, Turkey 5Newcastle Univ, NHS Foundation Trust, Newcastle, UK 6Debrecen Univ, Debrecen, Hungary 7Oklahoma Medical Research Foundation, Oklahoma, USA 8Université Paris Sud, INSERM, Paris, France 9Anhui Provincial Hosp, Hefei, China 10Pisa Univ, Pisa, Italy 11Strasbourg Univ, CNRS, Strasbourg, France 12Christian Medical College and Hosp, Vellore, India 13Hosp ‘Santa Maria della Misericordia’, Udine 14Sapienza Univ, Rome, Italy 15INCMNSS, México City, Mexico 16Hacettepe Univ, Ankara, Turkey 17Univ Medical Center, Utrecht, Netherlands 18Catholic Univ of Korea, Seoul, Korea, Republic Of 19Karolinska Institute, Stockholm, Sweden 20Univ Medical Centre, Ljubljana, Slovenia 21Univ Paris VII Publique-Hôpitaux de Paris, 2, Paris, France 22Perugia Univ, Perugia, Italy 23H. Vall d’Hebron, Barcelona, Spain 24Western Australia Univ, Crawley, Australia 25Malmö Hosp, Lund Univ, Lund, Sweden 26Kanazawa Univ Hosp, Ishikawa, Japan 27University College, London, UK 28Federal Univ Espírito Santo, Vitória, Brazil 29Wroclaw Medical Hospital, Wroclaw, Poland 30Uppsala Univ, Uppsala, Sweden 31H. Ramón y Cajal, Madrid, Spain 32Univ Medical Center, Groningen, Netherlands 33Nagasaki University, Nagasaki, Japan 34L’Aquila Univ, L’Aquila, Italy 35Brest Univ Hosp, CERAINO, Brest, France 36Technische Univ, Munchen, Germany 37Queen Mary Univ, London, USA 38Federal Univ São Paulo, Sao Paulo, Brazil 39Haukeland Univ Hosp, Bergen, Norway 40Hospital das Clínicas, USP, São Paulo, Brazil 41Cairo University, Cairo, Egypt 42Messina and Milan Univ, Milan, Italy 43Hosp and Univ of Montpellier, Montpellier, France 44German Hosp, Buenos Aires, Argentina 45H. CIMA- Sanitas, Barcelona, Spain
Objectives: To analyse the influence of epidemiology and ethnicity on the clinical systemic presentation at diagnosis of primary Sjögren syndrome (SjS).
Methods: The Big Data Sjögren Database included 10 475 worldwide patients from 22 countries fulfilling the 2002 criteria. Age at diagnosis, gender and ethnicity (77% White, 14% Asian, 6% Hispanic, 1% Black/African American, 2% others) were correlated with systemic involvement at diagnosis (retrospectively scored in 9974 patients using ESSDAI/clinESSDAI)
Results: Men had higher mean ESSDAI (8.0 vs 5.9, p<0.001) and clinESSDAI (8.4 vs 6.1, p<0.001) in comparison with women; the domains more active in men included lymphadenopathy (p<0.001), glandular (p<0.001), pulmonary (p=0.001), PNS (p<0.001) and CNS (p<0.001). Highest scores were also reported in patients with young-onset disease (<35 years) with respect to older onset (>65 years) for ESSDAI (6.5 vs 5.6, p=0.002) and clinESSDAI (6.4 vs 5.8, p=0.034). Highest ESSDAI scores were reported in Black/African American, followed by White, Asian and Hispanic patients (6.7 vs 6.3 vs 5.4 vs 4.9, respectively, p<0.001). BAA patients showed the highest frequency of activity in lymphadenopathy, articular, PNS, CNS and biological domains, Whites in glandular, cutaneous and muscular, Asians in pulmonary, renal and haematological and Hispanics in the constitutional domain.
Conclusions: This study provides the first evidence for a strong influence of epidemiology and ethnicity on the systemic phenotype at diagnosis of primary SjS
Disclosure of Interest: None declared
DOI: 10.1136/annrheumdis-2018-eular.6073
Citation: Ann Rheum Dis, volume 77, supplement Suppl, year 2018, page A110
Session: SLE, Sjögren’s and APS- new criteria, novel diagnostic tools and co-morbidities