How immunological profle drives clinical phenotype of primary Sjögren’s syndrome at diagnosis: analysis of 10,500 patients (Sjögren Big Data Project)

• Autores: Brito Zerón, Pilar; Acar Denizli, Nihan; Ng, Wan Fai; Zeher, Margit; Rasmussen, Astrid; Mandl, Thomas; Seror, Raphaele; Xiaolin, Li; Baldini, Chiara; Gottenberg, Jaques; Danda, Debashish; Quartuccio, Luca; Priori, Roberta; Hernandez Molina, Gabriela; Armagan, Berkan; Kruize, Aike; Kwok, Seung Ki; Kvarnström, Marika; Praprotnik, Sonja; Sene, Damien; Bartoloni, Elena; Solans, R.; Rischmueller, M.; Suzuki, Y.; Isenberg, D. A.; Valim, V.; Wiland, P.; Nordmark, G.; Fraile, G.; Retamozo, Maria Soledad
• Año de publicación: 2018
• Idioma: inglés
• Tipo de recurso: artículo
• Materia: Primary Sjögren’s syndrome; Salivary gland biopsy; Ro/La autoantibodies; Hypocomplementaemia; Cryoglobulinaemia
• Institución: Consejo Nacional de Investigaciones Científicas y Técnicas

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 cryoglo-bulinaemia 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 ESS- DAI evaluation were cryoglobulins (9 domains), low C3 (8 domains), anti-La (7 domains) and low C4 (6 domains).CONCLUSIONS: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.
Fil: Brito Zerón, Pilar. Hospital Sanitas CIMA; España. Universidad de Barcelona; España
Fil: Acar Denizli, Nihan. Mimar Sinan Fine Arts University; Turquía
Fil: Ng, Wan Fai. University of Newcastle; Reino Unido
Fil: Zeher, Margit. University of Debrecen; Hungría
Fil: Rasmussen, Astrid. Oklahoma Medical Research Foundation; Estados Unidos
Fil: Mandl, Thomas. Lund University; Suecia
Fil: Seror, Raphaele. Université Paris Sud; Francia
Fil: Xiaolin, Li. Anhui Provincial Hospital; China
Fil: Baldini, Chiara. Università degli Studi di Pisa; Italia
Fil: Gottenberg, Jaques. Université de Strasbourg; Francia. Centre National de la Recherche Scientifique; Francia
Fil: Danda, Debashish. Christian Medical College & Hospital; India
Fil: Quartuccio, Luca. University Hospital “Santa María della Misericordia”; Italia
Fil: Priori, Roberta. Università degli Studi di Roma "La Sapienza"; Italia
Fil: Hernandez Molina, Gabriela. Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán; México
Fil: Armagan, Berkan. Hacettepe University. Faculty of Medicine.Department of Internal Medicine; Turquía
Fil: Kruize, Aike. University Medical Center Utrecht; Países Bajos
Fil: Kwok, Seung Ki. The Catholic University of Korea; Corea del Sur
Fil: Kvarnström, Marika. Karolinska University Hospital.Department of Medicine.Unit of Rheumatology. Karolinska Institutet ; Suecia
Fil: Praprotnik, Sonja. University Medical Centre; Eslovenia
Fil: Sene, Damien. Université Paris Diderot - Paris 7; Francia
Fil: Bartoloni, Elena. Università di Perugia; Italia
Fil: Solans, R.. Hospital Vall d’Hebron; Italia
Fil: Rischmueller, M.. University of Western Australia; Australia
Fil: Suzuki, Y.. Kanazawa University Hospital; Japón
Fil: Isenberg, D. A.. University College London; Estados Unidos
Fil: Valim, V.. Federal University of Espírito Santo; Brasil
Fil: Wiland, P.. Wroclaw Medical Hospital; Polonia
Fil: Nordmark, G.. Uppsala Universitet; Suecia
Fil: Fraile, G.. Hospital Ramón y Cajal; España
Fil: Retamozo, Maria Soledad. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto de Investigaciones en Ciencias de la Salud. Universidad Nacional de Córdoba. Instituto de Investigaciones en Ciencias de la Salud; Argentina. Hospital Privado Centro Medico de Córdoba; Argentina; Argentina. Instituto Universitario de Ciencias Biomédicas de Córdoba; Argentina