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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)

, ; Brito-Zerón, Pilar; Acar-Denizli, Nihan; Ng, Wan-Fai; Zeher, Margit; Rasmussen, Astrid; Mandl, Thomas LU ; Seror, Raphaele; Li, Xiaomei and Baldini, Chiara, et al. (2018) In Clinical and Experimental Rheumatology 36 Suppl 112(3). p.102-112
Abstract

OBJECTIVES: 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... (More)

OBJECTIVES: 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.

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Clinical and Experimental Rheumatology
volume
36 Suppl 112
issue
3
pages
11 pages
publisher
Pacini
external identifiers
  • scopus:85055613559
ISSN
0392-856X
language
English
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yes
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d6ac6b68-a4bb-42ee-ae4a-8d8e298e74f3
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https://www.clinexprheumatol.org/abstract.asp?a=12899
date added to LUP
2018-08-31 15:28:57
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2019-08-14 04:21:42
@article{d6ac6b68-a4bb-42ee-ae4a-8d8e298e74f3,
  abstract     = {<p>OBJECTIVES: 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).</p><p>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.</p><p>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&lt;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).</p><p>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.</p>},
  author       = {,  and Brito-Zerón, Pilar and Acar-Denizli, Nihan and Ng, Wan-Fai and Zeher, Margit and Rasmussen, Astrid and Mandl, Thomas and Seror, Raphaele and Li, Xiaomei and Baldini, Chiara and Gottenberg, Jacques-Eric and Danda, Debashish and Quartuccio, Luca and Priori, Roberta and Hernandez-Molina, Gabriela and Armagan, Berkan and Kruize, Aike A and Kwok, Seung-Ki and Kvarnström, Marika and Praprotnik, Sonja and Sène, Damien and Bartoloni, Elena and Solans, Roser and Rischmueller, Maureen and Suzuki, Yasunori and Isenberg, David A and Valim, Valeria and Wiland, Piotr and Nordmark, Gunnel and Fraile, Guadalupe and Bootsma, Hendrika and Nakamura, Takashi and Giacomelli, Roberto and Devauchelle-Pensec, Valerie and Knopf, Andreas and Bombardieri, Michele and Trevisani, Virginia-Fernandes and Hammenfors, Daniel and Pasoto, Sandra G and Retamozo, Soledad and Gheita, Tamer A and Atzeni, Fabiola and Morel, Jacques and Vollenveider, Cristina and Horvath, Ildiko-Fanny and Sivils, Kathy L and Olsson, Peter and De Vita, Salvatore and Sánchez-Guerrero, Jorge and Kilic, Levent and Wahren-Herlenius, Marie and Mariette, X. and Ramos-Casals, M.},
  issn         = {0392-856X},
  language     = {eng},
  month        = {08},
  number       = {3},
  pages        = {102--112},
  publisher    = {Pacini},
  series       = {Clinical and Experimental Rheumatology},
  title        = {How immunological profile drives clinical phenotype of primary Sjögren's syndrome at diagnosis : analysis of 10,500 patients (Sjögren Big Data Project)},
  volume       = {36 Suppl 112},
  year         = {2018},
}