Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjögren's syndrome

Pilar Brito-Zerón; Nihan Acar-Denizli; Wan Fai Ng; Ildiko Fanny Horváth; Astrid Rasmussen; Raphaele Seror; Xiaomei Li; Chiara Baldini; Jacques Eric Gottenberg; Debashish Danda; Luca Quartuccio; Roberta Priori; Gabriela Hernandez-Molina; Berkan Armagan; Aike A. Kruize; Seung Ki Kwok; Marika Kvarnstrom; Sonja Praprotnik; Damien Sene; Roberto Gerli; Roser Solans; Maureen Rischmueller; Thomas Mandl; Yasunori Suzuki; David Isenberg; Valeria Valim; Piotr Wiland; Gunnel Nordmark; Guadalupe Fraile; Hendrika Bootsma; Hideki Nakamura; Roberto Giacomelli; Valerie Devauchelle-Pensec; Benedikt Hofauer; Michele Bombardieri; Virginia Fernandes Moça Trevisani; Daniel Hammenfors; Sandra G. Pasoto; Soledad Retamozo; Tamer A. Gheita; Fabiola Atzeni; Jacques Morel; Cristina Vollenweider; Margit Zeher; Kathy Sivils; Bei Xu; Stefano Bombardieri; Pulukool Sandhya; Salvatore De Vita; Antonina Minniti; Jorge Sánchez-Guerrero; Levent Kilic; Eefje Van Der Heijden; Sung Hwan Park; Marie Wahren-Herlenius; Xavier Mariette; Manuel Ramos-Casals

doi:10.1093/rheumatology/kez578

Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjögren's syndrome

Pilar Brito-Zerón
, Nihan Acar-Denizli
, Wan Fai Ng
, Ildiko Fanny Horváth
, Astrid Rasmussen
, Raphaele Seror
, Xiaomei Li
, Chiara Baldini
, Jacques Eric Gottenberg
, Debashish Danda
, Luca Quartuccio
, Roberta Priori
, Gabriela Hernandez-Molina
, Berkan Armagan
, Aike A. Kruize
, Seung Ki Kwok
, Marika Kvarnstrom
, Sonja Praprotnik
, Damien Sene
, Roberto Gerli

Roser Solans, Maureen Rischmueller, Thomas Mandl, Yasunori Suzuki, David Isenberg, Valeria Valim, Piotr Wiland, Gunnel Nordmark, Guadalupe Fraile, Hendrika Bootsma, Hideki Nakamura, Roberto Giacomelli, Valerie Devauchelle-Pensec, Benedikt Hofauer, Michele Bombardieri, Virginia Fernandes Moça Trevisani, Daniel Hammenfors, Sandra G. Pasoto, Soledad Retamozo, Tamer A. Gheita, Fabiola Atzeni, Jacques Morel, Cristina Vollenweider, Margit Zeher, Kathy Sivils, Bei Xu, Stefano Bombardieri, Pulukool Sandhya, Salvatore De Vita, Antonina Minniti, Jorge Sánchez-Guerrero, Levent Kilic, Eefje Van Der Heijden, Sung Hwan Park, Marie Wahren-Herlenius, Xavier Mariette, Manuel Ramos-Casals

Hospital CIMA Sanitas
University of Barcelona
Mimar Sinan Guzel Sanatlar University
Newcastle University
University of Debrecen
Oklahoma Medical Research Foundation
Institut national de la santé et de la recherche médicale
Anhui Provincial Hospital
University of Pisa
Strasbourg University Hospital
Christian Medical College
S. Maria Della Misericordia Hospital
University of Rome La Sapienza
Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran
Hacettepe University
Utrecht University
The Catholic University of Korea
Karolinska Institutet
University Medical Centre
Université Paris Cité
University of Perugia
Hospital Universitari Vall d'Hebron
University of Adelaide
Lund University
Kanazawa University
University College London
Universidade Federal do Espírito Santo
Wroclaw Medical Hospital
Uppsala University
Hospital Ramon y Cajal
University of Groningen
Nagasaki University
University of L'Aquila
CHU de Brest
University of Freiburg
Queen Mary University of London
Universidade Federal de São Paulo
University of Bergen
Universidade de São Paulo
Universidad Nacional de Córdoba
Instituto Universitario de Ciencias Biomédicas de Córdoba
Cairo University
IRCCS Galeazzi Orthopedic Institute
University of Messina
Université de Montpellier
Hospital Alemán

Research output: Contribution to journal › Article › peer-review

Abstract

Objective: To characterize the systemic phenotype of primary Sjögren's syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. Methods: The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren's syndrome from the five continents. Results: The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P < 0.001) compared with females, as did patients diagnosed at <35 years (6.7 vs 5.6 in patients diagnosed at >65 years, P < 0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P < 0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P < 0.001). Conclusion: The systemic phenotype of primary Sjögren's syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.

Original language	English
Pages (from-to)	2350-2359
Number of pages	10
Journal	Rheumatology
Volume	59
Issue number	9
DOIs	https://doi.org/10.1093/rheumatology/kez578
Publication status	Published - 1 Sep 2020
Externally published	Yes

Keywords

ethnicity
gender
geoepidemiology
phenotype
primary Sjögren's syndrome

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10.1093/rheumatology/kez578

Cite this

Brito-Zerón, P., Acar-Denizli, N., Ng, W. F., Horváth, I. F., Rasmussen, A., Seror, R., Li, X., Baldini, C., Gottenberg, J. E., Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, A. A., Kwok, S. K., Kvarnstrom, M., Praprotnik, S., Sene, D., ... Ramos-Casals, M. (2020). Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjögren's syndrome. Rheumatology, 59(9), 2350-2359. https://doi.org/10.1093/rheumatology/kez578

@article{ece672fa459a4b29bee79158b4c830e8,

title = "Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sj{\"o}gren's syndrome",

abstract = "Objective: To characterize the systemic phenotype of primary Sj{\"o}gren's syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. Methods: The Sj{\"o}gren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sj{\"o}gren's syndrome from the five continents. Results: The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8\% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P < 0.001) compared with females, as did patients diagnosed at <35 years (6.7 vs 5.6 in patients diagnosed at >65 years, P < 0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P < 0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P < 0.001). Conclusion: The systemic phenotype of primary Sj{\"o}gren's syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.",

keywords = "ethnicity, gender, geoepidemiology, phenotype, primary Sj{\"o}gren's syndrome",

author = "Pilar Brito-Zer{\'o}n and Nihan Acar-Denizli and Ng, \{Wan Fai\} and Horv{\'a}th, \{Ildiko Fanny\} and Astrid Rasmussen and Raphaele Seror and Xiaomei Li and Chiara Baldini and Gottenberg, \{Jacques Eric\} and Debashish Danda and Luca Quartuccio and Roberta Priori and Gabriela Hernandez-Molina and Berkan Armagan and Kruize, \{Aike A.\} and Kwok, \{Seung Ki\} and Marika Kvarnstrom and Sonja Praprotnik and Damien Sene and Roberto Gerli and Roser Solans and Maureen Rischmueller and Thomas Mandl and Yasunori Suzuki and David Isenberg and Valeria Valim and Piotr Wiland and Gunnel Nordmark and Guadalupe Fraile and Hendrika Bootsma and Hideki Nakamura and Roberto Giacomelli and Valerie Devauchelle-Pensec and Benedikt Hofauer and Michele Bombardieri and Trevisani, \{Virginia Fernandes Mo{\c c}a\} and Daniel Hammenfors and Pasoto, \{Sandra G.\} and Soledad Retamozo and Gheita, \{Tamer A.\} and Fabiola Atzeni and Jacques Morel and Cristina Vollenweider and Margit Zeher and Kathy Sivils and Bei Xu and Stefano Bombardieri and Pulukool Sandhya and \{De Vita\}, Salvatore and Antonina Minniti and Jorge S{\'a}nchez-Guerrero and Levent Kilic and \{Van Der Heijden\}, Eefje and Park, \{Sung Hwan\} and Marie Wahren-Herlenius and Xavier Mariette and Manuel Ramos-Casals",

note = "Publisher Copyright: {\textcopyright} 2019 The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].",

year = "2020",

month = sep,

day = "1",

doi = "10.1093/rheumatology/kez578",

language = "English",

volume = "59",

pages = "2350--2359",

journal = "Rheumatology",

issn = "1462-0324",

publisher = "Oxford University Press",

number = "9",

}

Brito-Zerón, P, Acar-Denizli, N, Ng, WF, Horváth, IF, Rasmussen, A, Seror, R, Li, X, Baldini, C, Gottenberg, JE, Danda, D, Quartuccio, L, Priori, R, Hernandez-Molina, G, Armagan, B, Kruize, AA, Kwok, SK, Kvarnstrom, M, Praprotnik, S, Sene, D, Gerli, R, Solans, R, Rischmueller, M, Mandl, T, Suzuki, Y, Isenberg, D, Valim, V, Wiland, P, Nordmark, G, Fraile, G, Bootsma, H, Nakamura, H, Giacomelli, R, Devauchelle-Pensec, V, Hofauer, B, Bombardieri, M, Trevisani, VFM, Hammenfors, D, Pasoto, SG, Retamozo, S, Gheita, TA, Atzeni, F, Morel, J, Vollenweider, C, Zeher, M, Sivils, K, Xu, B, Bombardieri, S, Sandhya, P, De Vita, S, Minniti, A, Sánchez-Guerrero, J, Kilic, L, Van Der Heijden, E, Park, SH, Wahren-Herlenius, M, Mariette, X & Ramos-Casals, M 2020, 'Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjögren's syndrome', Rheumatology, vol. 59, no. 9, pp. 2350-2359. https://doi.org/10.1093/rheumatology/kez578

TY - JOUR

T1 - Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjögren's syndrome

AU - Brito-Zerón, Pilar

AU - Acar-Denizli, Nihan

AU - Ng, Wan Fai

AU - Horváth, Ildiko Fanny

AU - Rasmussen, Astrid

AU - Seror, Raphaele

AU - Li, Xiaomei

AU - Baldini, Chiara

AU - Gottenberg, Jacques Eric

AU - Danda, Debashish

AU - Quartuccio, Luca

AU - Priori, Roberta

AU - Hernandez-Molina, Gabriela

AU - Armagan, Berkan

AU - Kruize, Aike A.

AU - Kwok, Seung Ki

AU - Kvarnstrom, Marika

AU - Praprotnik, Sonja

AU - Sene, Damien

AU - Gerli, Roberto

AU - Solans, Roser

AU - Rischmueller, Maureen

AU - Mandl, Thomas

AU - Suzuki, Yasunori

AU - Isenberg, David

AU - Valim, Valeria

AU - Wiland, Piotr

AU - Nordmark, Gunnel

AU - Fraile, Guadalupe

AU - Bootsma, Hendrika

AU - Nakamura, Hideki

AU - Giacomelli, Roberto

AU - Devauchelle-Pensec, Valerie

AU - Hofauer, Benedikt

AU - Bombardieri, Michele

AU - Trevisani, Virginia Fernandes Moça

AU - Hammenfors, Daniel

AU - Pasoto, Sandra G.

AU - Retamozo, Soledad

AU - Gheita, Tamer A.

AU - Atzeni, Fabiola

AU - Morel, Jacques

AU - Vollenweider, Cristina

AU - Zeher, Margit

AU - Sivils, Kathy

AU - Xu, Bei

AU - Bombardieri, Stefano

AU - Sandhya, Pulukool

AU - De Vita, Salvatore

AU - Minniti, Antonina

AU - Sánchez-Guerrero, Jorge

AU - Kilic, Levent

AU - Van Der Heijden, Eefje

AU - Park, Sung Hwan

AU - Wahren-Herlenius, Marie

AU - Mariette, Xavier

AU - Ramos-Casals, Manuel

N1 - Publisher Copyright: © 2019 The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].

PY - 2020/9/1

Y1 - 2020/9/1

N2 - Objective: To characterize the systemic phenotype of primary Sjögren's syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. Methods: The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren's syndrome from the five continents. Results: The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P < 0.001) compared with females, as did patients diagnosed at <35 years (6.7 vs 5.6 in patients diagnosed at >65 years, P < 0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P < 0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P < 0.001). Conclusion: The systemic phenotype of primary Sjögren's syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.

AB - Objective: To characterize the systemic phenotype of primary Sjögren's syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. Methods: The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren's syndrome from the five continents. Results: The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P < 0.001) compared with females, as did patients diagnosed at <35 years (6.7 vs 5.6 in patients diagnosed at >65 years, P < 0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P < 0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P < 0.001). Conclusion: The systemic phenotype of primary Sjögren's syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.

KW - ethnicity

KW - gender

KW - geoepidemiology

KW - phenotype

KW - primary Sjögren's syndrome

UR - https://www.scopus.com/pages/publications/85078897832

U2 - 10.1093/rheumatology/kez578

DO - 10.1093/rheumatology/kez578

M3 - Article

C2 - 31873754

AN - SCOPUS:85078897832

SN - 1462-0324

VL - 59

SP - 2350

EP - 2359

JO - Rheumatology

JF - Rheumatology

IS - 9

ER -

Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjögren's syndrome

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