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Rheumatology (Oxford). 2019 Dec 24. pii: kez578. doi: 10.1093/rheumatology/kez578. [Epub ahead of print]

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

Author information

1
Department of Medicine, Autoimmune Diseases Unit, Hospital CIMA - Sanitas, Barcelona, Spain.
2
Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Department of Autoimmune Diseases, ICMiD, University of Barcelona, Hospital Clínic, Barcelona, Spain.
3
Department of Statistics, Faculty of Science and Letters, Mimar Sinan Fine Arts University, Istanbul, Turkey.
4
Institute of Cellular Medicine, Newcastle University, NIHR Biomedical Research Centre, Newcastle Upon Tyne, UK.
5
Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary.
6
Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, USA.
7
Center for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique - Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicêtre, Université Paris Sud, INSERM, Paris, France.
8
Department of Rheumatology and Immunology, Anhui Provincial Hospital, Hefei, China.
9
Rheumatology Unit, University of Pisa, Pisa, Italy.
10
Department of Rheumatology, Strasbourg University Hospital, Université de Strasbourg, CNRS, Strasbourg, France.
11
Department of Clinical Immunology & Rheumatology, Christian Medical College & Hospital, Vellore, India.
12
Clinic of Rheumatology, Department of Medical Area, University Hospital 'Santa Maria della Misericordia', Udine, Italy.
13
Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Rome, Italy.
14
Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, México City, Mexico.
15
Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
16
Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
17
Division of Rheumatology, Department of Internal Medicine, College of Medicine, Seoul St Mary's Hospital, The Catholic University of Korea, Seoul, South Korea.
18
Division of Rheumatology, Department of Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
19
Department of Rheumatology, University Medical Centre, Ljubljana, Slovenia.
20
Département de Médecine Interne, Hôpital Lariboisière, Université Paris VII, Assistance Publique-Hôpitaux de Paris, Paris, France.
21
Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy.
22
Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain.
23
Department of Rheumatology, The Queen Elizabeth Hospital, Discipline of Medicine, University of Adelaide, South Australia, Australia.
24
Department of Rheumatology, Skane University Hospital Malmö, Lund University, Malmö, Sweden.
25
Division of Rheumatology, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan.
26
Centre for Rheumatology, Division of Medicine, University College London, London, UK.
27
Department of Medicine, Federal University of Espírito Santo, Vitória, Brazil.
28
Department of Rheumatology and Internal Medicine, Wroclaw Medical Hospital, Wroclaw, Poland.
29
Rheumatology, Department of Medical Sciences, University of Uppsala, Uppsala, Sweden.
30
Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain.
31
Department of Rheumatology & Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
32
Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
33
Clinical Unit of Rheumatology, School of Medicine, University of L'Aquila, L'Aquila, Italy.
34
Rheumatology Department, Brest University Hospital, Brest, France.
35
Otorhinolaryngology / Head and Neck Surgery, University Medical Center Freiburg, Freiburg, Germany.
36
Centre for Experimental Medicine and Rheumatology, Queen Mary University of London, London, UK.
37
Federal University of São Paulo, Sao Paulo, Brazil.
38
Section for Rheumatology, Department of Clinical Science, University of Bergen, Bergen, Norway.
39
Department of Rheumatology, Haukeland University Hospital, Bergen, Norway.
40
Rheumatology Division, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Brazil.
41
Instituto De Investigaciones En Ciencias De La Salud (INICSA), Universidad Nacional de Córdoba (UNC), Cordoba, Argentina.
42
Department of Rheumatology, Instituto Modelo de Cariología Privado S.R.L, Instituto Universitario de Ciencias Biomídicas de Córdoba, Cordoba, Argentina.
43
Rheumatology Department, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt.
44
IRCCS Galeazzi Orthopedic Institute, Milan, Italy.
45
Rheumatology Unit, University of Messina, Messina, Italy.
46
Department of Rheumatology, CHU Montpellier, University of Montpellier, Montpellier, France.
47
Department of Rheumatology, German Hospital, Buenos Aires, Argentina.

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.

KEYWORDS:

ethnicity; gender; geoepidemiology; phenotype; primary Sjögren’s syndrome

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