Childhood Sjögren syndrome: features of an international cohort and application of
      the 2016 ACR/EULAR classification criteria

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

Objective

Sjögren syndrome in children is a poorly understood autoimmune disease. We aimed to describe the clinical and diagnostic features of children diagnosed with Sjögren syndrome and explore how the 2016 ACR/EULAR classification criteria apply to this population.

Methods

An international workgroup retrospectively collected cases of Sjögren syndrome diagnosed under 18 years of age from 23 centres across eight nations. We analysed patterns of symptoms, diagnostic workup, and applied the 2016 ACR/EULAR classification criteria.

Results

We identified 300 children with Sjögren syndrome. The majority of patients n = 232 (77%) did not meet 2016 ACR/EULAR classification criteria, but n = 110 (37%) did not have sufficient testing done to even possibly achieve the score necessary to meet criteria. Even among those children with all criteria items tested, only 36% met criteria. The most common non-sicca symptoms were arthralgia [n = 161 (54%)] and parotitis [n = 140 (47%)] with parotitis inversely correlating with age.

Conclusion

Sjögren syndrome in children can present at any age. Recurrent or persistent parotitis and arthralgias are common symptoms that should prompt clinicians to consider the possibility of Sjögren syndrome. The majority of children diagnosed with Sjögren syndromes did not meet 2016 ACR/EULAR classification criteria. Comprehensive diagnostic testing from the 2016 ACR/EULAR criteria are not universally performed. This may lead to under-recognition and emphasizes a need for further research including creation of paediatric-specific classification criteria.

Related collections

Author and article information

Contributors

Matthew L Basiaga: (View ORCID Profile)

Journal

Title: Rheumatology

Publisher: Oxford University Press (OUP)

ISSN (Print): 1462-0324

ISSN (Electronic): 1462-0332

Publication date Created: December 06 2020

Publication date (Electronic): December 06 2020

Affiliations

[1 ]Division of Pediatric Rheumatology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN, USA

[2 ]Division of Rheumatology, Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, UT, USA

[3 ]Division of Rheumatology, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

[4 ]Department of Internal Medicine and Pediatrics, The University of Chicago Medical Center, Chicago, IL, USA

[5 ]Department of Pediatrics, Duke University School of Medicine, Durham, NC, USA

[6 ]Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland

[7 ]Children’s Mercy Hospital, University of Missouri-Kansas City, Kansas City, MO, USA

[8 ]Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy

[9 ]Department of Pediatric Rheumatology, Institute of Rheumatology, Belgrade, Serbia

[10 ]Department of Pediatrics, Columbia University Medical Center, New York, NY, USA

[11 ]Division of Rheumatology, Children’s National Hospital, George Washington University School of Medicine and Health Sciences, Washington, DC, USA

[12 ]National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, USA

[13 ]Department of Pediatrics, University of Texas McGovern Medical School at Houston, Houston, TX, USA

[14 ]Division of Pediatric Rheumatology, Children’s Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, USA

[15 ]Instituto de Puericultura e Pediatria Martagão Gesteira, Universidade Federal do Rio de Janeiro, Brazil

[16 ]Pediatric Rheumatology, Hospital Sant Joan de Déu, Esplugues (Barcelona), Spain

[17 ]Universitat de Barcelona, Barcelona, Spain

[18 ]Rheumatology Unit, Department of Medicine, School of Medical Science, University of Campinas, São Paulo, Brazil

[19 ]Division of Rheumatology, Department of Pediatrics, University of Indiana School of Medicine and Riley Hospital for Children at Indiana University Health, Indianapolis, IN, USA

[20 ]Pediatric Rheumatology Unit, São Paulo State University (UNESP), Botucatu, São Paulo, Brazil

[21 ]Rheumatology Service, Royal Children’s Hospital, Melbourne and Murdoch Children’s Research Institute, Melbourne, Australia

[22 ]Division of Pediatric Allergy, Immunology, & Rheumatology, Department of Pediatrics, College of Medicine, University of Florida, Gainesville, FL, USA

[23 ]Division of Oral Medicine, Department of Oral & Maxillofacial Diagnostic Sciences, College of Dentistry, University of Florida, Gainesville, FL, USA

[24 ]Rheumatology Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain

[25 ]Division of Immunology, Boston Children's Hospital, Department of Pediatrics, Harvard Medical School, Boston, MA, USA

[26 ]Department of Rheumatic Diseases, Tokyo Metropolitan Tama Medical Center, Tokyo, Japan

[27 ]Division of Rheumatology, Allergy, and Immunology, Stead Family Department of Pediatrics, Carver College of Medicine, University of Iowa, Iowa City, IA, USA

Article

DOI: 10.1093/rheumatology/keaa757

PMC ID: 8487648

PubMed ID: 33280020

SO-VID: de8a5908-cf6a-4aba-b5b1-ca2923c84388

License:

https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model

History

Data availability:

Comments

Comment on this article

scite_

Cited by 17

See all cited by

- Version 1

Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria

Read this article at