Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria.
Por:
Basiaga ML, Stern SM, Mehta JJ, Edens C, Randell RL, Pomorska A, Irga-Jaworska N, Ibarra MF, Bracaglia C, Nicolai R, Susic G, Boneparth A, Srinivasalu H, Dizon B, Kamdar AA, Goldberg B, Knupp-Oliveira S, Anton-Lopez J, Mosquera-Angarita JM, Appenzeller S, O'Neil KM, Protopapas SA, Saad-Magalhães C, Akikusa JD, Thatayatikom A, Cha S, Nieto-González JC, Lo MS, Treemarcki EB, Yokogawa N, Lieberman SM and Childhood Arthritis and Rheumatology Research Alliance and the International Ch
Publicada:
1 jul 2021
Ahead of Print:
6 dic 2020
Resumen:
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.
Filiaciones:
Basiaga ML:
Division of Pediatric Rheumatology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN, USA
Stern SM:
Division of Rheumatology, Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, UT, USA
Mehta JJ:
Division of Rheumatology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA
Edens C:
Department of Internal Medicine and Pediatrics, The University of Chicago Medical Center, Chicago, IL, USA
Randell RL:
Department of Pediatrics, Duke University School of Medicine, Durham, NC, USA
Pomorska A:
Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland
Irga-Jaworska N:
Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland
Ibarra MF:
Children's Mercy Hospital, University of Missouri-Kansas City, Kansas City, MO, USA
Bracaglia C:
Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
Nicolai R:
Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
Susic G:
Department of Pediatric Rheumatology, Institute of Rheumatology, Belgrade, Serbia
Boneparth A:
Department of Pediatrics, Columbia University Medical Center, New York, NY, USA
Srinivasalu H:
Division of Rheumatology, Children's National Hospital, George Washington University School of Medicine and Health Sciences, Washington, DC, USA
Dizon B:
Division of Rheumatology, Children's National Hospital, George Washington University School of Medicine and Health Sciences, Washington, DC, USA
National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, USA
Kamdar AA:
Department of Pediatrics, University of Texas McGovern Medical School at Houston, Houston, TX, USA
Goldberg B:
Division of Pediatric Rheumatology, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, USA
Knupp-Oliveira S:
Instituto de Puericultura e Pediatria Martagão Gesteira, Universidade Federal do Rio de Janeiro, Brazil
Anton-Lopez J:
Pediatric Rheumatology, Hospital Sant Joan de Déu, Esplugues (Barcelona), Spain
Universitat de Barcelona, Barcelona, Spain
Mosquera-Angarita JM:
Pediatric Rheumatology, Hospital Sant Joan de Déu, Esplugues (Barcelona), Spain
Appenzeller S:
Rheumatology Unit, Department of Medicine, School of Medical Science, University of Campinas, São Paulo, Brazil
O'Neil KM:
Division of Rheumatology, Department of Pediatrics, University of Indiana School of Medicine and Riley Hospital for Children at Indiana University Health, Indianapolis, IN, USA
Protopapas SA:
Division of Rheumatology, Department of Pediatrics, University of Indiana School of Medicine and Riley Hospital for Children at Indiana University Health, Indianapolis, IN, USA
Saad-Magalhães C:
Pediatric Rheumatology Unit, São Paulo State University (UNESP), Botucatu, São Paulo, Brazil
Akikusa JD:
Rheumatology Service, Royal Children's Hospital, Melbourne and Murdoch Children's Research Institute, Melbourne, Australia
Thatayatikom A:
Division of Pediatric Allergy, Immunology, & Rheumatology, Department of Pediatrics, College of Medicine, University of Florida, Gainesville, FL, USA
Cha S:
Division of Oral Medicine, Department of Oral & Maxillofacial Diagnostic Sciences, College of Dentistry, University of Florida, Gainesville, FL, USA
Nieto-González JC:
Rheumatology Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain
Lo MS:
Division of Immunology, Boston Children's Hospital, Department of Pediatrics, Harvard Medical School, Boston, MA, USA
Treemarcki EB:
Division of Rheumatology, Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, UT, USA
Yokogawa N:
Department of Rheumatic Diseases, Tokyo Metropolitan Tama Medical Center, Tokyo, Japan
Lieberman SM:
Division of Rheumatology, Allergy, and Immunology, Stead Family Department of Pediatrics, Carver College of Medicine, University of Iowa, Iowa City, IA, USA
Green Published
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