Artículos de revistas
Childhood Sjögren syndrome: Features of an international cohort and application of the 2016 ACR/EULAR classification criteria
Fecha
2021-07-01Registro en:
Rheumatology (United Kingdom), v. 60, n. 7, p. 3144-3155, 2021.
1462-0332
1462-0324
10.1093/rheumatology/keaa757
2-s2.0-85110554975
Autor
Mayo Clinic
University of Utah School of Medicine
The Children's Hospital of Philadelphia
The University of Chicago Medical Center
Duke University School of Medicine
Medical University of Gdansk
University of Missouri-Kansas City
Irccs Ospedale Pediatrico Bambino Gesù
Institute of Rheumatology
Columbia University Medical Center
George Washington University School of Medicine and Health Sciences
National Institute of Arthritis and Musculoskeletal and Skin Diseases
University of Texas McGovern Medical School at Houston
Emory University School of Medicine
Universidade Federal do Rio de Janeiro (UFRJ)
Hospital Sant Joan de Déu
Barcelona
Universidade Estadual de Campinas (UNICAMP)
Riley Hospital for Children at Indiana University Health
Universidade Estadual Paulista (UNESP)
Melbourne and Murdoch Children's Research Institute
University of Florida
Hospital General Universitario Gregorio Marañón
Harvard Medical School
Tokyo Metropolitan Tama Medical Center
University of Iowa
Institución
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.