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

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 work...

Descripción completa

Detalles Bibliográficos
Autores: 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, Antón J, Mosquera 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, Childhood Arthritis and Rheumatology Research Alliance and the International Ch
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2021
País:España
Institución:Fundació Sant Joan de Déu
Repositorio:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
OAI Identifier:oai:fsjd.fundanetsuite.com:p18481
Acceso en línea:https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=18481
https://academic.oup.com/rheumatology/article/60/7/3144/6024784
Access Level:acceso abierto
Palabra clave:*Sjögren Syndrome
*childhood Sjögren syndrome
*pediatric rheumatology
*recurrent parotitis
Descripción
Sumario: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.

Similares en LA Referencia