Childhood-onset of primary Sjögren's syndrome: phenotypic characterization at diagnosis of 158 children.
Manuel Ramos-CasalsNihan Acar-DenizliArjan VissinkPilar Brito-ZerónXiaomei LiFrancesco CarubbiRoberta PrioriNataša ToplakChiara BaldiniEnrique Faugier-FuentesAike A KruizeThomas MandlMinako TomiitaSaviana GandolfoKunio HashimotoGabriela Hernandez-MolinaBenedikt HofauerSamara Mendieta-ZerónAstrid RasmussenPulukool SandhyaDamien SeneVirginia Fernandes Moça TrevisaniDavid Alan IsenbergErik SundbergSandra G PasotoAgata SebastianYasunori SuzukiSoledad RetamozoBei XuRoberto GiacomelliAngelica GattamelataMasa BizjakStefano BombardieriRichard-Eduardo Loor-ChavezAnneline HinrichsPeter OlssonHendrika BootsmaScott M Liebermannull nullPublished in: Rheumatology (Oxford, England) (2021)
Childhood-onset primary SS involves around 1% of patients with primary SS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role in modulating the phenotypic expression of the disease.