Updates in childhood Sjogren's syndrome

Abstract

Purpose of reviewChildhood Sjogren's syndrome (cSS) is a rare, chronic autoimmune disease characterized by inflammation of the exocrine glands. cSS is underrecognized because of differences in clinical presentation compared with adults. Until recently, publications describing clinical manifestations in cSS were limited to case reports and case series with small numbers of patients. Diagnostic studies to assess glandular symptoms in adults, are less commonly obtained in children.Recent findingsRecent cohort studies describe presenting diagnostic clinical features in large populations of cSS and demonstrate how current classification criteria, used in adults, are not applicable to children. Recurrent parotitis is the consistent predominant manifestation that is inversely correlated with age. Novel salivary biomarkers and salivary gland ultrasonography are important objective measure, which may improve diagnosis and disease monitoring. Standardized treatment recommendations are needed.SummaryFindings from large cohort studies provide a framework for the future development of diagnostic criteria for cSS. Such criteria should incorporate objective measures that are easily obtained in children. Future research to improve understanding of the application of novel biomarkers and imaging and developing consensus on treatment recommendations is needed.

Single-cell transcriptomics unveil profiles and interplay of immune subsets in rare autoimmune childhood Sjögren’s disease

“…One of the limitations in our study stems from the absence of the cSjDspecific diagnostic criteria. Our clinical observations and laboratory findings support the possibility that cSjD and aSjD are likely to be distinct disease https://doi.org/10.1038/s42003-024-06124-6 entities 6,38 . The establishment of the cSjD-specific criteria, reflecting the importance of the most common clinical presentation of RP, is urgently needed in the field.…”

“…Accumulating evidence supports the increasing incidences of SjD occurring in children (childhood SjD, cSjD) 6 . cSjD has long been considered as a rare autoimmune disease affecting children and adolescents younger than 20 years of age 7 with an estimated prevalence of 1% of SjD patients 8 .…”

“…Myung-Chul Kim 1,2,3,4,5 , Umasankar De 1,2 , Nicholas Borcherding 6 , Lei Wang 1,2 , Joon Paek 5,6 , Indraneel Bhattacharyya 5,7 , Qing Yu 8 , Ryan Kolb 1,2 , Theodore Drashansky 9 , Akaluck Thatayatikom 10,11 , Weizhou Zhang 1,2,11 & Seunghee Cha 5,7,11 Childhood Sjögren's disease represents critically unmet medical needs due to a complete lack of immunological and molecular characterizations. This study presents key immune cell subsets and their interactions in the periphery in childhood Sjögren's disease.…”

Phases and Natural History of Sjögren's Disease: A New Model for an Old Disease?

“…Another potential exception of EGMs preceding glandular dysfunction is childhood‐onset SjD, in which sicca symptoms appear less frequently than adult‐onset SjD and may manifest after the appearance of EGMs such as renal tubular acidosis or neurologic complications ( 54 ). However, large cohort studies indicate recurrent parotitis is a consistent feature of childhood SjD and longitudinal studies are required to determine whether subclinical glandular inflammation occurs before EGMs ( 54 ).…”

Extranodal marginal zone lymphoma as the presenting feature of paediatric Sjögren syndrome

“…19 Importantly, children with SS can present atypically and are less likely to meet current classification criteria. 21,23,24 A relevant proportion of children presenting with lymphoma have a predisposing condition, albeit that each predisposing condition may be seen only rarely. 11 For some children, that predisposing condition has already been identified, but undoubtedly some go unrecognised and the true incidence is unknown.…”