TBA (19A170)

UK and Ireland Paediatric and Young Person Primary Sjögren Syndrome Cohort Study and Repository (UK/Ireland PpSS cohort study and repository)

Author(s)

Foley CM (1,2), Al Obaidi M (1), Ciurtin C2

Department(s)/Institutions

(1) Rheumatology Department, Great Ormond Street Hospital, London, UK (2) Centre for Adolescent Rheumatology, University College London, UK

Introduction

Sjögren syndrome, a chronic multisystem autoimmune disease is characterised by inflammation of the exocrine glands, principally the salivary and lacrimal glands. It can present with more extensive exocrinopathy as well as extra-glandular, systemic features.

Incidence and prevalence rates of primary SS (pSS) vary. Juvenile-onset-pSS is believed to be rare; however it is likely that it is under-recognised and therefore under-diagnosed. To date there have been no studies reporting accurate incidence or prevalence of paediatric pSS (PpSS).

Diagnosing pSS can be challenging. Many of the cardinal symptoms are non-specific and no gold standard biomarker of disease exists. Between 1965–2002 eleven diagnostic criteria sets were developed, none of which have gained universal acceptance or been validated for use in a paediatric population. Until recently, the most widely used criteria were those developed by the American-European Consensus Group.

It remains well recognised that international consensus on classification is important for standardisation, particularly in relation to research and monitoring treatment outcomes. With this in mind, the 2016 ACR/EULAR criteria were developed. However, there still remains a paucity of validated classification criteria for diagnosing PpSS. Paediatric-focussed criteria are required as features of PpSS differ from those observed in adults. Applying adult criteria to a paediatric population may lead to mis- and/or under-diagnosis.

Aims/Background

Identify epidemiological, clinical and laboratory characteristics of PpSS in a multi-centre cohort.

Develop universally accepted classification criteria validated for use in a paediatric population.

Method

Inclusion criterion for entry into the UK/Ireland PpSS cohort study and repository is a diagnosis of pSS made before 18-years. A data collection pack will be sent to willing author-participants. Demographic, clinical and laboratory/histological data at diagnosis and subsequent follow-up appointments will be collected. Biological-samples including blood, tears, saliva, urine, glandular and extra-glandular (e.g. renal) tissue will be collected prospectively if available. Outcome measures related to disease-activity and damage, as well as patient-reported outcomes will also be collected.

Results

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Conclusions

The UK/Ireland PpSS cohort study and repository will provide a powerful resource to help improve our understanding of this rare disease.