Clinical trial NCT04762108: Paediatric Sjögren Syndrome Cohort Study and Repository (PaedSSCoRe)
Official title: Paediatric Sjögren Syndrome Cohort Study and Repository (PaedSSCoRe)
Study type: OBSERVATIONAL
Overall status: ACTIVE_NOT_RECRUITING
Conditions: Sjogren's Syndrome Childhood-onset
Lead sponsor: University College, London
Sponsor class: OTHER
Collaborators: name: Cambridge University Hospitals NHS Foundation Trust
name: Children's Health Ireland
name: Great Ormond Street Hospital
name: Sheffield Children's NHS Foundation Trust
name: Sheffield Teaching Hospitals NHS Foundation Trust
name: Newcastle-upon-Tyne Hospitals NHS Trust
name: University Hospital Southampton NHS Foundation Trust
First posted: 2021-02-21
Enrollment: 300
Sex: ALL
Minimum age (years): 6
Standard ages: CHILD
ADULT
OLDER_ADULT
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: PROBABILITY_SAMPLE
Study population: Patients with Sjögren's syndrome with childhood-onset Healthy age matched controls Based on our preliminary estimations, there are currently 100-150 JSS patients under hospital care in the UK. The investigators envisaged to capture as many of the patients available, as well as the ones diagnosed for the duration of this project (10 years), estimating a recruitment number limit of 300 patients.

Brief summary: Sjögren syndrome (SS) in adults is characterised by inflammation of the exocrine glands, principally the salivary and lacrimal glands resulting in xerostomia (dry mouth) and xerophthalmia (dry eyes).It can also present with more extensive exocrinopathy as well as extra-glandular, systemic features.

SS is defined as primary SS (pSS) when it occurs in isolation, and as secondary SS, if associated with other autoimmune conditions. The incidence and prevalence rates of SS vary depending on the population. To date, there have been no studies reporting accurate incidence or prevalence of SS in childhood. Childhood onset SS defined as disease diagnosed before 18 years of age is believed to be rare; however, it is likely it is under-recognised and therefore under-diagnosed.

The overarching aim of this study is to identify epidemiological, clinical and laboratory characteristics of paediatric SS in a United Kingdom (UK) multi-centre cohort of patients. Using this data our goal is to develop universally accepted classification criteria that could be validated for use in a paediatric population.

Inclusion criterion for the study and repository is a diagnosis of SS made before 18 years by the referring physician. A data collection pack will be sent to authors willing to participate. Information collected will include but not exclusive to: demographic, clinical and laboratory/histological data at diagnosis and subsequent follow-up appointments. Biological samples including blood, tears, saliva, urine and 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 at set time points (every 6 months) and during flares.

PaedSSCoRe will capture data on a significant cohort of children with SS providing a powerful resource to help improve our understanding of the pathogenesis and natural course of this disease.

Prospective data collection will allow a fuller analysis of poor prognostic features, impact of therapy and damage accrual, and variable outcome of childhood SS.


Eligibility criteria: Inclusion Criteria:

1. All recruited patients should be diagnosed with Sjogren's syndrome alone or in association with other autoimmune conditions (rheumatic or not) based on their rheumatologist opinion or have clinical and serological/imaging features which in the opinion of their clinician raise the suspicion of Sjogren's syndrome.
2. Patients with symptoms onset /imaging, serological and glandular biopsy abnormalities suggestive of SS (suspected SS) or SS diagnosis made by expert clinicians prior to age 18
3. Patients/ Carers who can provide informed consent and agree to provide access to the results of their routine care investigations for research purposes irrespective of being able provide blood or saliva samples.
4. Patients of 6 years or age or above

Exclusion Criteria:

1. Inability to provide informed consent or have informed consent provided on their behalf.

Primary outcomes:
measure: Provide an estimate of the pre valence of relatively uncommon but serious manifestations of Sjögren's syndrome
timeFrame: 2 years
Secondary outcomes:
measure: Explore immune signatures in a multi-centre cohort of childhood onset Sjögren's syndrome
timeFrame: 2 years
measure: Assess the metabolomic/transcriptomic profiles of paediatric Sjögren's syndrome endotypes
timeFrame: 2 years
measure: Correlate -omic data with clinical and disease features to define the disease fingerprint associated with paediatric onset SS.
timeFrame: 2 years
Other outcomes:
Locations:
city: Dublin
country: Ireland
facility: Children's Health Ireland (CHI) at Crumlin
city: Cambridge
country: United Kingdom
facility: Cambridge University Hospitals NHS Foundation Trust
city: London
country: United Kingdom
facility: Great Ormond Street Hospital For Children NHS Foundation Trust
city: London
country: United Kingdom
facility: University College London Hospital
city: Newcastle
country: United Kingdom
facility: The Newcastle Upon Tyne Hospitals NHS Foundation Trust
city: Sheffield
country: United Kingdom
facility: Sheffield Children's NHS Foundation Trust
city: Sheffield
country: United Kingdom
facility: Sheffield Teaching Hospitals NHS Foundation Trust
city: Southampton
country: United Kingdom
facility: University Hospital Southampton NHS Foundation Trust
Study officials:
References: