Paediatric Sjögren Syndrome Cohort Study and Repository (PaedSSCoRe)

NCT04762108 | Active Not Recruiting

• 1 other identifier: 132557
• Study Type: observational
• Target: 300
• Locations: 2 countries
• Sites: 8

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.

Conditions

Sjogren's Syndrome Childhood-onset

Outcome Measures

Primary Outcomes (1)

• Provide an estimate of the pre valence of relatively uncommon but serious manifestations of Sjögren's syndrome

  Provide an estimate of the pre valence of relatively uncommon but serious manifestations of SS. The investigators will describe the prevalence of severe disease manifestations, such as lymphoma, central and peripheral nervous system and renal involvement (for which there is no data in paediatric populations) on all patients recruited to this cohort during this grant. The investigators will be correlating clinical manifestations to various parameters, such as age at onset, disease duration, ethnicity, gender, pubertal status, disease scores and patient and physician outcome measures aiming to establish disease trajectories.

  2 years

Secondary Outcomes (3)

• Explore immune signatures in a multi-centre cohort of childhood onset Sjögren's syndrome

  2 years

• Assess the metabolomic/transcriptomic profiles of paediatric Sjögren's syndrome endotypes

  2 years

• Correlate -omic data with clinical and disease features to define the disease fingerprint associated with paediatric onset SS.

  2 years

Study Arms (2)

Sjögren's syndrome

Sjögren's syndrome with childhood-onset

Other: Standard of care

Healthy control

Age matched healthy control

Interventions

Standard of care OTHER

Patients will be given standard of care as per their clinical needs

Sjögren's syndrome

Eligibility Criteria

• Age: 6 Years+
• Sex: all
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• 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.

You may qualify if:

• 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.
• 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
• 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.
• Patients of 6 years or age or above

You may not qualify if:

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

Sponsors & Collaborators

• University College, London: lead
• Cambridge University Hospitals NHS Foundation Trust: collaborator
• Children's Health Ireland: collaborator
• Great Ormond Street Hospital: collaborator
• Sheffield Children's NHS Foundation Trust: collaborator
• Sheffield Teaching Hospitals NHS Foundation Trust: collaborator
• Newcastle-upon-Tyne Hospitals NHS Trust: collaborator
• University Hospital Southampton NHS Foundation Trust: collaborator

Study Sites (8)

Children's Health Ireland (CHI) at Crumlin

Dublin, Ireland

Location

Cambridge University Hospitals NHS Foundation Trust

Cambridge, United Kingdom

Location

Great Ormond Street Hospital For Children NHS Foundation Trust

London, United Kingdom

Location

University College London Hospital

London, United Kingdom

Location

The Newcastle Upon Tyne Hospitals NHS Foundation Trust

Newcastle, United Kingdom

Location

Sheffield Children's NHS Foundation Trust

Sheffield, United Kingdom

Location

Sheffield Teaching Hospitals NHS Foundation Trust

Sheffield, United Kingdom

Location

University Hospital Southampton NHS Foundation Trust

Southampton, United Kingdom

Location

Biospecimen

Retention: SAMPLES WITH DNA

Blood, tears, saliva, urine and glandular and extra-glandular (e.g. skin and renal) tissue

MeSH Terms

Interventions

Standard of Care

Intervention Hierarchy (Ancestors)

Quality Indicators, Health Care; Quality of Health Care; Health Services Administration; Health Care Quality, Access, and Evaluation

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Target Duration: 10 Years
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: February 15, 2021
• First Posted: February 21, 2021
• Study Start: January 11, 2020
• Primary Completion (Estimated): January 10, 2030
• Study Completion (Estimated): January 10, 2030
• Last Updated: April 19, 2021

Record last verified: 2021-04

Data Sharing

• IPD Sharing: Will not share

Locations

IE (1); GB (7)