- ICH GCP
- US Clinical Trials Registry
- Clinical Trial NCT04762108
Paediatric Sjögren Syndrome Cohort Study and Repository (PaedSSCoRe) (PaedSSCoRe)
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.
Study Overview
Status
Conditions
Intervention / Treatment
Study Type
Enrollment (Anticipated)
Contacts and Locations
Study Locations
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Dublin, Ireland
- Children's Health Ireland (CHI) at Crumlin
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Cambridge, United Kingdom
- Cambridge University Hospitals NHS Foundation Trust
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London, United Kingdom
- University College London Hospital
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London, United Kingdom
- Great Ormond Street Hospital For Children NHS Foundation Trust
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Newcastle, United Kingdom
- The Newcastle Upon Tyne Hospitals NHS Foundation Trust
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Sheffield, United Kingdom
- Sheffield Teaching Hospitals NHS Foundation Trust
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Sheffield, United Kingdom
- Sheffield Children's NHS Foundation Trust
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Southampton, United Kingdom
- University Hospital Southampton NHS Foundation Trust
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Participation Criteria
Eligibility Criteria
Ages Eligible for Study
Accepts Healthy Volunteers
Genders Eligible for Study
Sampling Method
Study Population
Description
Inclusion Criteria:
- 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
Exclusion Criteria:
1. Inability to provide informed consent or have informed consent provided on their behalf.
Study Plan
How is the study designed?
Design Details
- Observational Models: Cohort
- Time Perspectives: Prospective
Cohorts and Interventions
Group / Cohort |
Intervention / Treatment |
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Sjögren's syndrome
Sjögren's syndrome with childhood-onset
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Patients will be given standard of care as per their clinical needs
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Healthy control
Age matched healthy control
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What is the study measuring?
Primary Outcome Measures
Outcome Measure |
Measure Description |
Time Frame |
---|---|---|
Provide an estimate of the pre valence of relatively uncommon but serious manifestations of Sjögren's syndrome
Time Frame: 2 years
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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.
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2 years
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Secondary Outcome Measures
Outcome Measure |
Measure Description |
Time Frame |
---|---|---|
Explore immune signatures in a multi-centre cohort of childhood onset Sjögren's syndrome
Time Frame: 2 years
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The investigators aim to explore the specific immune signatures identified in adults in children and adolescent patients with paediatric onset SS.
The investigators will assess their immune signatures (combined monocyte, B- and T-cell phenotype by multi-parameter flow cytometry)
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2 years
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Assess the metabolomic/transcriptomic profiles of paediatric Sjögren's syndrome endotypes
Time Frame: 2 years
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The pilot data from adults with SS suggest that certain endotypes could be enriched with an interferon-associated signature or metabolomic profile.
Characterisation of these mechanisms could pinpoint additional novel biomarkers for endotype classification and potential novel targets for therapy.
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2 years
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Correlate -omic data with clinical and disease features to define the disease fingerprint associated with paediatric onset SS.
Time Frame: 2 years
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Following initial cleaning of data to remove metabolites/genes/cytokines showing no differences between paediatric SS patients and healthy donors, the investigators will stratify differentially expressed metabolic and genetic markers according to the identified immune-phenotypes and generate a disease fingerprint (combined -omics analysis) for paediatric SS.
Correlations between gene expression and metabolomics and immune-phenotype (cells/cytokines) will be established using regression/cross-correlation analysis.
The investigators will confirm which patterns of immune-phenotype/metabolic/ transcriptomic profiles are most highly associated with childhood-onset compared to adult SS endotypes.
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2 years
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Collaborators and Investigators
Sponsor
Collaborators
Study record dates
Study Major Dates
Study Start (Actual)
Primary Completion (Anticipated)
Study Completion (Anticipated)
Study Registration Dates
First Submitted
First Submitted That Met QC Criteria
First Posted (Actual)
Study Record Updates
Last Update Posted (Actual)
Last Update Submitted That Met QC Criteria
Last Verified
More Information
Terms related to this study
Additional Relevant MeSH Terms
- Pathologic Processes
- Immune System Diseases
- Autoimmune Diseases
- Eye Diseases
- Disease
- Joint Diseases
- Musculoskeletal Diseases
- Rheumatic Diseases
- Connective Tissue Diseases
- Arthritis
- Stomatognathic Diseases
- Mouth Diseases
- Lacrimal Apparatus Diseases
- Arthritis, Rheumatoid
- Xerostomia
- Salivary Gland Diseases
- Dry Eye Syndromes
- Syndrome
- Sjogren's Syndrome
Other Study ID Numbers
- 132557
Plan for Individual participant data (IPD)
Plan to Share Individual Participant Data (IPD)?
IPD Plan Description
Drug and device information, study documents
Studies a U.S. FDA-regulated drug product
Studies a U.S. FDA-regulated device product
This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.
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