Sjogren's Syndrome Childhood-onsetPaedSSCoReNCT04762108
Paediatric Sjögren Syndrome Cohort Study and Repository (PaedSSCoRe)
An observational study of Sjogren's Syndrome Childhood-onset, run by University College, London at 8 sites in 2 countries.
Trial 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. Read more about this study
- Who is it for?
- Ages 6 years and older
- Conditions the study is for
- Sjogren's Syndrome Childhood-onset
- How many people
- 300 participants
- Where
- 8 sitesin 2 countries
- Study dates
- 11 Jan 2020to 10 Jan 2030
- How long will I take part?
- 10 Years
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 6 years and older
- All sexes
- Child, Adult, Older adult
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.
Who may be able to join
- 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
Who cannot join
- 1. Inability to provide informed consent or have informed consent provided on their behalf.
Trial locations and contacts
8 sites in 2 countries.
8 sites in 2 countries
United Kingdom7 sites
Cambridge University Hospitals NHS Foundation Trust
Cambridge
Great Ormond Street Hospital For Children NHS Foundation Trust
London
University College London Hospital
London
The Newcastle Upon Tyne Hospitals NHS Foundation Trust
Newcastle
Sheffield Children's NHS Foundation Trust
Sheffield
Sheffield Teaching Hospitals NHS Foundation Trust
Sheffield
University Hospital Southampton NHS Foundation Trust
Southampton
About this study
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.
Read more
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
- Being tested
- Standard of care
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
Standard of careOther
Patients will be given standard of care as per their clinical needs
| Study group | Role | What they receive |
|---|---|---|
| Sjögren's syndrome | Sjögren's syndrome with childhood-onset Receives: Other: Standard of care | |
| Healthy control | Age matched healthy control |
What is being measured
The primary outcome is the main question the study is designed to answer.
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.
Measured: 2 years
Secondary outcomes (3)
Explore immune signatures in a multi-centre cohort of childhood onset Sjögren's syndrome
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)
Measured: 2 years
Assess the metabolomic/transcriptomic profiles of paediatric Sjögren's syndrome endotypes
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.
Measured: 2 years
Correlate -omic data with clinical and disease features to define the disease fingerprint associated with paediatric onset SS.
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.
Measured: 2 years
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted21 Feb 2021
- Study start11 Jan 2020
- Primary completion10 Jan 2030 (estimated)
- Study completion10 Jan 2030 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Sjogren's Syndrome Childhood-onsetStudies of the same condition, open and under way.
- More trials run by University College, LondonThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 300 participants (estimated)
- Target duration
- 10 Years
Sponsor and investigators
- Lead sponsor
- University College, London
- Sponsor type
- University, hospital or other
- Collaborators
- Cambridge University Hospitals NHS Foundation Trust, Children's Health Ireland, Great Ormond Street Hospital, Sheffield Children's NHS Foundation Trust, Sheffield Teaching Hospitals NHS Foundation Trust, Newcastle-upon-Tyne Hospitals NHS Trust, University Hospital Southampton NHS Foundation Trust
- Responsible party
- Sponsor
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT04762108
- Sponsor's protocol ID
- 132557
- Acronym
- PaedSSCoRe
- Status last verified
- Apr 2021
- First submitted
- 15 Feb 2021
- Last updated
- 19 Apr 2021
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No: The study will be complying to General Data Protection Regulation (GDPR) and Data protection regulations and personal identifiable information will not be made available to researchers under any circumstances. Data that will be shared will need to be approved by the steering committee in accordance to these regulations.
Study at a glance
From the registry record, last updated by the sponsor on 19 Apr 2021. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Paediatric Sjögren Syndrome Cohort Study and Repository (PaedSSCoRe)
- ClinicalTrials.gov ID
- NCT04762108
- Acronym / sponsor ID
- PaedSSCoRe / 132557
- Conditions
- Sjogren's Syndrome Childhood-onset
- Study type
- Observational
- Recruitment status
- Active, not recruiting
- Enrolment
- 300 participants (estimated)
- Lead sponsor
- University College, LondonUniversity, hospital or other
- Collaborators
- Cambridge University Hospitals NHS Foundation Trust, Children's Health Ireland, Great Ormond Street Hospital, Sheffield Children's NHS Foundation Trust, Sheffield Teaching Hospitals NHS Foundation Trust, Newcastle-upon-Tyne Hospitals NHS Trust, University Hospital Southampton NHS Foundation Trust
- Study start
- 11 Jan 2020 (actual)
- Primary completion
- 10 Jan 2030 (estimated)
- Study completion
- 10 Jan 2030 (estimated)
- First posted
- 21 Feb 2021 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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 design
- Observational model
- Cohort
- Time perspective
- Prospective
- Target follow-up
- 10 Years
- Number of arms
- 2
| Arm | Type | Receives |
|---|---|---|
| Sjögren's syndrome | – | Other: Standard of careSjögren's syndrome with childhood-onset |
| Healthy control | – | –Age matched healthy control |
Who may qualify
- Ages
- 6 years and older
- Sex
- All
- Age groups
- Child, Adult, Older adult
- 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.
Key inclusion criteria (4 listed)
- 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
Key exclusion criteria (1 listed)
- 1. Inability to provide informed consent or have informed consent provided on their behalf.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| Standard of carePatients will be given standard of care as per their clinical needs | Other | Sjögren's syndrome |
Primary outcome
Provide an estimate of the pre valence of relatively uncommon but serious manifestations of Sjögren's syndrome
Time frame: 2 years
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.
3 secondary outcomes
Explore immune signatures in a multi-centre cohort of childhood onset Sjögren's syndrome
Time frame: 2 years
Assess the metabolomic/transcriptomic profiles of paediatric Sjögren's syndrome endotypes
Time frame: 2 years
Correlate -omic data with clinical and disease features to define the disease fingerprint associated with paediatric onset SS.
Time frame: 2 years
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 19 Apr 2021.
- Individual participant dataSharing planned: No. The study will be complying to General Data Protection Regulation (GDPR) and Data protection regulations and personal identifiable information will not be made available to researchers under any circumstances. Data that will be shared will need to be approved by the steering committee in accordance to these regulations.
Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
The record lists no contact. Use the official record to reach the sponsor.
8 sites in 2 countries: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Sjogren's Syndrome Childhood-onset, run by University College, London at 8 sites in 2 countries.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 4 inclusion and 1 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: under way, not looking for participants, as of the sponsor's last update on 19 Apr 2021. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 8 sites in United Kingdom, Ireland. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Provide an estimate of the pre valence of relatively uncommon but serious manifestations of Sjögren's syndrome (time frame: 2 years). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 19 Apr 2021.