Paediatric Sjögren Syndrome Cohort Study and Repository (PaedSSCoRe)
试验速览
- 阶段
- 不适用
- 状态
- 进行中（未招募）
- 入组人数
- 300
- 试验地点
- 8
- 主要终点
- Provide an estimate of the pre valence of relatively uncommon but serious manifestations of Sjögren's syndrome
研究概览
简要总结
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.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 6 Years 至 —（Child, Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •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
排除标准
- •Inability to provide informed consent or have informed consent provided on their behalf.
结局指标
主要结局
Provide an estimate of the pre valence of relatively uncommon but serious manifestations of Sjögren's syndrome
时间窗: 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.
次要结局
- Explore immune signatures in a multi-centre cohort of childhood onset Sjögren's syndrome(2 years)
- Correlate -omic data with clinical and disease features to define the disease fingerprint associated with paediatric onset SS.(2 years)
- Assess the metabolomic/transcriptomic profiles of paediatric Sjögren's syndrome endotypes(2 years)