Juvenile-onset systemic lupus erythematosus (JSLE)

JSLE (commonly referred to as Childhood Lupus) is a chronic autoimmune disease in children that can affect any part of the body including skin, joints and other major organs. Between 15-20% of lupus patients develop the disease in childhood.

Despite advances in our understanding of JSLE, the exact underlying causes remain unknown. Based on the facts that disease activity is higher, organ complications are more common and severe, and gender distribution is distinctly different in children when compared to adults, it is striking that diagnosis and treatment are based on observations in adult SLE patients.

Liverpool is the UK’s only Centre of Excellence for Childhood Lupus and it is the national lead for the UK JSLE Study Group. It also hosts the National Coordinating Centre for the UK JSLE Cohort Study and Repository (CI: M. Beresford), exploring the clinical and immunopathological characteristics of this disease and fostering a robust translational program of research directed towards clinical trials in JSLE.

The EATC4Children is involved in trying to understand disease mechanisms and develop biomarkers for early diagnosis of disease.

‘Living with Lupus’

Developing treatment targets and outcome measures with children and young people to TARGET LUPUS (‘Targeting disease, Agreeing Recommendations and reducing Glucocorticoids through Effective Treatment, in LUPUS’). This study aims to explore, in-depth, the views of JSLE patients/parents on the treatment targets and outcome measures being considered by TARGET LUPUS treat to target study (CI: E. Smith).


BPSU JSLE Study

This study looks at the incidence and prevalence of JSLE in the UK and builds on the work of the UK JSLE Cohort Study. Not only is this study important to deliver key data in this population group but it has also provided an important training opportunity for trainees/new principal investigators (CI’s: H. Lythgoe M. Beresford).


Tracking cancer and mortality in UK children and young people with lupus

This study is part of the UK JSLE Cohort Study and Repository and aims to investigate mortality rates and cancer incidence in JSLE (CI: M. Beresford).


Masterplans (MAximizing Sle ThERapeutic Potential by Application of Novel and Stratified approaches)

Masterplans is a stratified medicine consortium that is studying how different groups of patients within the lupus community respond to specific treatments. The aim is to identify markers of systemic and renal inflammation and their potential as predictors of treatment responses (to Mycophenolate Mofetil, Belimumab and Rituximab). This work will be split into two parts: examination of the pro-inflammatory S100 proteins in urine and plasma, and testing a six protein urine biomarker panel, which has previously been evaluated in children (CI: M. Beresford, Co-CI: C. Hedrich, Co-CI: A. Midgley).


Epigenetics and Lupus

This study examines the effects of CREMα on epigenetic events in T lymphocytes (CI: C. Hedrich).


Genetic factors in juvenile-onset systemic lupus erythematosus; future guides for individualized treatment

This study involves the genotyping of 400 JSLE patients, which inform biomarker development, patient stratification, and future experimental medicine studies (CI’s: M. Beresford and C. Hedrich).


Investigating Serum Protein Expression in Patients with Juvenile Onset Systemic Lupus Erythematosus pre- and post-puberty

Mechanisms driving JSLE may be different depending on the age at disease onset. Children that develop lupus before puberty may be influenced more by genetic changes and children that develop lupus after puberty may require hormonal or environmental factors. Meso Scale Discovery multiplex technology is being used to measure the expression of 71 different proteins in the serum of JSLE patients and healthy controls pre- and post-puberty (CI: A. Midgley, Co-CI: C. Hedrich). 


Professor Christian Hedrich and Dr Eve Smith discuss the challenges of treating childhood-onset lupus.

They are joined by Sammy Ainsworth, Youth and Family Participation Officer.

 

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