Research in JDM
British Society for Rheumatology guideline on management of paediatric, adolescent and adult patients with idiopathic inflammatory myopathy
The BSR Guideline on managing myositis is the first of its kind to address management of myositis in children, adolescents and adults in one combined resource. It was produced by a highly mutli disciplinary team of health care professionals, as well as patients and carers. The JDM Cohort and Biomarker Study Steering Committee specifically endorsed this guideline.
Oldroyd, A. G. S., Lilleker, J. B., Amin, T., Aragon, O., et al. (2022)
Association with HLA-DRβ1 position 37 distinguishes juvenile dermatomyositis from adult-onset myositis
Working with collaborators in the US, Canada and Norway, we assembled the largest number of samples from patients with JDM that have ever been used for a genetic study. We found that patients with JDM were more likely to have changes in a gene that has an important role in identifying molecules that have come from bacteria or viruses and then triggering an immune response. This information might be useful for helping us to understand what causes JDM and what makes JDM different from adult myositis.
Deakin, C.T., Bowes, J., Rider, L. G., Miller, F. W., et al. (2022)
Juvenile dermatomyositis. Where are we now?
This review focuses on the recent developments in the understanding of juvenile dermatomyositis (JDM). Describing new insights into JDM for long-term outlook, disease course and health-related quality of life. Additionally, highlighting new, emerging treatments.
McCann, L. J., Livermore, P., Wilkinson, M. G. L., Wedderburn, L. R.
The Vasculopathy of Juvenile Dermatomyositis: endothelial injury, hypercoagulability, and increased arterial stiffness.
Papadopoulou, C., Hong, Y., Krol, P., Al Obaidi, M., et al.
Anti-cN-1A Autoantibodies are Absent in Juvenile Dermatomyositis.
Rietveld, A., Wienke, J., Visser, E., Vree Egberts, W., et al.
Identification and prediction of novel classes of long-term disease trajectories for patients with juvenile dermatomyositis using growth mixture models.
The uncertainty of long-term outcomes is a difficulty for patients and families following a new diagnosis of JDM. Using data from the UK JDM Cohort and biomarker study (supported by Myositis UK), we identified two groups of patients with distinct patterns of disease trajectories over time. Most patients (89%) had a milder disease course, but a minority of patients (11%) experienced ongoing severe disease. We also showed that patients whose skin and lungs are affected in certain ways when they are diagnosed are at higher risk of developing ongoing severe disease. This research helps us understand the range of disease courses in JDM. We hope that this research might help identify patients who need more aggressive treatment at an early stage of their disease.
Deakin, C. T., Papadopoulou, C., McCann, L. J., Martin, N., et al.
A survey to understand the feelings towards and impact of COVID-19 on the households of juvenile dermato myositis patients from a parent or carer perspective
The aim of this study was to gain a better understanding of how parents and carers feel about the effects and impact of the COVID-19 pandemic ‘lock down’ and how this impacted upon their child or young person with Juvenile Dermatomyositis (JDM). We approached by email 139 participants from the Juvenile Dermatomyositis Cohort Biomarker Study (JDCBS). We asked their parent and carers to complete a questionnaire that consisted of 20 questions about the impact of the pandemic on their child or young person’s clinical care. Results showed that COVID-19 has disrupted the treatment of JDM. Parents and carers are worried, concerned and anxious about the effects of COVID-19 on their child or young person. Parents and carers had access to enough, useful information to support their child or young person with JDM. The uncertainties during this time need to continue to be addressed so that we can adapt the care and support for JDM patients.
Wilkinson, M. G. L., Wu, W., O’Brien, K., Deakin, C. T., et al.
Favorable antibody responses to human coronaviruses in children and adolescents with autoimmune rheumatic diseases.
Deakin, C. T., Cornish, G. H., Ng, K. W., Faulkner, N., et al.
100,000 Genomes Pilot on Rare-Disease Diagnosis in Health Care - Preliminary Report
100,000 Genomes Project Pilot Investigators, Smedley, D., Smith, K. R., Martin, A., et al.
Mapping the current psychology provision for children and young people with juvenile dermatomyositis.
Livermore, P., Gibson, F., Mulligan, K., Wedderburn, L. R., et al.
JAK inhibitors: a potential treatment for JDM in the context of the role of interferon-driven pathology
Wilkinson, M. G., Deakin, C. T., Papadopoulou, C., Eleftheriou, D., et al.
Use of Rescue Therapy with IVIG or Cyclophosphamide in Juvenile Myositis.
Doudouliaki, T., Papadopoulou, C., Deakin, C. T.
Treatment of Calcinosis in Juvenile Dermatomyositis
Kul Cinar, O., Papadopoulou, C., Pilkington, C. A.
Using peripheral blood immune signatures to stratify patients with adult and juvenile inflammatory myopathies.
Wilkinson, M. G. L., Radziszewska, A., Wincup, C., Ioannou, Y., et al.
Retrospective analysis of infliximab and adalimumab treatment in a large cohort of juvenile dermatomyositis patients
In rare diseases like JDM, it can be difficult to get evidence for whether new medicines work. In this paper, using data from the UK JDM Cohort and biomarker study (supported by Myositis UK), we describe the clinical scores of patients with JDM who were treated with two different antibody drugs called infliximab and adalimumab, which target the “TNF” molecule. Measures of skin, muscle and global disease improved over time in patients treated with these drugs. The evidence isn’t as strong as a clinical trial, because there wasn’t a group of patients who didn’t receive these drugs to compare to. Nevertheless, we show these drugs are safe and that there may be some benefit for patients with JDM who take them.
Campanilho-Marques, R., Deakin, C. T., Simou, S., Papadopoulou, C., et al.
Preexisting and de novo humoral immunity to SARS-CoV-2 in humans.
Ng, K. W., Faulkner, N., Cornish, G. H., Rosa, A., et al.
Development and validation of a composite disease activity score for measurement of muscle and skin involvement in juvenile dermatomyositis.
Update in Juvenile Dermatomyositis.
Assessment of active inflammation in juvenile dermatomyositis: a novel magnetic resonance imaging-based scoring system.
Juvenile Dermatomyositis: New Developments in Pathogenesis, Assessment and Treatment.