Research in JDM

JDCBS Publications

2022

British Society for Rheumatology guideline on management of paediatric, adolescent and adult patients with idiopathic inflammatory myopathy

Lay summary

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)

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Association with HLA-DRβ1 position 37 distinguishes juvenile dermatomyositis from adult-onset myositis

Lay summary

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)

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Juvenile dermatomyositis. Where are we now?

Lay Summary

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.

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2021

The Vasculopathy of Juvenile Dermatomyositis: endothelial injury, hypercoagulability, and increased arterial stiffness.

Lay Summary

Coming soon.

Papadopoulou, C., Hong, Y., Krol, P., Al Obaidi, M., et al.

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Anti-cN-1A Autoantibodies are Absent in Juvenile Dermatomyositis.

Lay Summary

Coming soon.

Rietveld, A., Wienke, J., Visser, E., Vree Egberts, W., et al.

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Identification and prediction of novel classes of long-term disease trajectories for patients with juvenile dermatomyositis using growth mixture models.

Lay Summary

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.

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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

Lay Summary

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.

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Favorable antibody responses to human coronaviruses in children and adolescents with autoimmune rheumatic diseases.

Lay Summary

Coming soon.

Deakin, C. T., Cornish, G. H., Ng, K. W., Faulkner, N., et al.

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100,000 Genomes Pilot on Rare-Disease Diagnosis in Health Care - Preliminary Report

Lay Summary

Coming soon.

100,000 Genomes Project Pilot Investigators, Smedley, D., Smith, K. R., Martin, A., et al.

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Mapping the current psychology provision for children and young people with juvenile dermatomyositis.

Lay Summary

Coming soon.

Livermore, P., Gibson, F., Mulligan, K., Wedderburn, L. R., et al.

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JAK inhibitors: a potential treatment for JDM in the context of the role of interferon-driven pathology

Lay Summary

Coming soon.


Wilkinson, M. G., Deakin, C. T., Papadopoulou, C., Eleftheriou, D.,
 et al.

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Use of Rescue Therapy with IVIG or Cyclophosphamide in Juvenile Myositis.

Lay Summary

Coming soon.


Doudouliaki, T., Papadopoulou, C., Deakin, C. T.

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Treatment of Calcinosis in Juvenile Dermatomyositis

Lay Summary

Coming soon.


Kul Cinar, O., Papadopoulou, C., Pilkington, C. A.

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2020

Using peripheral blood immune signatures to stratify patients with adult and juvenile inflammatory myopathies.

Lay Summary

Coming soon.


Wilkinson, M. G. L., Radziszewska, A., Wincup, C., Ioannou, Y., et al.

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Retrospective analysis of infliximab and adalimumab treatment in a large cohort of juvenile dermatomyositis patients

Lay Summary

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.

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Preexisting and de novo humoral immunity to SARS-CoV-2 in humans.

Lay Summary

Coming soon.


Ng, K. W., Faulkner, N., Cornish, G. H., Rosa, A., et al.

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2019

An international survey of developing classification criteria for juvenile dermatomyositis-scleroderma overlap.

Khaosut, P., et al. (2019)

You give me a name that I can’t say, but I have to explain what it is every day: the power of poetry to share stories from young people with a rare disease.

Livermore, P., Wedderburn, L.R., & Gibson, F. (2019)

Focused HLA analysis in Caucasians with myositis identifies significant associations with autoantibody subtypes.

Rothwell, S., et al. (2019)

Being on the juvenile dermatomyositis rollercoaster: a qualitative study.

Livermore, P., et al. (2019)

Development and validation of a composite disease activity score for measurement of muscle and skin involvement in juvenile dermatomyositis.

Rosina, S., et al. (2019)

2018

A Bayesian semiparametric Markov regression model for juvenile dermatomyositis.

De Iorio, M., et al. (2018)

Clinical signs and symptoms in a joint model of four disease activity parameters in juvenile dermatomyositis: a prospective, longitudinal, multicenter cohort study.

Van Dijkhuizen, E.H.P., et al. (2018)

Systemic and Tissue Inflammation in Juvenile Dermatomyositis: From Pathogenesis to the Quest for Monitoring Tools.

Wienke, J., et al. (2018)

CD19(+) CD24(hi) CD38(hi) B Cells Are Expanded in Juvenile Dermatomyositis and Exhibit a Pro-Inflammatory Phenotype After Activation Through Toll-Like Receptor 7 and Interferon-alpha.

Piper, C.J.M., et al. (2018)

Juvenile dermatomyositis: Latest advances.

Wu, Q., Wedderburn L.R., & McCann, L.J. (2018)

Juvenile dermatomyositis: novel treatment approaches and outcomes

Varnier, G., Pilkington, C.A., & Wedderburn L.R. (2018)

Histological heterogeneity in a large clinical cohort of juvenile idiopathic inflammatory myopathy: analysis by myositis autoantibody and pathological features.

Yasin, S.A., et al. (2018)

Expression of myxovirus-resistance protein A: a possible marker of muscle disease activity and autoantibody specificities in juvenile dermatomyositis.

Soponkanaporn, S., et al. (2018)

The development of an optimal core dataset set in Juvenile Dermatomyositis for clinical use to inform research

McCann, L.J., et al. (2018)

Efficacy and Safety of Cyclophosphamide Treatment in Severe Juvenile Dermatomyositis Shown by Marginal Structural Modeling.

Deakin, C.T., et al. (2018)

2017

Modelling disease activity in juvenile dermatomyositis: A Bayesian approach.

Van Dijkhuizen, E.H.P., et al. (2017)

Effective induction therapy for anti-SRP associated myositis in childhood: A small case series and review of the literature.

Binns, E., et al. (2017)

EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups: a methodology report.

Bottai, M., et al. (2017)

2016 American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.

Aggarwal, R., et al. (2017)

Response to: ‘Antisynthetase syndrome or what else? Different perspectives indicate the need for new classification criteria’ by Cavagna et al.

Lilleker, J. B., et al. (2017)

The EuroMyositis registry: an international collaborative tool to facilitate myositis research.

Lilleker, J. B., et al. (2017)

Autoantibodies in juvenile-onset myositis: Their diagnostic value and associated clinical phenotype in a large UK cohort.

Tansley, S.L., et al. (2017)

Anti-HMGCR autoantibodies in juvenile idiopathic inflammatory myopathies identify a rare but clinically important subset of patients.

Tansley, S.L., et al. (2017)

Consensus – Based Recommendations for the Management of Juvenile Dermatomyositis.

Enders, F.B., et al. (2017)

2016

Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new-onset juvenile dermatomyositis: a randomised trial

Ruperto N., et al. (2016)

Dense genotyping of immune-related loci in idiopathic inflammatory myopathies confirms HLA alleles as the strongest genetic risk factor and suggests different genetic background for major clinical subgroups.

Rothwell, S., et al. (2016)

OP0221 Efficacy and Safety of Tumour Necrosis Factor-Alpha Antagonists in A Large Cohort of Juvenile Dermatomyositis Patients

Campanilho-Marques, R., et al. (2016)

Muscle Biopsy in combination with myositis specific autoantibodies aids prediction of outcome in juvenile dermatomyositis (JDM).

Deakin, C.T., et al. (2016)

2015

Methotrexate polyglutamates as a potential marker of adherence to long-term therapy in children with juvenile idiopathic arthritis and juvenile dermatomyositis: an observational, cross-sectional study.

Hawwa, A.F., et al. (2015)

Development of an internationally agreed minimal dataset for juvenile dermatomyositis (JDM) for clinical and research use.

McCann, L.J., et al. (2015)

Comparing and contrasting clinical and serological features of juvenile and adult-onset myositis: implications for pathogenesis and outcomes.

Tansley, S., & Wedderburn L.R. (2015)

Genome-wide Association Study Identifies HLA 8.1 Ancestral Haplotype alleles as the Major Genetic Risk Factors for Myositis Phenotypes.

Miller, F.W., et al. (2015)

2014

Increased presence of FOXP3+ regulatory T cells in inflamed muscle of patients with active juvenile dermatomyositis compared to peripheral blood.

Vercoulen, Y., et al. (2014)

Calcinosis in Juvenile dermatomyositis is influenced by both anti-NXP2 autoantibody status and age at disease onset.

Tansley, S.L., et al. (2014)

Genotyping of immune-related genetic variants identifies TYK2 as a novel risk locus for idiopathic inflammatory myopathies.

Jani, M., et al. (2014)

Anti-MDA5 autoantibodies in juvenile dermatomyositis identify a distinct clinical phenotype: a prospective cohort study.

Tansley, S., et al. (2014)

Developing a provisional, international Minimal Dataset for Juvenile Dermatomyositis: for use in clinical practice to inform research.

McCann, L.J., et al. (2014)

2013

Morphometric analyses of normal pediatric brachial biceps and quadriceps muscle tissue.

Sallum, A.M.E., et al. (2013)

Limb girdle muscular dystrophy type 2B masquerading as inflammatory myopathy: case report

Jethwa, H., et al. (2013)

Adult and Juvenile Dermatomyositis: are the distinct clinical features explained by our current understanding of serological subgroups and pathogenic mechanisms?

Tansley, S.L., McHugh, N.J., & Wedderburn, L.R. (2013)

The PRINTO criteria for clinically inactive disease in juvenile dermatomyositis.

Lazarevic, D., et al. (2013)

Myeloid related proteins induce muscle derived inflammatory mediators in Juvenile Dermatomyositis.

Nistala, K., et al. (2013)

Validation of a score tool for measurement of pathological severity in juvenile dermatomyositis and correlation with clinical severity of disease.

Varsani, H., et al. (2013)

Update in Juvenile Dermatomyositis.

Nistala, K., & Wedderburn, L.R. (2013)

Genome-wide association study of dermatomyositis reveals genetic overlap with other autoimmune disorders.

Miller, F.W., et al. (2013)

2012

Maternal microchimerism in muscle biopsies from children with juvenile dermatomyositis.

Ye, Y., et al. (2012)

Genetic Association Study of NF-kB genes in UK Caucasian Adult and Juvenile Onset Idiopathic Inflammatory Myopathy.

Chinoy, H., et al. (2012)

Juvenile Dermatomyositis: new insights and new treatment strategies. Therapeutic Advances in Musculoskeletal Disease.

Martin, N., Li, C.K., & Wedderburn, L.R. (2012)

Comparison of children with onset of Juvenile Dermatomyositis symptoms before or after their fifth birthday in the UK and Ireland JDM Cohort study.

Martin, N., et al. (2012)

2011

Comparison of clinical features and drug therapies among European and Latin American patients with juvenile dermatomyositis.

Guseinova, D., et al. (2011)

A National Registry for Juvenile Dermatomyositis and other Paediatric Idiopathic Inflammatory Myopathies: 10 years experience; The Juvenile Dermatomyositis National (UK and Ireland) Cohort Biomarker Study and Repository for Idiopathic Inflammatory Myopathies.

Martin, N., et al. (2011)

Assessment of active inflammation in juvenile dermatomyositis: a novel magnetic resonance imaging-based scoring system.

Davis, W.R., et al. (2011)

2010

Juvenile dermatomyositis with tongue calcinosis and poor growth.

Maritsi, D., Leahy, A., & Pilkington, C.A. (2010)

The Paediatric Rheumatology International Trials Organisation provisional criteria for the evaluation of response to therapy in juvenile dermatomyositis. A Paediatric Rheumatology International Trials Organisation (PRINTO); Pediatric Rheumatology Collaborative Study Group (PRCSG)

Ruperto, N., et. al. (2010)

Treatment approaches to juvenile dermatomyositis (JDM) across North America: The Childhood Arthritis and Rheumatology Research Alliance (CARRA) JDM Treatment Survey.

Stringer, E., et al. (2010)

A Long-term outcome and prognostic factors of juvenile dermatomyositis: A multinational, multicenter study of 490 patients.

Ravelli, A., et al. (2010)

Protocols for the initial treatment of moderately severe juvenile dermatomyositis: results of a Children’s Arthritis and Rheumatology Research Alliance Consensus Conference.

Huber, A.M., et al. (2010)

2009

Up-regulation of MHC class I in transgenic mice results in reduced force-generating capacity in slow-twitch muscle.

Salomonsson, S., et al. (2009)

Health-related quality of life of patients with juvenile dermatomyositis: results from the Pediatric Rheumatology International Trials Organisation multinational quality of life cohort study.

Apaz, M.T., et al. (2009)

HLA-DPB1 associations differ between DRB1*03 positive anti-Jo-1 and anti-PM-Scl antibody positive idiopathic inflammatory myopathy.

Chinoy, H., et al. (2009)

Autoantibodies to a 140-kd Protein in Juvenile Dermatomyositis Are Associated With Calcinosis.

Gunawardena, H., et al. (2009)

Over expression of MHC class l heavy chain protein in young skeletal muscle leads to severe myositis: implications for juvenile myositis.

Li, C.K., et al. (2009)

Juvenile dermatomyositis: extra-muscular manifestations and their management.

Lowry, C.A., & Pilkington, C.A. (2009)

Juvenile Dermatomyositis: New Developments in Pathogenesis, Assessment and Treatment.

Wedderburn, L.R., & Rider, L.G. (2009)

2008

Age dependent inhibition of ectopic calcification: A possible role for Fetuin-A and Osteopontin in patients with Juvenile Dermatomyositis with Calcinosis.

Marhaug, G., et al. (2008)

Effectiveness of infliximab in the treatment of refractory juvenile dermatomyositis with calcinosis.

Riley, P., et al. (2008)

Heat Shock Protein 60 in Inflamed Muscle Tissue is the Target of Regulatory Auto Reactive T cells in Patients with Juvenile Dermatomyositis.

Elst, E.F., et al. (2008)

Clinical associations of autoantibodies to a p155/140 kDa doublet protein in juvenile dermatomyositis.

Gunawardena, H., et al. (2008)

Quantification of normal range of inflammatory changes in morphologically normal pediatric muscle.

Varsani, H., et al. (2008)

The PTPN22 gene is associated with juvenile and adult UK Caucasian idiopathic inflammatory myopathy independent of the HLA 8.1 haplotype.

Chinoy, H., et al. (2008)

2007

International consensus on a proposed score system for muscle biopsy evaluation in patients with JDM, for potential use in clinical trials.

Wedderburn, L.R., et al. (2007)

Oropharyngeal dysphagia in juvenile dermatomyositis (JDM): an evaluation of videofluoroscopy swallow study (VFSS) changes in relation to clinical symptoms and objective muscle scores.

McCann, L.J., et al. (2007)

HLA class II haplotype and autoantibody associations in children with juvenile dermatomyositis and juvenile dermatomyositis-scleroderma overlap.

Wedderburn, L.R., et al. (2007)

Failure to over express MHC class l on muscle biopsy in a case of amyopathic juvenile dermatomyositis.

McCann, L.J., et al. (2007)

2006

The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland) – clinical characteristics of children recruited within the first 5 years.

McCann, L.J., et al. (2006)

An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM).

Brown, V.E., et al. (2006)

2005

Quantitative Assessments of the Effects of Exercise on Muscles in Juvenile Dermatomyositis.

Maillard, S.M., et al. (2005)

Paediatric Idiopathic Inflammatory Muscle Disease: Recognition and Management.

Pilkington, C.A., & Wedderburn, L.R. (2005)

Importance of aggressive treatment in juvenile dermatomyositis.

Vojinovic, J., et al. (2005)

2004

MHC Class I Over-expression on Muscles in early Juvenile Dermatomyositis.

Li, C., et al. (2004)

Intravenous cyclophosphamide pulse therapy in juvenile dermatomyositis. A review of efficacy and safety.

Riley, P., et al. (2004)

Quantitative assessment of MRI T2 relaxation time of thigh muscles in children with dermatomyositis.

Maillard, S.M., et al. (2004)

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