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Treatment of Juvenile Dermatomyositis: An Update

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Abstract

The idiopathic inflammatory myopathies of childhood consist of a heterogeneous group of autoimmune diseases characterised by proximal muscle weakness and pathognomonic skin rashes. The overall prognosis of juvenile myositis has improved significantly over recent years, but the long-term outcome differs substantially from patient to patient, suggestive of distinct clinical phenotypes with variable responses to treatment. High doses of corticosteroids remain the cornerstone of therapy along with other immunosuppressant therapies depending on disease severity and response. The advent of biological drugs has revolutionised the management of various paediatric rheumatologic diseases, including inflammatory myopathies. There are few data from randomised controlled trials to guide management decisions; thus, several algorithms for the treatment of juvenile myositis have been developed using international expert opinion. The general treatment goals now include elimination of active disease and normalisation of physical function, so as to preserve normal growth and development, and to prevent long-term damage and deformities. This review summarises the newer and possible future therapies of juvenile inflammatory myopathies, including evidence supporting their efficacy and safety.

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Authors and Affiliations

  1. UCL Great Ormond Street Institute of Child Health, 30 Guilford St, London, WC1N 1EH, UK

    Charalampia Papadopoulou & Lucy R. Wedderburn

  2. Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK

    Charalampia Papadopoulou & Lucy R. Wedderburn

  3. NIHR GOSH Biomedical Research Centre (BRC), London, UK

    Lucy R. Wedderburn

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  1. Charalampia Papadopoulou
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Correspondence to Lucy R. Wedderburn.

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LW is supported by Arthritis Research UK, Great Ormond Street Hospital (GOSH) Children’s Charity and the NIHR Biomedical Research Centre at GOSH.

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Charalampia Papadopoulou and Lucy R. Wedderburn have no conflicts of interest to declare.

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Papadopoulou, C., Wedderburn, L.R. Treatment of Juvenile Dermatomyositis: An Update. Pediatr Drugs 19, 423–434 (2017). https://doi.org/10.1007/s40272-017-0240-6

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