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Use of Rescue Therapy with IVIG or Cyclophosphamide in Juvenile Myositis

  • Pediatric Rheumatology (S Ozen, Section Editor)
  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

This review aims to present the literature available to date on the use of intravenous immunoglobulin and cyclophosphamide for juvenile patients with inflammatory myopathies, to evaluate the strength of the evidence so far for both these medications, and to reach conclusions about their efficacy.

Recent Findings

Juvenile idiopathic inflammatory myopathies, mainly represented by juvenile dermatomyositis (JDM), are rare diseases but quite debilitating for the patients. JDM is an autoimmune condition with predominantly muscle and skin involvement but also systemic features affecting the cardiovascular, respiratory, and gastrointestinal systems. The mainstay therapy is based on corticosteroids and methotrexate, but often other therapeutic alternatives are sought for patients with severe or refractory disease. The rarity of these conditions makes research for new medications even more challenging. Innovative trial designs or statistical methods can be used to emulate a randomized study and investigate drug effectiveness.

Summary

Despite the lack of Level I evidence on the use and efficacy of intravenous immunoglobulin and cyclophosphamide, their use is advocated by a substantial number of case reports and case series as well as analyses using marginal structural models.

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Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Authors and Affiliations

  1. Department of Rheumatology, Great Ormond Street Hospital NHS Foundation Trust, London, UK

    Theonymfi Doudouliaki & Charalampia Papadopoulou

  2. Infection, Immunity and Inflammation Research and Teaching Department, UCL, Great Ormond Street Institute of Child Health, 30 Guilford Street, London, WC1N 1EH, UK

    Charalampia Papadopoulou & Claire T. Deakin

  3. Centre for Adolescent Rheumatology Versus Arthritis at UCL, UCLH and GOSH, London, UK

    Claire T. Deakin

  4. NIHR Biomedical Research Centre at Great Ormond Street Hospital, London, UK

    Claire T. Deakin

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  1. Theonymfi Doudouliaki
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Correspondence to Claire T. Deakin.

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Doudouliaki, T., Papadopoulou, C. & Deakin, C.T. Use of Rescue Therapy with IVIG or Cyclophosphamide in Juvenile Myositis. Curr Rheumatol Rep 23, 24 (2021). https://doi.org/10.1007/s11926-021-00990-3

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