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  • Oral presentation
  • Open Access

Effectiveness of anti-TNF-α agents in the treatment of refractory juvenile dermatomyositis

  • 1Email author,
  • 2,
  • 3,
  • 1 and
Pediatric Rheumatology20119 (Suppl 1) :O29

https://doi.org/10.1186/1546-0096-9-S1-O29

  • Published:

Keywords

  • Infliximab
  • Myopathy
  • Adalimumab
  • Skin Disease
  • Calcinosis

Background

Juvenile dermatomyositis (JDM) is a rare, chronic inflammatory disease. Anti-TNF-α agents are increasingly being used to treat disease that is refractory to other treatments. There is a lack of literature regarding the effectiveness of anti-TNF-α agents in JDM.

Aim

To assess the response of refractory JDM patients to anti-TNF-α agents.

Methods

The Juvenile Dermatomyositis National (UK and Ireland) Cohort Biomarker Study and Repository for Idiopathic Inflammatory Myopathies database was searched for patients treated with anti-TNF-α agents.

Results

INFLIXIMAB: 28/30(93%) patients had data available. Indicators for starting infliximab were: muscle weakness (70%), non-ulcerative skin disease (57%), calcinosis (33%), and nail fold changes (30%). All patients with a low CMAS (n=18) improved. Physician VAS improved in 20/26(77%), CHAQ score in 11/16(69%), skin disease in 19/23(83%), calcinosis in 6/13(46%) and muscle enzymes in 6/10(60%). Prednisolone dose decreased in 17/21(81%).

ADALIMUMAB: 10/11(91%) had previously been treated with infliximab. 6/10(60%) were changed to improve disease control: 4 had persistent skin disease, 2 improved; 3 had progressive calcinosis, 1 improved, 2 remained stable. 8/9(89%) maintained previous gains or made further improvement in other parameters other than skin and calcinosis.

ETANERCEPT: 4/7(57%) patients had data available. 2 improved, 2 were switched to infliximab, one for increasing calcinosis, one for compliance issues.

Conclusions

Infliximab provides clinical benefit to patients with JDM refractory to other treatments; particularly muscle weakness. Switching to adalimumab benefited some patients; gains made on infliximab were maintained in most cases.

Authors’ Affiliations

(1)
Rheumatology Department, Great Ormond Street Hospital, London, UK
(2)
Rheumatology Unit, UCL Institute of Child Health, University College London, UK
(3)
Rheumatology Department, Birmingham Children’s Hospital NHS Foundation Trust, UK

Copyright

© Boulter et al; licensee BioMed Central Ltd. 2011

This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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