Recommendations for the diagnosis, treatment, and management of juvenile dermatomyositis (JDM) have been established by a group of European experts in pediatric rheumatology. The guidelines come from the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE) initiative and have been recently published in the Annals of the Rheumatic Diseases.1

The result was a 3-part consensus document (>80% agreement) in JDM management that included overarching principles, recommendations for diagnosis, and recommendations for treatment. The enumerated points were all developed using the European League Against Rheumatism (EULAR) procedures for creating best practice recommendations.2,3

The committee of 19 experts in pediatric rheumatology was joined by 2 experts in exercise physiology and physical therapy to develop the JDM recommendations.

“Clear recommendations can help clinicians in the care of patients with JDM, as no international consensus regarding diagnosis and treatment is currently available and management therefore varies,” the panel wrote. “Standardization of diagnostic tests and treatment regimens will enable collaborative research studies to increase knowledge of this rare disease,” they added.

Recommendations In Brief

High-Yield Data Summary

  • Juvenile dermatomyositis should be managed through close monitoring of patients’ disease status and well-being by an experienced multidisciplinary team.

Overarching Principles

  1. All children with suspected idiopathic inflammatory myopathies should be referred to a specialized center.
  2. High-risk patients require immediate/urgent referral to a specialized center.
  3. Patient- and parent-reported outcome measures should be used for assessing disease activity during diagnosis and disease monitoring.
  4. Use validated tools to measure health status.
  5. Regularly assess disease activity (muscle, skin, major organs) using standardized methods.
  6. Disease damage should be assessed at least once a year using a standardized measure.
  7. All patients should have the opportunity to register within a research registry/repository.

Diagnosis Recommendations 

  • General recommendations include alternate diagnoses to consider and a list of diagnostic investigations.
  • Specific recommendations include assessment of muscle disease, skin disease, JDM-associated lung disease, JDM-associated cardiac disease, calcinosis, biomarkers, and autoantibodies.

Treatment Recommendations

The recommendations are broken down into 2 areas, with flow charts provided to guide clinical decision-making in treatment of mild/moderate (Figure 1A) or severe (Figure 1B) disease in patients with newly diagnosed and treatment-refractory JDM.

Figure 1A. Flow chart for the treatment of mild/moderate disease in newly diagnosed and refractory patients with juvenile dermatomyositis (JDM). Image by BMJ Publishing Group Ltd and the European League Against Rheumatism is licensed under CC BY-NC 4.0. Copyright © 2017. (Click image to enlarge)


Figure 1B. Flow chart for the treatment of severe disease in newly diagnosed and refractory patients with juvenile dermatomyositis (JDM). Image by BMJ Publishing Group Ltd and the European League Against Rheumatism is licensed under CC BY-NC 4.0. Copyright © 2017. (Click image to enlarge)

Summary and Clinical Applicability

Aggressive treatment of JDM may prevent organ damage and subsequent complications, yet acknowledged that treatment currently relies on the experience of the treating rheumatologist. It advised that JDM should be managed with a multidisciplinary approach that includes pediatric rheumatologists, specialist nurses, physiotherapists, and other specialists as needed.

“Close monitoring of patients’ disease status and well-being by an experienced multidisciplinary team is essential for a good clinical outcome,” the recommendations committee noted, adding that “Recent evidence highlights the importance of treating skin disease aggressively as it is associated with high morbidity.”

Disclosures

Dr Baildam is on the speakers bureau for Roche/Chugai and is an advisory board member for AbbVie and Pfizer. Dr Feldman is a consultant for Novartis, Pfizer, and Bristol-Myers Squibb. Dr Lahdenne is a consultant for Bristol-Myers Squibb and Pfizer. Dr Ozen is a consultant for Novartis and is on the speakers bureau for Sobi. Dr. Pilkington is a consultant for Roche; is on the speakers bureau for Pfizer and Novartis; and has received grant support from Sobi, Novartis, AbbVie, Roche, Pfizer, and Medac. Dr Ravelli has received grant/research support from Pfizer and The Myositis Association; is a consultant for Novartis and Roche; and is on the speakers bureau for AbbVie, Novartis, Pfizer, and Roche. Dr Uziel is a consultant for Novartis and is on the speakers bureau for AbbVie, Neopharm, Novartis, and Roche. Dr Wulffraat has received grant/research support from EAHC, AbbVie, GSK, and Roche and is a consultant for Genzyme, Novartis, Pfizer, and Roche.

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References

  1. Enders FB, Bader-Meunier B, Baildam E, et al. Consensus-based recommendations for the management of juvenile dermatomyositis. Ann Rheum Dis. 2017;76:329-340.
  2. Dougados M, Betteridge N, Burmester GR, et al. EULAR standardised operating procedures for the elaboration, evaluation, dissemination, and implementation of recommendations endorsed by the EULAR standing committees. Ann Rheum Dis. 2004;63:1172-1176.
  3. van der Heijde D, Aletaha D, Carmona L, et al. 2014 update of the EULAR standardised operating procedures for EULAR-endorsed recommendations. Ann Rheum Dis. 2015;74:8-13.

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