Table 3

Recommendations regarding treatment

LSAgreement (%)
Sun protection, including the routine use of sunblock on sun-exposed areas should be encouraged for patients with JDM.4D100
When treating patients with JDM, it is particularly important to have a physiotherapist and a specialist nurse actively involved as part of a multidisciplinary team.4D100
Treatment of JDM should include a safe and appropriate exercise programme, monitored by a physiotherapist.4D100
We recommend the induction regimen for treatment of new onset patients with JDM to be based on high dose of corticosteroids (oral or intravenous) combined with MTX.1BA100
High-dose corticosteroids should be administered systemically either orally or intravenously in moderate–severe JDM.2AB100
High-dose corticosteroids should be administered intravenously if there are concerns about absorption.3C100
Corticosteroid dose should be weaned as the patient shows clinical improvement.4D100
Addition of MTX or ciclosporin A leads to better disease control than prednisolone alone; safety profiles favour the combination of methotrexate and prednisolone.1BA100
MTX should be started at a dose of 15–20 mg/m2/week (max absolute dose of 40 mg /week) preferably administered subcutaneously at disease onset.4D100
If a newly diagnosed patient has inadequate response to treatment, intensification of treatment should be considered within the first 12 weeks, after consultation with an expert centre.4D100
Intravenous immunoglobulin may be a useful adjunct for resistant disease, particularly when skin features are prominent.2B-4C100
MMF may be a useful therapy for muscle and skin disease (including calcinosis).3C100
Ongoing skin disease reflects ongoing systemic disease and therefore should be treated by increasing systemic immunosuppression. Topical tacrolimus (0.1%)/topical steroids may help localised skin disease, particularly for symptomatic redness or itching.4D100
In patients who are intolerant to methotrexate, change to another DMARD, including ciclosporin A or MMF.3C100
For patients with severe disease (such as major organ involvement/extensive ulcerative skin disease), addition of intravenous cyclophosphamide should be considered.3C100
B cell depletion therapy (rituximab) can be considered as an adjunctive therapy for those with refractory disease. Clinicians should be aware that rituximab can take up to 26 weeks to work.1BD100
Anti-TNF therapies can be considered in refractory disease; infliximab or adalimumab are favoured over etanercept.3D92
In the presence of developing or established calcinosis, intensification of immunosuppressive therapy should be considered.3C100
There is no high-level evidence of when to stop therapy; however, consideration may be given to withdrawing treatment if a patient has been off steroids and in remission on methotrexate (or alternative DMARD) for a minimum of 1 year.4D100
  • Agreement indicates percentage of experts that agreed on the recommendation during the final voting round of the consensus meeting.

  • 1A, meta-analysis of randomised controlled trial; 1B, randomised controlled study; 2A, controlled study without randomisation; 2B, quasi-experimental study; 3, descriptive study; 4 expert opinion; A, based on level 1 evidence; B, based on level 2 or extrapolated from level 1; C, based on level 3 or extrapolated from level 1 or 2; D, based on level 4 or extrapolated from level 3 or 4 expert opinion; DMARD, disease-modifying antirheumatic drug; JDM, juvenile dermatomyositis; L, level of evidence; MMF, mycophenolate mofetil; MTX, methotrexate; S, strength of recommendation; TNF, tumour necrosis factor.