Recommendations regarding treatment
| L | S | Agreement (%) | |
|---|---|---|---|
| Sun protection, including the routine use of sunblock on sun-exposed areas should be encouraged for patients with JDM. | 4 | D | 100 |
| 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. | 4 | D | 100 |
| Treatment of JDM should include a safe and appropriate exercise programme, monitored by a physiotherapist. | 4 | D | 100 |
| 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. | 1B | A | 100 |
| High-dose corticosteroids should be administered systemically either orally or intravenously in moderate–severe JDM. | 2A | B | 100 |
| High-dose corticosteroids should be administered intravenously if there are concerns about absorption. | 3 | C | 100 |
| Corticosteroid dose should be weaned as the patient shows clinical improvement. | 4 | D | 100 |
| Addition of MTX or ciclosporin A leads to better disease control than prednisolone alone; safety profiles favour the combination of methotrexate and prednisolone. | 1B | A | 100 |
| 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. | 4 | D | 100 |
| 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. | 4 | D | 100 |
| Intravenous immunoglobulin may be a useful adjunct for resistant disease, particularly when skin features are prominent. | 2B-4 | C | 100 |
| MMF may be a useful therapy for muscle and skin disease (including calcinosis). | 3 | C | 100 |
| 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. | 4 | D | 100 |
| In patients who are intolerant to methotrexate, change to another DMARD, including ciclosporin A or MMF. | 3 | C | 100 |
| For patients with severe disease (such as major organ involvement/extensive ulcerative skin disease), addition of intravenous cyclophosphamide should be considered. | 3 | C | 100 |
| 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. | 1B | D | 100 |
| Anti-TNF therapies can be considered in refractory disease; infliximab or adalimumab are favoured over etanercept. | 3 | D | 92 |
| In the presence of developing or established calcinosis, intensification of immunosuppressive therapy should be considered. | 3 | C | 100 |
| 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. | 4 | D | 100 |
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.