Fig. 1

Treatment strategies in juvenile dermatomyositis (JDM). Patients with new-onset active JDM are treated with 1 of 3 glucocorticoid regimens and always receive an additional disease-modifying antirheumatic drug, preferably methotrexate with or without intravenous immune globulins. If the predefined treatment targets are achieved, glucocorticoids are tapered according to a scheduled outlined in Table 3. If treatment targets are not reached, a modification in therapy is needed, i.e. either addition of a new therapy or switching therapy. There was no consensus as to a specific sequence of changes in therapy. Patients may also be treated according to these strategies if they have pre-existing, active JDM (see right side of this figure). *Repeated cycles of intravenous methylprednisolone 20–30 mg/kg (max. 1000 mg) daily for 3–5 days in a row. **Prednisone equivalent 0.5–2 mg/kg (max. 80 mg) daily. †Prednisone equivalent 0.2–0.5 mg/kg daily. ‡Prednisone equivalent 2 mg/kg (max. 80 mg) daily. ¶Single cycle of intravenous methylprednisolone 20–30 mg/kg (max. 1000 mg) daily for 3–5 days. Abbreviations: AZA, azathioprin; CSA, cyclosporin A; CYC, cyclophosphamide; DMARD, disease-modifying antirheumatic drug; GC, glucocorticoids; IVIG, intravenous immune globulins; IVMP, intravenous methylprednisolone pulse; JDM, juvenile dermatomyositis; MTX, methotrexate; MMF, mycophenolate mofetil; RTX, rituximab; s.c., subcutaneously; TNFi, tumor necrosis factor inhibitor