Management for juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is diagnosed and usually managed by paediatric rheumatologists. Management depends on the organ involvement, severity and comorbidities. Urgently refer a patient for specialist management if JDM is severe or if there is systemic involvement, especially if speech, swallowing or cardiorespiratory function is affected. The goal of management in all people, including those with relapsing disease, is recovery of muscle strength and function.

Juvenile dermatomyositis is treated initially with high-dose systemic corticosteroids (eg pulse intravenous methylprednisolone or high-dose oral prednisolone [or prednisone]) with doses tapered in response to recovery of muscle strength. Recovery of muscle strength and function should guide therapy, not normalisation of muscle enzyme concentrations (which tends to occur before clinical response).

Methotrexate is considered the first-line disease-modifying treatment for most children and adolescents with JDMOldroyd, 2022. Combination therapy, using methotrexate or ciclosporin with systemic corticosteroids, has a corticosteroid-sparing effect and is more effective than corticosteroids aloneRuperto, 2016. Other therapies may include intravenous immunoglobulin (IVIg)¹, mycophenolate, rituximab and tumour necrosis factor (TNF) inhibitors. Janus kinase inhibitors (eg tofacitinib) are emerging as important treatments for children and adolescents with refractory disease and JDM-associated interstitial lung diseaseSabbagh, 2019Yu, 2021. Cyclophosphamide may be used in children and adolescents with severe vasculopathy involving the gastrointestinal tract or skin associated with severe ulceration and bleeding.

Management for the skin manifestations of JDM may involve potent topical corticosteroids and oral hydroxychloroquine. Preventing solar damage (eg using broad-spectrum sun protection) is also very important.

No treatment has been consistently reported to be of benefit for subcutaneous calcification, but spontaneous resolution can occur. Surgical excision may be necessary for symptomatic localised lesions.