Management for adult dermatomyositis

Dermatomyositis is diagnosed and usually comanaged by dermatologists and rheumatologists. Urgently refer a patient for specialist management if dermatomyositis is severe or if there is systemic involvement, especially if speech, swallowing or cardiorespiratory function is affected.

The goal of management in all people with dermatomyositis is recovery of muscle strength and function; see also Nonpharmacological management for idiopathic inflammatory myopathies for advice about exercise.

Immunomodulatory therapy is required for severe or systemic disease; treatment may include:

• high-dose systemic corticosteroids (eg pulse intravenous methylprednisolone or high-dose oral prednisolone [or prednisone])
• intravenous immunoglobulin¹Aggarwal, 2022
• conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) (eg methotrexate, mycophenolate, azathioprine, cyclophosphamide, ciclosporin).

Emerging therapies include biological and targeted-synthetic disease-modifying antirheumatic drugs (b/tsDMARDs) such as rituximabWaldman, 2020, tocilizumabWaldman, 2020 and tofacitinibTakanashi, 2022; however, strong evidence is not available due to the rarity of these conditions and the lack of randomised controlled trials.

Management for the cutaneous features of dermatomyositis may involve potent topical corticosteroids and oral hydroxychloroquine. Preventing solar damage (eg using broad-spectrum sun protection) is also very important, especially for people taking immunomodulatory drugs (particularly azathioprine, mycophenolate and methotrexate).