Treatment of inflammatory myopathy: emerging therapies and therapeutic targets

Abstract

Despite the lack of placebo-controlled trials, glucocorticoids are considered the mainstay of initial treatment for idiopathic inflammatory myopathy and myositis-associated interstitial lung disease. Glucocorticoid-sparing agents are often given concomitantly with other immunosuppressive agents, particularly in patients with moderate or severe disease. First-line conventional immunosuppressive drugs include either methotrexate or azathioprine, and when they fail, more aggressive therapy includes mycophenolate mofetil, tacrolimus or cyclosporine, intravenous immunoglobulin, rituximab, or cyclophosphamide, used alone or in various combinations. Further investigations are required to assess the role of more novel therapies in the treatment of myositis and myositis-associated interstitial lung disease.

Keywords:

• dermatomyositis
• idiopathic inflammatory myopathy
• myositis
• polymyositis
• treatment

Financial & competing interests disclosure

R Aggarwal has received research grants from Pfizer, Mallinckrodt and Genentech. CV Oddis has received research grants from Genentech, Mallinckrodt, and Novartis. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Key issues

• Despite the lack of controlled trials, systemic glucocorticoids are considered the mainstay of initial treatment of inflammatory myopathies (IIMs) and myositis-associated interstitial lung disease.

• Glucocorticoid-sparing agents, either methotrexate or azathioprine, are often started concomitantly with systemic glucocorticoids, particularly in IIM patients with moderate or severe disease.

• In patients who do not show adequate response to systemic glucocorticoids combined with methotrexate or azathioprine, other conventional or biologic immunosuppressive or immunomodulatory agents are sequentially used alone or in various combinations.

• The treatment of refractory IIMs can be challenging, and efficacy data on novel drugs including biologic agents remains limited.

Notes