Figure 2 Proposed approach to treating myositis-associated interstitial lung disease Figure 2 | Proposed approach to treating myositis-associated interstitial lung disease. The treatment of interstitial lung disease (ILD) can be broken down into treating either mild to moderate disease or severe disease119. Treatment of mild to moderate disease includes an aggressive approach involving high-dose glucocorticoids (either oral or intravenous administration) as an induction therapy followed by maintenance therapy with slowly tapering glucocorticoids and the addition of any one of several immunosuppressive agents such as mycophenolate mofetil (MMF), azathioprine, tacrolimus or ciclosporin. If mild to moderate disease is refractory to this induction or maintenance therapy, a more aggressive approach with either rituximab or cyclophosphamide or a combination of MMF and tacrolimus is used. The management of severe myositis-associated ILD includes induction therapy with pulse glucocorticoids along with more potent immunosuppressive drugs, such as cyclophosphamide or rituximab, early on in the course of treatment followed by maintenance therapy with MMF, tacrolimus or ciclosporin (or sometimes azathioprine). If severe disease is refractory to induction or maintenance therapy, a more aggressive approach is used with a combination of rituximab and cyclophosphamide. If disease is still progressive, then other biologic agents being investigated in clinical trials for myositis are used, such as abatacept. Maintenance therapy of all forms of myositis-associated ILD depends on the response to initial treatment and subsequent progression. In cases of relapse, high doses of steroids (oral, intravenous or pulse therapy, depending on the disease severity) are given along with alternative immunosuppressive agents (not shown). Adapted from Ref. 119, Future Medicine. Adapted from Moghadam-Kia, S., Oddis, C. V. & Aggarwal, R. Update on the treatment of myositis. Int. J. Clin. Rheumatol. 9, 505–518 (2014). Oddis, C. V. & Aggarwal, R. (2018) Treatment in myositis Nat. Rev. Rheumatol. doi:10.1038/nrrheum.2018.42