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. 2020 Dec 1;39(4):289–301. doi: 10.36185/2532-1900-032

Table I.

A step-by step approach in the treatment of inflammatory myopathies: 2020 and beyond.

Dermatomyosits (DM) 1-3,72-83

  1. High-dose prednisone (oral or intermittent intravenous in acute cases)

  2. In steroid-responsive patients add an immunosuppressant [mycophenolate, (most preferable) azathioprine, or methotrexate]

  3. High-dose intravenous immunoglobulin (IVIg) if steps 1-2 fail

  4. Rituximab, if IVIg is not sufficiently effective

  5. Consider new biologics including eculizumab, other anti-B cell agents or JAK inhibitors

  6. Most promising future: anti-complement agents such as eculizumab, ravulizumab (ultomiris), zilucoplan

Polymyositis (PM) 1-3,72-83

  1. High-dose prednisone (oral or intermittent intravenous in acute cases)

  2. In steroid-responsive patients add an immunosuppressant [mycophenolate, (most preferable) azathioprine, or methotrexate]

  3. High-dose intravenous immunoglobulin (IVIg), if steps 1-2 fail

  4. Rituximab, if IVIg is not sufficiently effective

  5. If above unsatisfactory, reconsider the diagnosis and explore it with a new muscle biopsy

Necrotizing Autoimmune Myositis (NAM) 1-3,72-83

  1. High-dose prednisone (intravenously 1g/daily for 5 days may be needed in acute cases)

  2. High-dose intravenous immunoglobulin (IVIg)

  3. Rituximab, if IVIg not sufficiently effective

  4. Consider new biologics, including eculizumab, other anti-B cell agents or JAK inhibitors

  5. Most promising future: anti-complement agents, such as eculizumab, ravulizumab (ultomiris), zilucoplan

Anti-synthetase syndrome-Overlap Myositis (Anti-SS-OM) 1-3,72-83

  1. High-dose prednisone (oral or intermittent intravenous in acute cases)

  2. In steroid-responsive patients add an immunosuppressant [mycophenolate, (most preferable) azathioprine, or methotrexate]

  3. High-dose intravenous immunoglobulin (IVIg) if steps 1-2 fail

  4. Rituximab, if IVIg is not sufficiently effective

  5. If interstitial lung disease, may also consider cyclophosphamide

Inclusion Body Myositis 1-3,84-92

  1. Physical therapy; CoQ10; encourage participation in a controlled study

  2. If dysphagia is prominent, IVIg

  3. All trials with immunosuppressants, immunomodulating agents, muscle growth factors TGF-β inhibitors have failed. Among them, most promising was alemtuzumab in an uncontrolled study