Biologic therapy in the idiopathic inflammatory myopathies

Rheumatol Int. 2020 Feb;40(2):191-205. doi: 10.1007/s00296-019-04467-6. Epub 2019 Nov 4.


The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases resulting from inflammation of muscle and manifesting as weakness, though a range of extra-muscular manifestations are observed. These are often correlated closely with disease subtype and the presence of myositis-specific/myositis-associated antibodies. IIM are notoriously difficult to treat and often refractory to glucocorticoid therapy and synthetic immunosuppressants. Both the innate and adaptive immune systems are implicated in the pathogenesis of IIM. A growing understanding of the key cytokines as well as the cell-mediated and antibody effectors of disease has identified multiple potential targets for biologic therapy. The most widely used of these is B-cell depletion via rituximab though the tumour necrosis factor inhibitors and other biologic therapies used in diseases such as rheumatoid arthritis, systemic lupus erythematosus and multiple sclerosis have also been trialled. This review summarises the literature thus far on biologic therapy in IIM, highlighting both the significant trials that influence current treatment regimens and also the continuing need for further research to inform more effective therapies.

Keywords: Biologics; Etanercept; Idiopathic inflammatory myopathies; Infliximab; Rituximab.

Publication types

  • Review

MeSH terms

  • Abatacept / therapeutic use
  • Alemtuzumab / therapeutic use
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Antirheumatic Agents / therapeutic use*
  • Basiliximab / therapeutic use
  • Biological Products / therapeutic use*
  • Dermatomyositis / drug therapy
  • Dermatomyositis / immunology
  • Humans
  • Immunologic Factors / therapeutic use*
  • Immunosuppressive Agents / therapeutic use
  • Myositis / drug therapy*
  • Myositis / immunology
  • Myositis, Inclusion Body / drug therapy
  • Myositis, Inclusion Body / immunology
  • Polymyositis / drug therapy
  • Polymyositis / immunology
  • Rituximab / therapeutic use
  • Tumor Necrosis Factor Inhibitors / therapeutic use*


  • Antibodies, Monoclonal, Humanized
  • Antirheumatic Agents
  • Biological Products
  • Immunologic Factors
  • Immunosuppressive Agents
  • Tumor Necrosis Factor Inhibitors
  • Alemtuzumab
  • Rituximab
  • Abatacept
  • Basiliximab
  • tocilizumab
  • sifalimumab