• Liliana R. Santos
  • David IsenbergEmail author
Part of the Contemporary Clinical Neuroscience book series (CCNE)


Idiopathic inflammatory myopathies are a heterogeneous group of chronic autoimmune disorders that mainly affect the proximal muscles. The most common types include dermatomyositis (DM), polymyositis (PM), necrotizing autoimmune myopathy (NAM), and sporadic inclusion body myositis (sIBM). Patients typically present with progressive, proximal weakness and functional impairment. Extramuscular manifestations may also be present. Laboratory investigations with raised serum creatine kinase (CK) and myositis-specific antibodies (MSA) may help in diagnosis, differentiating the clinical phenotype and confirming the myositis subtype. The major goals of treatment are to eliminate the inflammation, restore muscle performance, reduce mortality, and improve quality of life.


Myositis Polymyositis Idiopathic inflammatory myopathies Necrotizing myopathy Inclusion body myositis Myositis-specific antibodies 


  1. 1.
    Dalakas M. Inflammatory muscle diseases. N Engl J Med. 2015;372:1734–47. Scholar
  2. 2.
    Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292(7):344–7. Scholar
  3. 3.
    Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. 1975;292(8):403–7. Scholar
  4. 4.
    Dalakas MC. Polymyositis, dermatomyositis and inclusion body myositis. N Engl J Med. 1991;325:1487–98. Scholar
  5. 5.
    Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet. 2003;362:971–82. PMID: 14511932. Scholar
  6. 6.
    Linklater H, Pipitone N, Rose MR, Norwood F, Campbell R, Salvarani C, et al. Classifying idiopathic inflammatory myopathies: comparing the performance of six existing criteria. Clin Exp Rheumatol. 2013;31:767–9. PMID:23806844PubMedGoogle Scholar
  7. 7.
    Troyanov Y, Targoff IN, Tremblay JL, Goulet JR, Raymond Y, Senécal JL. Novel classification of idiopathic inflammatory myopathies based on overlap syndrome features and autoantibodies: analysis of 100 French Canadian patients. Medicine (Baltimore). 2005;84(4):231–49.. PMID:16010208CrossRefGoogle Scholar
  8. 8.
    Lundberg IE, Tjarnlund A, Bottai M, Werth VP, Pilkington C, Visser M, et al. European league against rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major sub- groups. Ann Rheum Dis. 2017;76(12):1955–64. PMID: 29079590. PMCID: PMC5736307. Scholar
  9. 9.
    Castañeda S, Cavagna L, González-Gay MA; AENEAS (American-European NEtwork of Antisynthetase Syndrome) collaborative group members. Comments on the “2017 Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups”. Points of concern Arthritis Rheumatol 2018 70.
  10. 10.
    Leclair V, Lunberg IE. New myositis classification criteria – What we have learned since Bohan and Peter. Curr Rheumatol Rep. 2018;20:18.CrossRefGoogle Scholar
  11. 11.
    Lundberg IE, Visser M, Werth V. Classification of myositis. Nat Rev Rheumatol. 2018;14:269. Scholar
  12. 12.
    Dimachkie MM, Barohn RJ. Idiopathic inflammatory myopathies. Semin Neurol. 2012;32(3):227–36. Scholar
  13. 13.
    Tan JA, Roberts Thomson PJ, Blumbergs P, Hakenforf P, Cox SR, Limaye V. Incidence and prevalence of idiopathic inflammatory myopathies in South Australia: a 30-year epidemiologic study of histology-proven cases. Int J Rheum Dis. 2013;16(3):331–8. PMID: 23981756. Scholar
  14. 14.
    Dobloug C, Garen T, Bitter H, Stjarne J, Stenseth G, Grøvle L, et al. Prevalence and clinical characteristics of adult polymyositis and dermatomyositis; data from a large and unselected Norwegian cohort. Ann Rheum Dis. 2015;74:1551–6. PMID: 24695011. Scholar
  15. 15.
    Furst DE, Amato AA, Iorga SR, Gajria K, Fernandes AW. Epidemiology of adult idiopathic inflammatory myopathies in a U.S. managed care plan. Muscle Nerve. 2012;45:676–83. PMID:22499094. Scholar
  16. 16.
    Chahin N, Engel AG. Correlation of muscle biopsy, clinical course and outcome in PM and sporadic IBM. Neurology. 2008;70(6):1273–9. PMID: 17881720. Scholar
  17. 17.
    Meyer A, Meyer N, Schaeffer M, Gottenberg JE, Geny B, Sibilia J. Incidence and prevalence of inflammatory myopathies: a systematic review. Rheumatology (Oxford). 2015;54(1):50–63. Epub 2014 Jul 26.CrossRefGoogle Scholar
  18. 18.
    Ohta A, Nagai M, Nishina M, Tomimitsu H, Kohsaka H. Prevalence and incident of polymyositis and dermatomyositis in Japan. Mod Rheumatol. 2014;24(3):477–80. PMID:24252012. Scholar
  19. 19.
    Sultan SM, Ioannou Y, Moss K, Isenberg DA. Out-come in patients with idiopathic inflammatory myositis: morbidity and mortality. Rheumatology (Oxford). 2002;41:22–6. PMID:11792875CrossRefGoogle Scholar
  20. 20.
    Dalakas MC. Review: an update on inflammatory and autoimmune myopathies. Neuropathol Appl Neurobiol. 2011;37:226–42. PMID: 21155862. Scholar
  21. 21.
    Sontheimer RD. Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol. 1999;11(6):475–82.. PMID:10551671CrossRefGoogle Scholar
  22. 22.
    Morrow J, Nelson JL, Watts R, Isenberg DA. Autoimmune rheumatic disease. 2nd ed. Oxford: Oxford University Press; 1999. p. 174.Google Scholar
  23. 23.
    Malik A, Hayat G, Kalia JS, Guzman MA. Idiopathic inflammatory myopathies: clinical approach and management. Front Neurol. 2016;7:64. Scholar
  24. 24.
    Balbir-Gurman A, Denton C, Nichols B, Knight C, Nahir A, Martin G, Black C. Non-invasive measurement of biomechanical skin properties in systemic sclerosis. Ann Rheum Dis. 2002;61(3):237–41. PMCID: PMC1754026CrossRefPubMedPubMedCentralGoogle Scholar
  25. 25.
    Euwer RL, Sontheimer RD. Amyopathic dermatomyositis: a review. J Investig Dermatol. 1993;100(1):124–7. Scholar
  26. 26.
    Rider LF, Miller FW. Deciphering the clinical presentations, pathogenesis and treatment of idiopathic inflammatory myopathies. JAMA. 2011;305:183–90. PMID:21224460. PMCID:PMC4047218. Scholar
  27. 27.
    Iaccatino L, Ghirardello A, Bettio S, Zen M, Gatto M, Punzi L. The clinical features, diagnosis and classification of dermatomyositis. J Autoimmm. 2014;48-49:122–7. PMID:24467910. Scholar
  28. 28.
    Marie I, Hachulla E, Chérin P, Dominique S, Hatron PY, Hellot MF, Devulder B, Herson S, Levesque H, Courtois H. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum. 2002;47(6):614–22.. PMID:12522835CrossRefGoogle Scholar
  29. 29.
    Yazici Y, Kagen LJ. Clinical presentation of the idiopathic inflammatory myopathies. Rheum Dis Clin N Am. 2002;28(4):823–32. PMID:12506774CrossRefGoogle Scholar
  30. 30.
    Sekul EA, Dalakas MC. Inclusion body myositis: new concepts. Semin Neurol. 1993;13(3):256–63. PMID:8272596CrossRefGoogle Scholar
  31. 31.
    van der Meulen MFG, Bronner IM, Hoogendijk JE, Burger H, van Venrooij WJ, Voskuyl AE, et al. Polymyositis: an overdiagnosed entity. Neurology. 2003;61(3):316–21. Scholar
  32. 32.
    Dalakas M. Myositis. Are autoantibodies pathogenic in necrotizing myopathy? Nat Rev Rheum. 2018. PMID:29651118.CrossRefGoogle Scholar
  33. 33.
    Miller FW, Lamb JA, Schmidt J, Nagaraju K. Risk factors and disease mechanisms in myositis. Nat Rev Rheumatol. 2018;14:255–68. PMID:29674613. Scholar
  34. 34.
    Reed AM, Ytterberg SR. Genetic and environmental risk factors for idiopathic inflammatory myopathies. Rheum Dis Clin N Am. 2002;28:891–916. PMID:12506777CrossRefGoogle Scholar
  35. 35.
    Kuo CF, Grainge MJ, Valdes AM, See LC, Luo SF, Yu KH, Zhang W, Doherty M. Familial aggregation of systemic lupus erythematosus and coaggregation of autoimmune diseases in affected families. JAMA Intern Med. 2015;175(9):1518–26. Scholar
  36. 36.
    Reed AM, Stirling JD. Association of the HLA- DQA1∗0501 allele in multiple racial groups with juvenile dermatomyositis. Hum Immunol. 1995;44:131–5. PMID: 8666549CrossRefGoogle Scholar
  37. 37.
    Lintner KE, Patwardhan A, Rider LG, Abdul-Aziz R, Wu YL, Lundström E, Padyukov L, Zhou B, Alhomosh A, Newsom D, White P, Jones KB, O’Hanlon TP, Miller FW, Spencer CH, Yu CY. Gene copy-number variations (CNVs) of complement C4 and C4A deficiency in genetic risk and pathogenesis of juvenile dermatomyositis. Ann Rheum Dis. 2016;75(9):1599–606. Scholar
  38. 38.
    Wedderburn LR, Rider L. Juvenile dermatomyositis: new developments in pathogenesis, assessment and treatment. Best Pract Res Clin Rheumatol. 2009;23(5):665–78. PMID:19853831.PMCID:PMC2774891. Scholar
  39. 39.
    Santmyire-Rosenberger B, Dugan EM. Skin involvement in dermatomyositis. Curr Opin Rheumatol. 2003;15(6):714–22. PMID:14569200CrossRefGoogle Scholar
  40. 40.
    Rowe DJ, Isenberg DA, McDougall J, Beverley PC. Characterization of polymyositis infiltrates using monoclonal antibodies to human leucocyte antigens. Clin Exp Immunol. 1981;45(2):290–8. PMID:7032768PubMedPubMedCentralGoogle Scholar
  41. 41.
    Goebels N, Michaelis D, Engelhardt M, et al. Differential expression of perforin in muscle infiltrating T cells in polymyositis and dermatomyositis. J Clin Invest. 1996;97:2905–10. PMC507387CrossRefPubMedPubMedCentralGoogle Scholar
  42. 42.
    Bradshaw EM, Orihuela A, McArdel SL, et al. A local antigen driven humoral response in present in the inflammatory myopathies. J Immunol. 2007;178:547–56. Scholar
  43. 43.
    Campellone JV, Lacomis D, Giuliani MJ, Oddis CV. Percutaneous needle muscle biopsy in the evaluation of patients with suspected inflammatory myopathy. Arthritis Rheum. 1997;40(10):1886–91. PMID:9336426.<1886::AID-ART24>3.0.CO;2-J.CrossRefPubMedGoogle Scholar
  44. 44.
    McHugh N, Tansley SL. Autoantibodies in myositis. Nat Rev Rheumatol. 2018;14:290–302.CrossRefGoogle Scholar
  45. 45.
    Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkjaer L, Airio A, et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001;357:96–100. Scholar
  46. 46.
    Leatham H, Schadt C, Chisolm S, Fretwell D, Chung L, Callen JP, Fiorentino D. Evidence supports blind screening for internal malignancy in dermatomyositis: Data from 2 large US dermatology cohorts. Medicine (Baltimore). 2018 Jan. 2018;97(2):e9639. Scholar
  47. 47.
    Gordon PA, Winer JB, Hoogendijk JE, Choy EH. Immunosuppressant and immunomodulatory treatment for dermatomyositis and polymyositis. In: Gordon PA, editor. Cochrane Database of Systematic Reviews. Chichester, UK: John Wiley & Sons Ltd; 2012. p. CD003643.Google Scholar
  48. 48.
    Joffe M, et al. Drug therapy of the idiopathic inflammatory myopathies, predictors of response to prednisone, azathioprine and methotrexate and a comparison of their efficacy. Am J Med. 1993;94:379–87. Scholar
  49. 49.
    Newman E, Scott DW. The use of low dose methotrexate in the treatment of polymyositis and dermatomyositis. J Clin Rheumatol. 1995;1:99–102.. PMID:19077954.CrossRefGoogle Scholar
  50. 50.
    Ruperto N. Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new onset juvenile dermatomyositis: a randomized trial. Lancet. 2016;387:671–8. Scholar
  51. 51.
    Ernste FC, Reed AM. Idiopathic inflammatory myopathies: current trends in pathogenesis, clinical features, and up-to-date treatment recommendations. Mayo Clin Proc. 2013;88:83–105. Scholar
  52. 52.
    Villalba L, Hicks JE, Adams EM, Sherman JB, Gourley MF, Leff RL, et al. Treatment of refractory myositis: a randomized crossover study of two new cytotoxic regimens. Arthritis Rheum. 1998;41:392–9.CrossRefGoogle Scholar
  53. 53.
    Edge JC, Outland JD, Dempsey JR, Callen JP. Mycophenolate mofetil as an effective corticosteroid- sparing therapy for recalcitrant dermatomyositis. Arch Dermatol. 2006;142:65–9. Scholar
  54. 54.
    Pisoni CN, Cuadrado MJ, Khamashta MA, Hughes GRV, D’Cruz DP. Mycophenolate mofetil treatment in resistant myositis. Rheumatology (Oxford). 2007;46:516–8. Scholar
  55. 55.
    Danieli MG. Intravenous immunoglobulin as add on treatment wit mycophenolate mofetil in severe myositis. Autoimmune Rev. 2009;9:124–7. Scholar
  56. 56.
    Morganroth PA, Kreider ME, Werth VP. Mycophenolate mofetil for interstitial lung disease in dermatomyositis. Arthritis Care Res (Hoboken). 2010;62:1496–501. Scholar
  57. 57.
    Fischer A. Mycophenolate mofetil improves lung function in connective tissue disease associated interstitial lung disease. J Rheumatol. 2013;40:640–6. Scholar
  58. 58.
    Yamasaki Y, Yamada H, Yamasaki M, Ohkubo M, Azuma K, Matsuoka S, et al. Intravenous cyclophosphamide therapy for progressive interstitial pneumonia in patients with polymyositis/dermatomyositis. Rheumatology (Oxford). 2007;46:124–30. Scholar
  59. 59.
    Oddis CV, Aggarwal R. Treatment in myositis. Nat Rev Rheumatol. 2018;14:279–89.CrossRefGoogle Scholar
  60. 60.
    Oddis CV, Sciurba FC, Elmagd KA, Starzl TE. Tacrolimus in refractory polymyositis with interstitial lung disease. Lancet. 1999;353:1762–3. Scholar
  61. 61.
    Qushmaq KA, Chalmers A, Esdaile JM. Cyclosporin A in the treatment of refractory adult polymyositis/dermatomyositis: population based experience in 6 patients and literature review. J Rheumatol. 2000;27:2855–9. PMID:11128676PubMedGoogle Scholar
  62. 62.
    Mitsui T, et al. The effects of FK506 on refractory inflammatory myopathies. Acta Neur Belg. 2011;111:188–94. PMID:22141281Google Scholar
  63. 63.
    Vencovský J, Jarosová K, Machácek S, Studýnková J, Kafková J, Bartůnková J, et al. Cyclosporine A versus methotrexate in the treatment of polymyositis and dermatomyositis. Scand J Rheumatol. 2000;29:95–102. Scholar
  64. 64.
    Fasano S, Gordon P, Hajji R, Loyo E, Isenberg DA. Rituximab in the treatment of inflammatory myopathies: a review. Rheumatology. 2017;56:26–36. Scholar
  65. 65.
    Oddis CV. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized placebo-phase trial. Arthritis Rheum. 2013;65:314–24. Scholar
  66. 66.
    Schoffenbaeur A. A randomized, double blind placebo controlled trial of infliximab in refractory polymyositis and dermatomyositis. Semin Arthritis Rheum. 2017;47(6):858–64. Scholar
  67. 67.
    Riolo G, Towheed TE. Anti-tumor necrosis factor inhibitor therapy induced dermatomyositis and fasciitis. J Rheumatol. 2012;39:192–4.PubMedGoogle Scholar
  68. 68.
    Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM, et al. 2016 American college of rheumatology/european league against rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: an international myositis assessment and clinical studies group/paediatric rheumatology international trials organisation collaborative initiative. Arthritis Rheumatol. 2017;69:898–910. Scholar
  69. 69.
    Alexanderson H. Physical exercise as a treatment for adult and juvenile myositis. J Intern Med. 2016;280(1):75–96. Scholar
  70. 70.
    Rider LG, Aggarwal R, Machado PM, Hogrel JY, Reed AM, Christopher-Stine L, Ruperto N. Update on outcome assessment in myositis. Nat Rev Rheumatol. 2018;14:303–18. PMID:29651119. Scholar
  71. 71.
    Silva MA, Cogollo E, Isenberg DA. Why do patients with myositis die? A retrospective analysis of a single centre cohort. Clin Exp Rheumatol. 2016;34(5):820–6. PMID:27494511Google Scholar
  72. 72.
    Parker M, Lilleker J, Roberts M, Chinoy H. Idiopathic Inflammatory myopathies. Medicine. 2018.Google Scholar

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Internal Medicine Service, Hospital Santa Maria, Lisbon Academic Medical CenterLisbonPortugal
  2. 2.Centre for Rheumatology, Division of MedicineUniversity College of LondonLondonUK

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