Abstract
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.
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References
Dalakas M. Inflammatory muscle diseases. N Engl J Med. 2015;372:1734–47. https://doi.org/10.1056/NEJMra1402225.
Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292(7):344–7. https://doi.org/10.1056/NEJM197502132920706.
Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. 1975;292(8):403–7. https://doi.org/10.1056/NEJM197502202920807.
Dalakas MC. Polymyositis, dermatomyositis and inclusion body myositis. N Engl J Med. 1991;325:1487–98. https://doi.org/10.1056/NEJM199111213252107.
Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet. 2003;362:971–82. PMID: 14511932. https://doi.org/10.1016/S0140-6736(03)14368-1.
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:23806844
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:16010208
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. https://doi.org/10.1136/annrheumdis-2017-211468.
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. https://doi.org/10.1002/art.40478.www.google.
Leclair V, Lunberg IE. New myositis classification criteria – What we have learned since Bohan and Peter. Curr Rheumatol Rep. 2018;20:18.
Lundberg IE, Visser M, Werth V. Classification of myositis. Nat Rev Rheumatol. 2018;14:269. https://doi.org/10.1038/nrrheum.2018.41.
Dimachkie MM, Barohn RJ. Idiopathic inflammatory myopathies. Semin Neurol. 2012;32(3):227–36. https://doi.org/10.1055/s-0032-1329201.
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. https://doi.org/10.1111/j.1756-185X.2011.01669.x.
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. https://doi.org/10.1136/annrheumdis-2013-205127.
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. https://doi.org/10.1002/mus.23302.
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. https://doi.org/10.1212/01.wnl.0000277527.69388.fe.
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. https://doi.org/10.1093/rheumatology/keu289. Epub 2014 Jul 26.
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. https://doi.org/10.3109/14397595.2013.844308.
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:11792875
Dalakas MC. Review: an update on inflammatory and autoimmune myopathies. Neuropathol Appl Neurobiol. 2011;37:226–42. PMID: 21155862. https://doi.org/10.1111/j.1365-2990.2010.01153.x.
Sontheimer RD. Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol. 1999;11(6):475–82.. PMID:10551671
Morrow J, Nelson JL, Watts R, Isenberg DA. Autoimmune rheumatic disease. 2nd ed. Oxford: Oxford University Press; 1999. p. 174.
Malik A, Hayat G, Kalia JS, Guzman MA. Idiopathic inflammatory myopathies: clinical approach and management. Front Neurol. 2016;7:64. https://doi.org/10.3389/fneur.2016.00064.
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. https://doi.org/10.1136/ard.61.3.237. PMCID: PMC1754026
Euwer RL, Sontheimer RD. Amyopathic dermatomyositis: a review. J Investig Dermatol. 1993;100(1):124–7. https://doi.org/10.1038/jid.1993.35.
Rider LF, Miller FW. Deciphering the clinical presentations, pathogenesis and treatment of idiopathic inflammatory myopathies. JAMA. 2011;305:183–90. PMID:21224460. PMCID:PMC4047218. https://doi.org/10.1001/jama.2010.1977.
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. https://doi.org/10.1016/j.jaut.2013.11.005.
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:12522835
Yazici Y, Kagen LJ. Clinical presentation of the idiopathic inflammatory myopathies. Rheum Dis Clin N Am. 2002;28(4):823–32. PMID:12506774
Sekul EA, Dalakas MC. Inclusion body myositis: new concepts. Semin Neurol. 1993;13(3):256–63. PMID:8272596
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. https://doi.org/10.1212/WNL.61.3.316.
Dalakas M. Myositis. Are autoantibodies pathogenic in necrotizing myopathy? Nat Rev Rheum. 2018. https://doi.org/10.1038/nrrheum.2018.54. PMID:29651118.
Miller FW, Lamb JA, Schmidt J, Nagaraju K. Risk factors and disease mechanisms in myositis. Nat Rev Rheumatol. 2018;14:255–68. PMID:29674613. https://doi.org/10.1038/nrrheum.2018.48.
Reed AM, Ytterberg SR. Genetic and environmental risk factors for idiopathic inflammatory myopathies. Rheum Dis Clin N Am. 2002;28:891–916. PMID:12506777
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. https://doi.org/10.1001/jamainternmed.2015.3528.
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: 8666549
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. https://doi.org/10.1136/annrheumdis-2015-207762.
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. https://doi.org/10.1016/j.berh.2009.07.007.
Santmyire-Rosenberger B, Dugan EM. Skin involvement in dermatomyositis. Curr Opin Rheumatol. 2003;15(6):714–22. PMID:14569200
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:7032768
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. https://doi.org/10.1172/JCI118749. PMC507387
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. https://doi.org/10.4049/jimmunol.178.1.547.
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. https://doi.org/10.1002/1529-0131(199710)40:10<1886::AID-ART24>3.0.CO;2-J.
McHugh N, Tansley SL. Autoantibodies in myositis. Nat Rev Rheumatol. 2018;14:290–302.
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. https://doi.org/10.1016/S0140-6736(00)03540-6.
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. https://doi.org/10.1097/MD.0000000000009639.
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.
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. https://doi.org/10.1016/0002-9343(93)90148-I.
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.
Ruperto N. Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new onset juvenile dermatomyositis: a randomized trial. Lancet. 2016;387:671–8. https://doi.org/10.1016/S0140-6736(15)01021-1.
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. https://doi.org/10.1016/j.mayocp.2012.10.017.
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.
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. https://doi.org/10.1001/archderm.142.1.65.
Pisoni CN, Cuadrado MJ, Khamashta MA, Hughes GRV, D’Cruz DP. Mycophenolate mofetil treatment in resistant myositis. Rheumatology (Oxford). 2007;46:516–8. https://doi.org/10.1093/rheumatology/kel336.
Danieli MG. Intravenous immunoglobulin as add on treatment wit mycophenolate mofetil in severe myositis. Autoimmune Rev. 2009;9:124–7. https://doi.org/10.1016/j.autrev.2009.04.003.
Morganroth PA, Kreider ME, Werth VP. Mycophenolate mofetil for interstitial lung disease in dermatomyositis. Arthritis Care Res (Hoboken). 2010;62:1496–501. https://doi.org/10.1002/acr.20212.
Fischer A. Mycophenolate mofetil improves lung function in connective tissue disease associated interstitial lung disease. J Rheumatol. 2013;40:640–6. https://doi.org/10.3899/jrheum.121043.
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. https://doi.org/10.1093/rheumatology/kel112.
Oddis CV, Aggarwal R. Treatment in myositis. Nat Rev Rheumatol. 2018;14:279–89.
Oddis CV, Sciurba FC, Elmagd KA, Starzl TE. Tacrolimus in refractory polymyositis with interstitial lung disease. Lancet. 1999;353:1762–3. https://doi.org/10.1016/S0140-6736(99)01927-3.
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:11128676
Mitsui T, et al. The effects of FK506 on refractory inflammatory myopathies. Acta Neur Belg. 2011;111:188–94. PMID:22141281
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. https://doi.org/10.1080/030097400750001897.
Fasano S, Gordon P, Hajji R, Loyo E, Isenberg DA. Rituximab in the treatment of inflammatory myopathies: a review. Rheumatology. 2017;56:26–36. https://doi.org/10.1093/rheumatology/kew146.
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. https://doi.org/10.1002/art.37754.
Schoffenbaeur A. A randomized, double blind placebo controlled trial of infliximab in refractory polymyositis and dermatomyositis. Semin Arthritis Rheum. 2017;47(6):858–64. https://doi.org/10.1016/j.semarthrit.2017.10.010.
Riolo G, Towheed TE. Anti-tumor necrosis factor inhibitor therapy induced dermatomyositis and fasciitis. J Rheumatol. 2012;39:192–4.
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. https://doi.org/10.1002/art.40064.
Alexanderson H. Physical exercise as a treatment for adult and juvenile myositis. J Intern Med. 2016;280(1):75–96. https://doi.org/10.1111/joim.12481.
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. https://doi.org/10.1038/nrrheum.2018.33.
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:27494511
Parker M, Lilleker J, Roberts M, Chinoy H. Idiopathic Inflammatory myopathies. Medicine. 2018.
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Santos, L.R., Isenberg, D. (2019). Myositis. In: Mitoma, H., Manto, M. (eds) Neuroimmune Diseases. Contemporary Clinical Neuroscience. Springer, Cham. https://doi.org/10.1007/978-3-030-19515-1_27
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