Abstract
The idiopathic inflammatory myopathies (IIM), often referred to collectively but less specifically as “myositis,” have an estimated prevalence of ~14 per 100,000 with an annual incidence of ~8 per 1,000,000 population, which appears to be increasing over time. Any age group can be affected, but myositis commonly presents between 30 and 60 years of age. Differences are seen based on autoantibody subtypes as, for example, anti-signal recognition particle (SRP) seems more common in younger adults. Females are affected about twice as often as males, but again there are important differences when considering specific clinical subtypes as inclusion body myositis (IBM) is more common in males. Current research is focussed on understanding the complex interactions between genetic susceptibility and exposures to environmental triggers. The majority of genetic risk factors identified to date lie within the major histocompatibility complex (MHC), a region which contains genes responsible for antigen presentation, but a small number of genetic risk factors have been found outside this region and implicated in innate and adaptive immune functions. Important environmental associations have been already identified, including exposures to ultraviolet radiation, smoking and certain medications such as statins.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Bohan A, Peter JB. Polymyositis and dermatomyositis (parts 1 and 2). N Engl J Med. 1975;292:344–7, 403–7.
Meyer A, Meyer N, Schaeffer M, Gottenberg J-E, Geny B, Sibilia J. Incidence and prevalence of inflammatory myopathies: a systematic review. Rheumatology (Oxford). 2015;54:50–63.
Bernatsky S, Joseph L, Pineau CA, Belisle P, Boivin JF, Banerjee D, Clarke AE. Estimating the prevalence of polymyositis and dermatomyositis from administrative data: age, sex and regional differences. Ann Rheum Dis. 2009;68:1192–6.
Smoyer-Tomic KE, Amato AA, Fernandes AW. Incidence and prevalence of idiopathic inflammatory myopathies among commercially insured, Medicare supplemental insured, and Medicaid enrolled populations: an administrative claims analysis. BMC Musculoskelet Disord. 2012;13:103.
Griggs R, Askanas V, DiMauro S, Engel A, Karpati G, Mendell J, Rowland L. Inclusion body myositis and myopathies. Ann Neurol. 1995;38:705–13.
Mastaglia FL, Phillips BA. Idiopathic inflammatory myopathies: epidemiology, classification, and diagnostic criteria. Rheum Dis Clin N Am. 2002;28:723–41.
Rose MR. 188th ENMC international workshop: inclusion body myositis, 2–4 December 2011, Naarden, The Netherlands. Neuromuscul Disord. 2013;23:1044–55.
Phillips BA, Zilko PJ, Mastaglia FL. Prevalence of sporadic inclusion body myositis in Western Australia. Muscle Nerve. 2000;23:970–2.
Needham M, Corbett A, Day T, Christiansen F, Fabian V, Mastaglia FL. Prevalence of sporadic inclusion body myositis and factors contributing to delayed diagnosis. J Clin Neurosci. 2008;15:1350–3.
Tan JA, Roberts-Thomson PJ, Blumbergs P, Hakendorf 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;16:331–8.
Wilson FC, Ytterberg SR, St. Sauver JL, Reed AM. Epidemiology of sporadic inclusion body myositis and polymyositis in Olmsted County, Minnesota. J Rheumatol. 2008;35:445–7.
Suzuki N, Aoki M, Mori-Yoshimura M, Hayashi YK, Nonaka I, Nishino I. Increase in number of sporadic inclusion body myositis (sIBM) in Japan. J Neurol. 2012;259:554–6.
Dobloug GC, Antal EA, Sveberg L, Garen T, Bitter H, Stjärne J, Grøvle L, Gran JT, Molberg Ø. High prevalence of inclusion body myositis in Norway; a population-based clinical epidemiology study. Eur J Neurol. 2014;22:672–80.
Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkjaer L, Airio A, Evans SR, Felson DT. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001;357:96–100.
Zantos D, Zhang Y, Felson D. The overall and temporal association of cancer with polymyositis and dermatomyositis. J Rheumatol. 1994;21:1855–9.
Dobloug GC, Garen T, Brunborg C, Gran JT, Molberg Ø. Survival and cancer risk in an unselected and complete Norwegian idiopathic inflammatory myopathy cohort. Semin Arthritis Rheum. 2015;45:301–8.
Jones J, Wortmann R. Idiopathic inflammatory myopathies-a review. Clin Rheumatol. 2015;34:839–44.
Muro Y, Sugiura K, Nara M, Sakamoto I, Suzuki N, Akiyama M. High incidence of cancer in anti-small ubiquitin-like modifier activating enzyme antibody-positive dermatomyositis. Rheumatology (Oxford). 2015;54:1745–7.
Symmons DP, Sills JA, Davis SM. The incidence of juvenile dermatomyositis: results from a nation-wide study. Br J Rheumatol. 1995;34:732–6.
Darin N, Tulinius M. Neuromuscular disorders in childhood: a descriptive epidemiological study from western Sweden. Neuromuscul Disord. 2000;10:1–9.
Mendez EP, Lipton R, Ramsey-Goldman R, Roettcher P, Bowyer S, Dyer A, Pachman LM. US incidence of juvenile dermatomyositis, 1995–1998: results from the National Institute of Arthritis and Musculoskeletal and Skin Diseases registry. Arthritis Rheum. 2003;49:300–5.
Denardo B, Tucker L, Miller L, Szer I, Schaller J. Demography of a regional pediatric rheumatology patient population. Affiliated Children’s Arthritis Centers of New England. J Rheumatol. 1994;21:1553–61.
Bergua C, Chiavelli H, Simon J, Boyer O, Jouen F, Stenzel W, Martinet J. Immune-mediated necrotizing myopathy. Z Rheumatol. 2016;75:151–6.
Klein M, Mann H, Pleštilová L, Zámečník J, Betteridge Z, McHugh N, Vencovský J. Increasing incidence of immune-mediated necrotizing myopathy: single-centre experience. Rheumatology (Oxford). 2015;54:2010–4.
Molberg Ø, Dobloug C. Epidemiology of sporadic inclusion body myositis. Curr Opin Rheumatol. 2016;28:657–60.
Tansley SL, McHugh NJ, Wedderburn LR. Adult and juvenile dermatomyositis: are the distinct clinical features explained by our current understanding of serological subgroups and pathogenic mechanisms? Arthritis Res Ther. 2013;15:211.
Rider L, Gurley R, Pandey J, et al. Clinical, serologic, and immunogenetic features of familial idiopathic inflammatory myopathy. Arthritis Rheum. 1998;41:710–9.
Ozaki T, Yamashita H, Hosono Y, Nakashima R, Mimori T, Takahashi Y, Kaneko H, Kano T, Mimori A. Two patients in the same family with anti-ARS antibody-associated myositis. Mod Rheumatol. 2014;24:699–700.
Gi LR, Lin J, Plotz PH, Bale SJ, Wilder R, Mbauya A, Miller FW. Familial autoimmunity in pedigrees of idiopathic inflammatory myopathy patients suggests common genetic risk factors for many autoimmune diseases. Rheumatism. 1998;41:400–5.
Niewold TB, Wu SC, Smith M, Morgan GA, Pachman LM. Familial aggregation of autoimmune disease in juvenile dermatomyositis. Pediatrics. 2011;127:e1239–46.
Hyttinen V, Kaprio J, Kinnunen L, Koskenvuo M, Tuomilehto J. Genetic liability of Type 1 diabetes and the onset age among 22,650 young Finnish twin pairs. Diabetes. 2003;52:1052–5.
Van Der Woude D, Houwing-Duistermaat JJ, Toes REM, Huizinga TWJ, Thomson W, Worthington J, Van Der Helm-Van Mil AHM, De Vries RRP. Quantitative heritability of anti-citrullinated protein antibody-positive and anti-citrullinated protein antibody-negative rheumatoid arthritis. Arthritis Rheum. 2009;60:916–23.
Okada S, Weatherhead E, Targoff IN, Wesley R, Miller FW. Global surface ultraviolet radiation intensity may modulate the clinical and immunologic expression of autoimmune muscle disease. Arthritis Rheum. 2003;48:2285–93.
Love LA, Weinberg CR, McConnaughey DR, Oddis CV, Medsger TA, Reveille JD, Arnett FC, Targoff IN, Miller FW. Ultraviolet radiation intensity predicts the relative distribution of dermatomyositis and anti-Mi-2 autoantibodies in women. Arthritis Rheum. 2009;60:2499–504.
Chinoy H, Adimulam S, Marriage F, et al. Interaction of HLA-DRB1*03 and smoking for the development of anti-Jo-1 antibodies in adult idiopathic inflammatory myopathies: a European-wide case study. Ann Rheum Dis. 2012;71:961–5.
Padyukov L, Suva C, Stolt P, Alfredsson L, Klareskog L. A gene-environment interaction between smoking and shared epitope genes in HLA-DR provides a high risk of seropositive rheumatoid arthritis. Arthritis Rheum. 2004;50:3085–92.
Klareskog L, Stolt P, Lundberg K, et al. A new model for an etiology of rheumatoid arthritis: smoking may trigger HLA-DR (shared epitope)-restricted immune reactions to autoantigens modified by citrullination. Arthritis Rheum. 2006;54:38–46.
Mammen AL, Gaudet D, Brisson D, Christopher-Stine L, Lloyd TE, Leffell MS, Zachary AA. Increased frequency of DRB1*11:01 in anti-hydroxymethylglutaryl- coenzyme a reductase-associated autoimmune myopathy. Arthritis Care Res. 2012;64:1233–7.
Chinoy H, Fertig N, Oddis CV, Ollier WER, Cooper RG. The diagnostic utility of myositis autoantibody testing for predicting the risk of cancer-associated myositis. Ann Rheum Dis. 2007;66:1345–9.
Shah AA, Casciola-Rosen L, Rosen A. Review: cancer-induced autoimmunity in the rheumatic diseases. Arthritis Rheumatol. 2015;67:317–26.
Atli B, Yamac M, Yildiz Z. Optimization of submerged fermentation conditions for lovastatin production by the culinary-medicinal oyster mushroom, Pleurotus ostreatus (higher Basidomycetes). Int J Med Mushrooms. 2013;15:487–95.
Gan L, Miller FW. State of the art: what we know about infectious agents and myositis. Curr Opin Rheumatol. 2011;23:585–94.
Shamriz O, Mizrahi H, Werbner M, Shoenfeld Y, Avni O, Koren O. Microbiota at the crossroads of autoimmunity. Autoimmun Rev. 2016;15:859–69.
Rothwell S, Cooper RG, Lundberg IE, et al. Dense genotyping of immune-related loci in idiopathic inflammatory myopathies confirms HLA alleles as the strongest genetic risk factor and suggests different genetic background for major clinical subgroups. Ann Rheum Dis. 2016;75:1558–66.
Rothwell S, Cooper RG, Lundberg IE, et al. Immune-array analysis in sporadic inclusion body myositis reveals HLA-DRB1 amino acid heterogeneity across the myositis spectrum. Arthritis Rheumatol. 2017;69:1090–9.
Kirino Y, Remmers EF. Genetic architectures of seropositive and seronegative rheumatic diseases. Nat Rev Rheumatol. 2015;11:401–14.
Sugiura T, Kawaguchi Y, Goto K, Hayashi Y, Tsuburaya R, Furuya T, Gono T, Nishino I, Yamanaka H. Positive association between STAT4 polymorphisms and polymyositis/dermatomyositis in a Japanese population. Ann Rheum Dis. 2012;71:1646–50.
Chen S, Wu W, Li J, et al. Single nucleotide polymorphisms in the FAM167A-BLK gene are associated with polymyositis/dermatomyositis in the Han Chinese population. Immunol Res. 2015;62:153–62.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2020 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Parker, M.J.S., Chinoy, H., Cooper, R.G., Lamb, J.A. (2020). Myositis Basics/Who Gets Myositis. In: Aggarwal, R., Oddis, C. (eds) Managing Myositis. Springer, Cham. https://doi.org/10.1007/978-3-030-15820-0_2
Download citation
DOI: https://doi.org/10.1007/978-3-030-15820-0_2
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-15819-4
Online ISBN: 978-3-030-15820-0
eBook Packages: MedicineMedicine (R0)