Skip to main content

Myositis Basics/Who Gets Myositis

  • Chapter
  • First Online:
Managing Myositis

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.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 159.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Bohan A, Peter JB. Polymyositis and dermatomyositis (parts 1 and 2). N Engl J Med. 1975;292:344–7, 403–7.

    Article  CAS  PubMed  Google Scholar 

  2. 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.

    Article  CAS  Google Scholar 

  3. 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.

    Article  CAS  PubMed  Google Scholar 

  4. 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.

    Article  PubMed  PubMed Central  Google Scholar 

  5. 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.

    Article  CAS  PubMed  Google Scholar 

  6. Mastaglia FL, Phillips BA. Idiopathic inflammatory myopathies: epidemiology, classification, and diagnostic criteria. Rheum Dis Clin N Am. 2002;28:723–41.

    Article  Google Scholar 

  7. Rose MR. 188th ENMC international workshop: inclusion body myositis, 2–4 December 2011, Naarden, The Netherlands. Neuromuscul Disord. 2013;23:1044–55.

    Article  CAS  PubMed  Google Scholar 

  8. Phillips BA, Zilko PJ, Mastaglia FL. Prevalence of sporadic inclusion body myositis in Western Australia. Muscle Nerve. 2000;23:970–2.

    Article  CAS  PubMed  Google Scholar 

  9. 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.

    Article  PubMed  Google Scholar 

  10. 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.

    Article  PubMed  Google Scholar 

  11. 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.

    PubMed  Google Scholar 

  12. 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.

    Article  PubMed  Google Scholar 

  13. 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.

    Article  PubMed  Google Scholar 

  14. 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.

    Article  CAS  PubMed  Google Scholar 

  15. Zantos D, Zhang Y, Felson D. The overall and temporal association of cancer with polymyositis and dermatomyositis. J Rheumatol. 1994;21:1855–9.

    CAS  PubMed  Google Scholar 

  16. 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.

    Article  PubMed  Google Scholar 

  17. Jones J, Wortmann R. Idiopathic inflammatory myopathies-a review. Clin Rheumatol. 2015;34:839–44.

    Article  PubMed  Google Scholar 

  18. 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.

    Article  Google Scholar 

  19. Symmons DP, Sills JA, Davis SM. The incidence of juvenile dermatomyositis: results from a nation-wide study. Br J Rheumatol. 1995;34:732–6.

    Article  CAS  PubMed  Google Scholar 

  20. Darin N, Tulinius M. Neuromuscular disorders in childhood: a descriptive epidemiological study from western Sweden. Neuromuscul Disord. 2000;10:1–9.

    Article  CAS  PubMed  Google Scholar 

  21. 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.

    Article  PubMed  Google Scholar 

  22. 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.

    CAS  PubMed  Google Scholar 

  23. Bergua C, Chiavelli H, Simon J, Boyer O, Jouen F, Stenzel W, Martinet J. Immune-mediated necrotizing myopathy. Z Rheumatol. 2016;75:151–6.

    Article  CAS  PubMed  Google Scholar 

  24. 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.

    Article  CAS  Google Scholar 

  25. Molberg Ø, Dobloug C. Epidemiology of sporadic inclusion body myositis. Curr Opin Rheumatol. 2016;28:657–60.

    Article  PubMed  Google Scholar 

  26. 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.

    Article  PubMed  PubMed Central  Google Scholar 

  27. Rider L, Gurley R, Pandey J, et al. Clinical, serologic, and immunogenetic features of familial idiopathic inflammatory myopathy. Arthritis Rheum. 1998;41:710–9.

    Article  CAS  PubMed  Google Scholar 

  28. 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.

    Article  PubMed  Google Scholar 

  29. 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.

    Article  Google Scholar 

  30. Niewold TB, Wu SC, Smith M, Morgan GA, Pachman LM. Familial aggregation of autoimmune disease in juvenile dermatomyositis. Pediatrics. 2011;127:e1239–46.

    Article  PubMed  PubMed Central  Google Scholar 

  31. 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.

    Article  CAS  PubMed  Google Scholar 

  32. 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.

    Article  PubMed  Google Scholar 

  33. 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.

    Article  PubMed  Google Scholar 

  34. 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.

    Article  PubMed  PubMed Central  Google Scholar 

  35. 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.

    Article  CAS  PubMed  Google Scholar 

  36. 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.

    Article  CAS  PubMed  Google Scholar 

  37. 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.

    Article  CAS  PubMed  Google Scholar 

  38. 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.

    Article  CAS  Google Scholar 

  39. 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.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Shah AA, Casciola-Rosen L, Rosen A. Review: cancer-induced autoimmunity in the rheumatic diseases. Arthritis Rheumatol. 2015;67:317–26.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. 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.

    Article  CAS  PubMed  Google Scholar 

  42. Gan L, Miller FW. State of the art: what we know about infectious agents and myositis. Curr Opin Rheumatol. 2011;23:585–94.

    Article  CAS  PubMed  Google Scholar 

  43. Shamriz O, Mizrahi H, Werbner M, Shoenfeld Y, Avni O, Koren O. Microbiota at the crossroads of autoimmunity. Autoimmun Rev. 2016;15:859–69.

    Article  CAS  PubMed  Google Scholar 

  44. 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.

    Article  CAS  PubMed  Google Scholar 

  45. 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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Kirino Y, Remmers EF. Genetic architectures of seropositive and seronegative rheumatic diseases. Nat Rev Rheumatol. 2015;11:401–14.

    Article  CAS  PubMed  Google Scholar 

  47. 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.

    Article  CAS  PubMed  Google Scholar 

  48. 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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Matthew J. S. Parker .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

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)

Publish with us

Policies and ethics