Skip to main content

Paraneoplastic Myopathy

  • Chapter
  • First Online:
Immune-Mediated Myopathies and Neuropathies
  • 620 Accesses

Abstract

Paraneoplastic syndromes are diseases caused by malignancies through means other than mass effect or metastasis [1]. Even though they occur in only about 10% of patients with cancer, physicians are wise to be vigilant for them for several reasons: a paraneoplastic phenomenon can be the first sign of cancer in an undiagnosed individual, and it can be severe enough to cause death. Despite the tumor being distinctly separate from the area where the paraneoplastic syndrome manifests, it is important to realize that paraneoplastic phenomena are not caused by metastases of the neoplasm. It has been recognized that compared with the normal population, patients with idiopathic inflammatory myopathies (IIM) live with an increased risk of developing malignancy. Cancer-associated myositis (CAM) and the malignancy of which it is a paraneoplastic phenomenon can have a parallel clinical course, and relapse of malignancy can be accompanied by a similar resurgence of myositis. Patients with CAM rarely have myositis-specific and myositis-associated autoantibodies. The features of an inflammatory myopathy that are associated with a lower risk of cancer include the anti-Jo-1 antibody, anti-extractable nuclear antigens (including anti-SM, anti-RNP, anti-RO/LA) antibodies, interstitial lung disease, joint involvement, and Raynaud phenomenon. Age-appropriate cancer screening should be ensured in patients with inflammatory myopathy, and this vigilance for underlying cancer should be continued in case the malignancy develops years after the myopathy. Other means of discovering a malignancy in this setting include imaging the chest, abdomen, and pelvis and checking serum levels of tumor markers.

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 119.00
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. Khan F, Kleppel H, Meara A. Paraneoplastic musculoskeletal syndromes. Rheum Dis Clin N Am. 2020;46:577–86.

    Article  Google Scholar 

  2. Ponyi A, Constantin T, Garami M, et al. Cancer-associated myositis: clinical features and prognostic signs. Ann N Y Acad Sci. 2005;1051:64–71.

    Article  PubMed  Google Scholar 

  3. Madan V, Chinoy H, Griffiths CE, Cooper RG. Defining cancer risk in dermatomyositis. Part I. Clin Exp Dermatol. 2009;34:451–5.

    Article  CAS  PubMed  Google Scholar 

  4. Andras C, Ponyi A, Constantin T, et al. Dermatomyositis and polymyositis associated with malignancy: a 21-year retrospective study. J Rheumatol. 2008;35:438–44.

    PubMed  Google Scholar 

  5. Zahr ZA, Baer AN. Malignancy in myositis. Curr Rheumatol Rep. 2011;13:208–15.

    Article  PubMed  Google Scholar 

  6. Bernet LL, Lewis MA, Rieger KE, Casciola-Rosen L, Fiorentino DF. Ovoid palatal patch in dermatomyositis: a novel finding associated with anti-TIF1gamma (p155) antibodies. JAMA Dermatol. 2016;152:1049–51.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Fiorentino DF, Chung LS, Christopher-Stine L, et al. Most patients with cancer-associated dermatomyositis have antibodies to nuclear matrix protein NXP-2 or transcription intermediary factor 1gamma. Arthritis Rheum. 2013;65:2954–62.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Albayda J, Pinal-Fernandez I, Huang W, et al. Antinuclear matrix protein 2 autoantibodies and edema, muscle disease, and malignancy risk in dermatomyositis patients. Arthritis Care Res (Hoboken). 2017;69:1771–6.

    Article  CAS  PubMed  Google Scholar 

  9. 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  PubMed  Google Scholar 

  10. Matsuo H, Yanaba K, Umezawa Y, Nakagawa H, Muro Y. Anti-SAE antibody-positive dermatomyositis in a Japanese patient: a case report and review of the literature. J Clin Rheumatol. 2019;25:e115–e6.

    Article  PubMed  Google Scholar 

  11. Joseph CG, Darrah E, Shah AA, et al. Association of the autoimmune disease scleroderma with an immunologic response to cancer. Science. 2014;343:152–7.

    Article  CAS  PubMed  Google Scholar 

  12. Aussy A, Boyer O, Cordel N. Dermatomyositis and immune-mediated necrotizing myopathies: a window on autoimmunity and cancer. Front Immunol. 2017;8:992.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Buchbinder R, Forbes A, Hall S, Dennett X, Giles G. Incidence of malignant disease in biopsy-proven inflammatory myopathy. A population-based cohort study. Ann Intern Med. 2001;134:1087–95.

    Article  CAS  PubMed  Google Scholar 

  14. Sigurgeirsson B, Lindelof B, Edhag O, Allander E. Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study. N Engl J Med. 1992;326:363–7.

    Article  CAS  PubMed  Google Scholar 

  15. Hill CL, Zhang Y, Sigurgeirsson B, et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001;357:96–100.

    Article  CAS  PubMed  Google Scholar 

  16. Casciola-Rosen L, Nagaraju K, Plotz P, et al. Enhanced autoantigen expression in regenerating muscle cells in idiopathic inflammatory myopathy. J Exp Med. 2005;201:591–601.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Pinal-Fernandez I, Ferrer-Fabregas B, Trallero-Araguas E, et al. Tumour TIF1 mutations and loss of heterozygosity related to cancer-associated myositis. Rheumatology (Oxford). 2018;57:388–96.

    Article  CAS  PubMed  Google Scholar 

  18. Chen H, Peng Q, Yang H, et al. Increased levels of soluble programmed death ligand 1 associate with malignancy in patients with dermatomyositis. J Rheumatol. 2018;45:835–40.

    Article  CAS  PubMed  Google Scholar 

  19. Waldman R, DeWane ME, Lu J. Dermatomyositis: diagnosis and treatment. J Am Acad Dermatol. 2020;82:283–96.

    Article  PubMed  Google Scholar 

  20. Qiang JK, Kim WB, Baibergenova A, Alhusayen R. Risk of malignancy in dermatomyositis and polymyositis. J Cutan Med Surg. 2017;21:131–6.

    Article  PubMed  Google Scholar 

  21. Best M, Molinari N, Chasset F, Vincent T, Cordel N, Bessis D. Use of anti-transcriptional intermediary factor-1 gamma autoantibody in identifying adult dermatomyositis patients with cancer: a systematic review and meta-analysis. Acta Derm Venereol. 2019;99:256–62.

    Article  CAS  PubMed  Google Scholar 

  22. Venturini L, You J, Stadler M, et al. TIF1gamma, a novel member of the transcriptional intermediary factor 1 family. Oncogene. 1999;18:1209–17.

    Article  CAS  PubMed  Google Scholar 

  23. Aussy A, Freret M, Gallay L, et al. The IgG2 isotype of anti-transcription intermediary factor 1gamma autoantibodies is a biomarker of cancer and mortality in adult dermatomyositis. Arthritis Rheumatol. 2019;71:1360–70.

    Article  CAS  PubMed  Google Scholar 

  24. Greenberg SA. Inclusion body myositis: clinical features and pathogenesis. Nat Rev Rheumatol. 2019;15:257–72.

    Article  PubMed  Google Scholar 

  25. Allenbach Y, Benveniste O, Stenzel W, Boyer O. Immune-mediated necrotizing myopathy: clinical features and pathogenesis. Nat Rev Rheumatol. 2020;16:689–701.

    Article  CAS  PubMed  Google Scholar 

  26. Allenbach Y, Keraen J, Bouvier AM, et al. High risk of cancer in autoimmune necrotizing myopathies: usefulness of myositis specific antibody. Brain. 2016;139:2131–5.

    Article  PubMed  Google Scholar 

  27. Kadoya M, Hida A, Hashimoto Maeda M, et al. Cancer association as a risk factor for anti-HMGCR antibody-positive myopathy. Neurol Neuroimmunol Neuroinflamm. 2016;3:e290.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Tiniakou E, Pinal-Fernandez I, Lloyd TE, et al. More severe disease and slower recovery in younger patients with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase-associated autoimmune myopathy. Rheumatology (Oxford). 2017;56:787–94.

    CAS  PubMed  Google Scholar 

  29. Levin MI, Mozaffar T, Al-Lozi MT, Pestronk A. Paraneoplastic necrotizing myopathy: clinical and pathological features. Neurology. 1998;50:764–7.

    Article  CAS  PubMed  Google Scholar 

  30. Vu HJ, Pham D, Makary R, Nguyen T, Shuja S. Paraneoplastic necrotizing myopathy presenting as severe muscle weakness in a patient with small-cell lung cancer: successful response to chemoradiation therapy. Clin Adv Hematol Oncol. 2011;9:557–6.

    PubMed  Google Scholar 

  31. Opinc AH, Makowska JS. Antisynthetase syndrome - much more than just a myopathy. Semin Arthritis Rheum. 2021;51:72–83.

    Article  CAS  PubMed  Google Scholar 

  32. Hervier B, Devilliers H, Stanciu R, et al. Hierarchical cluster and survival analyses of antisynthetase syndrome: phenotype and outcome are correlated with anti-tRNA synthetase antibody specificity. Autoimmun Rev. 2012;12:210–7.

    Article  CAS  PubMed  Google Scholar 

  33. Trallero-Araguas E, Grau-Junyent JM, Labirua-Iturburu A, et al. Clinical manifestations and long-term outcome of anti-Jo1 antisynthetase patients in a large cohort of Spanish patients from the GEAS-IIM group. Semin Arthritis Rheum. 2016;46:225–31.

    Article  CAS  PubMed  Google Scholar 

  34. Castaneda-Pomeda M, Prieto-Gonzalez S, Grau JM. Antisynthetase syndrome and malignancy: our experience. J Clin Rheumatol. 2011;17:458.

    Article  PubMed  Google Scholar 

  35. Mileti LM, Strek ME, Niewold TB, Curran JJ, Sweiss NJ. Clinical characteristics of patients with anti-Jo-1 antibodies: a single center experience. J Clin Rheumatol. 2009;15:254–5.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Boleto G, Perotin JM, Eschard JP, Salmon JH. Squamous cell carcinoma of the lung associated with anti-Jo1 antisynthetase syndrome: a case report and review of the literature. Rheumatol Int. 2017;37:1203–6.

    Article  CAS  PubMed  Google Scholar 

  37. Rozelle A, Trieu S, Chung L. Malignancy in the setting of the anti-synthetase syndrome. J Clin Rheumatol. 2008;14:285–8.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

Copyright information

© 2023 The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd.

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Gaspar, B.L. (2023). Paraneoplastic Myopathy. In: Immune-Mediated Myopathies and Neuropathies. Springer, Singapore. https://doi.org/10.1007/978-981-19-8421-1_11

Download citation

  • DOI: https://doi.org/10.1007/978-981-19-8421-1_11

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-19-8420-4

  • Online ISBN: 978-981-19-8421-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics