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.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Khan F, Kleppel H, Meara A. Paraneoplastic musculoskeletal syndromes. Rheum Dis Clin N Am. 2020;46:577–86.
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.
Madan V, Chinoy H, Griffiths CE, Cooper RG. Defining cancer risk in dermatomyositis. Part I. Clin Exp Dermatol. 2009;34:451–5.
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.
Zahr ZA, Baer AN. Malignancy in myositis. Curr Rheumatol Rep. 2011;13:208–15.
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.
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.
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.
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.
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.
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.
Aussy A, Boyer O, Cordel N. Dermatomyositis and immune-mediated necrotizing myopathies: a window on autoimmunity and cancer. Front Immunol. 2017;8:992.
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.
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.
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.
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.
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.
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.
Waldman R, DeWane ME, Lu J. Dermatomyositis: diagnosis and treatment. J Am Acad Dermatol. 2020;82:283–96.
Qiang JK, Kim WB, Baibergenova A, Alhusayen R. Risk of malignancy in dermatomyositis and polymyositis. J Cutan Med Surg. 2017;21:131–6.
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.
Venturini L, You J, Stadler M, et al. TIF1gamma, a novel member of the transcriptional intermediary factor 1 family. Oncogene. 1999;18:1209–17.
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.
Greenberg SA. Inclusion body myositis: clinical features and pathogenesis. Nat Rev Rheumatol. 2019;15:257–72.
Allenbach Y, Benveniste O, Stenzel W, Boyer O. Immune-mediated necrotizing myopathy: clinical features and pathogenesis. Nat Rev Rheumatol. 2020;16:689–701.
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.
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.
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.
Levin MI, Mozaffar T, Al-Lozi MT, Pestronk A. Paraneoplastic necrotizing myopathy: clinical and pathological features. Neurology. 1998;50:764–7.
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.
Opinc AH, Makowska JS. Antisynthetase syndrome - much more than just a myopathy. Semin Arthritis Rheum. 2021;51:72–83.
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.
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.
Castaneda-Pomeda M, Prieto-Gonzalez S, Grau JM. Antisynthetase syndrome and malignancy: our experience. J Clin Rheumatol. 2011;17:458.
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.
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.
Rozelle A, Trieu S, Chung L. Malignancy in the setting of the anti-synthetase syndrome. J Clin Rheumatol. 2008;14:285–8.
Author information
Authors and Affiliations
Rights and permissions
Copyright information
© 2023 The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd.
About this chapter
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)