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Malignancy in Myositis

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Abstract

Dermatomyositis is associated with an underlying malignancy in about 24% of cases. This association is also true for polymyositis but is less prominent. The malignancy is usually an adenocarcinoma of the ovary, lung, or gastrointestinal tract in Western countries and nasopharyngeal carcinoma in Southeast Asia, Southern China, and Northern Africa. Factors predictive of malignancy in myositis patients include more severe skin and muscle disease and the absence of overlap connective tissue disease features, such as interstitial lung disease. Anti-p155/140 antibodies have a strong predictive value for malignancy in adult patients. Patients with dermatomyositis or polymyositis require an evaluation for occult malignancy at the time of diagnosis and, in some cases, in the event of a subsequent recurrence. This paraneoplastic phenomenon may stem from an immune reaction to antigens expressed in both cancer cells and regenerating fibers in affected muscle.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Stertz G. Polymyositis. Berl Klin Wochenschr. 1916;53:489.

    Google Scholar 

  2. Buchbinder R, Hill CL. Malignancy in patients with inflammatory myopathy. Curr Rheumatol Rep. 2002;4(5):415–26.

    Article  PubMed  Google Scholar 

  3. Baer AN, Wortmann RL. The risk of malignancy in patients with dermatomyositis and polymyositis. In: Kagen LJ, editor. The Inflammatory Myopathies. New York: Humana Press; 2009. p. 307–18.

    Chapter  Google Scholar 

  4. • Zhang W, Jiang SP, Huang L. Dermatomyositis and malignancy: a retrospective study of 115 cases. Eur Rev Med Pharmacol Sci. 2009;13(2):77-80. This was a retrospective review of 115 cases of cancer-associated DM seen at a tertiary medical center in Guangdong, China, between 1974 and 2008. Nasopharyngeal carcinoma constituted 51% of the associated malignancies.

    PubMed  Google Scholar 

  5. • Prohic A, Kasumagic-Halilovic E, Simic D, et al. Clinical and biological factors predictive of malignancy in dermatomyositis. J Eur Acad Dermatol Venereol. 2009;23(5):591-2. The clinical and laboratory features of 32 patients with DM were reviewed in order to identify risk factors for associated cancer. Cutaneous necrosis and higher levels of muscle enzyme levels were significantly more common in the 8 patients with cancer than the 24 without cancer.

    Article  PubMed  CAS  Google Scholar 

  6. •• Fardet L, Dupuy A, Gain M, et al. Factors associated with underlying malignancy in a retrospective cohort of 121 patients with dermatomyositis. Medicine (Baltimore). 2009;88(2):91-7. In this retrospective study of 121 adult DM patients seen at 2 French tertiary medical centers, 29 had cancer-associated myositis. The authors identified independent factors associated with underlying malignancy using multivariate analysis. These included age older than 52 years, rapid onset of skin and muscle symptoms, presence of skin necrosis or periungual erythema, and a baseline low complement 4 level.

    Article  Google Scholar 

  7. • Toumi S, Ghnaya H, Braham A, et al. Polymyositis and dermatomyositis in adults. Tunisian multicentre study. Rev Med Interne. 2009;30(9):747-53. This was a retrospective, multicenter study of 70 adult patients with PM and DM seen in hospital centers in Tunisia. Nine patients had an associated malignancy, three with nasopharyngeal and three with breast carcinomas.

    Article  PubMed  CAS  Google Scholar 

  8. • Diallo M, Fall AK, Diallo I, et al. Dermatomyositis and polymyositis: 21 cases in Senegal. Med Trop (Mars). 2010;70(2):166-8. This was a retrospective review of 21 black African patients hospitalized for PM or DM at the Principal Hospital in Dakar, Senegal. Three of the patients, all with DM, had an associated malignancy.

    CAS  Google Scholar 

  9. • Na SJ, Kim SM, Sunwoo IN, et al. Clinical characteristics and outcomes of juvenile and adult dermatomyositis. J Korean Med Sci. 2009;24(4):715-21. The authors studied the clinical features and outcomes of 16 patients with juvenile DM and 48 with adult DM. Neck muscle weakness and subcutaneous calcification were more common in the juvenile DM patients. Six adult DM patients had an underlying malignancy, five of whom died from cancer within 4 years of myositis onset.

    Article  PubMed  Google Scholar 

  10. •• Antiochos BB, Brown LA, Li Z, et al. Malignancy is associated with dermatomyositis but not polymyositis in Northern New England, USA. J Rheumatol. 2009;36(12):2704-10. This was a retrospective review of 198 patients with IIM seen at the Dartmouth-Hitchcock Medical Center between 1985 and 2008. Malignancy developed within 5 years of the diagnosis of IIM in 16% of the patients, most commonly involving the breast, lung, pancreas, and colon. Interstitial lung disease was not present in any of the IIM patients who had an associated malignancy.

    Article  PubMed  Google Scholar 

  11. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292(7):344–7.

    Article  PubMed  CAS  Google Scholar 

  12. Chow WH, Gridley G, Mellemkjaer L, et al. Cancer risk following polymyositis and dermatomyositis: a nationwide cohort study in Denmark. Cancer Causes Control. 1995;6(1):9–13.

    Article  PubMed  CAS  Google Scholar 

  13. Airio A, Pukkala E, Isomaki H. Elevated cancer incidence in patients with dermatomyositis: a population based study. J Rheumatol. 1995;22(7):1300–3.

    PubMed  CAS  Google Scholar 

  14. Stockton D, Doherty VR, Brewster DH. Risk of cancer in patients with dermatomyositis or polymyositis, and follow-up implications: a Scottish population-based cohort study. Br J Cancer. 2001;85(1):41–5.

    Article  PubMed  CAS  Google Scholar 

  15. Sigurgeirsson B, Lindelof B, Edhag O, et al. Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study N Engl J Med. 1992;326(6):363–7.

    CAS  Google Scholar 

  16. Buchbinder R, Forbes A, Hall S, et al. Incidence of malignant disease in biopsy-proven inflammatory myopathy. A population-based cohort study Ann Intern Med. 2001;134(12):1087–95.

    CAS  Google Scholar 

  17. •• Chen YJ, Wu CY, Huang YL, et al. Cancer risks of dermatomyositis and polymyositis: a nationwide cohort study in Taiwan. Arthritis Res Ther. 2010;12(2):R70. The authors conducted a nationwide cohort study of 1,012 DM and 643 PM patients without a prior history of malignancy, identified in a National Health Insurance Database for the years 1997 to 2007. Cancers developed in 95 of the DM and 33 of the PM patients, most often in the first year of observation. The cancer risks for both DM and PM were significantly elevated as assessed by standardized incidence ratios.

    Article  PubMed  Google Scholar 

  18. Hill CL, Zhang Y, Sigurgeirsson B, et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001;357(9250):96–100.

    Article  PubMed  CAS  Google Scholar 

  19. •• Huang YL, Chen YJ, Lin MW, et al. Malignancies associated with dermatomyositis and polymyositis in Taiwan: a nationwide population-based study. Br J Dermatol. 2009;161(4):854-60. In this nationwide cohort study of 1,059 DM and 661 PM patients identified in a National Health Insurance Database, the types of associated cancer in 136 DM and 46 PM patients were analyzed. Cancers of the nasopharynx, lung, and breast were most common in the DM patients, and cancers of the breast, uterine cervix, and lung were most common in the PM patients. The risk of nasopharyngeal carcinoma was increased 66-fold over that of the general population.

    Article  Google Scholar 

  20. Cherin P, Piette JC, Herson S, et al. Dermatomyositis and ovarian cancer: a report of 7 cases and literature review. J Rheumatol. 1993;20(11):1897–9.

    PubMed  CAS  Google Scholar 

  21. Fujita J, Tokuda M, Bandoh S, et al. Primary lung cancer associated with polymyositis/dermatomyositis, with a review of the literature. Rheumatol Int. 2001;20(2):81–4.

    Article  PubMed  CAS  Google Scholar 

  22. Masuda H, Urushibara M, Kihara K. Successful treatment of dermatomyositis associated with adenocarcinoma of the prostate after radical prostatectomy. J Urol. 2003;169(3):1084.

    Article  PubMed  Google Scholar 

  23. Yoshinaga A, Hayashi T, Ishii N, et al. Successful cure of dermatomyositis after treatment of nonseminomatous testicular cancer. Int J Urol. 2005;12(6):593–5.

    Article  PubMed  Google Scholar 

  24. Bonnetblanc JM, Bernard P, Fayol J. Dermatomyositis and malignancy. A multicenter cooperative study. Dermatologica. 1990;180(4):212–6.

    Article  PubMed  CAS  Google Scholar 

  25. Andras C, Ponyi A, Constantin T, et al. Dermatomyositis and polymyositis associated with malignancy: a 21-year retrospective study. J Rheumatol. 2008;35(3):438–44.

    PubMed  Google Scholar 

  26. 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(4):591–601.

    Article  PubMed  CAS  Google Scholar 

  27. 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 

  28. Hunger RE, Durr C, Brand CU. Cutaneous leukocytoclastic vasculitis in dermatomyositis suggests malignancy. Dermatology. 2001;202(2):123–6.

    Article  PubMed  CAS  Google Scholar 

  29. • Selva-O'Callaghan A, Fonollosa-Pla V, Trallero-Araguas E, et al. Nailfold capillary microscopy in adults with inflammatory myopathy. Semin Arthritis Rheum. 2010;39(5):398-404. In this study of nailfold capillary alterations in patients with inflammatory myopathy, the authors noted a severe capillary derangement pattern in 3 of 6 patients with paraneoplastic myositis, as opposed to 3 of the remaining 47 IIM patients.

    Article  PubMed  Google Scholar 

  30. Basset-Seguin N, Roujeau JC, Gherardi R, et al. Prognostic factors and predictive signs of malignancy in adult dermatomyositis. A study of 32 cases. Arch Dermatol. 1990;126(5):633–7.

    Article  PubMed  CAS  Google Scholar 

  31. Ang P, Sugeng MW, Chua SH. Classical and amyopathic dermatomyositis seen at the National Skin Centre of Singapore: a 3-year retrospective review of their clinical characteristics and association with malignancy. Ann Acad Med Singapore. 2000;29(2):219–23.

    PubMed  CAS  Google Scholar 

  32. Amerio P, Girardelli CR, Proietto G, et al. Usefulness of erythrocyte sedimentation rate as tumor marker in cancer associated dermatomyositis. Eur J Dermatol. 2002;12(2):165–9.

    PubMed  Google Scholar 

  33. Amoura Z, Duhaut P, Huong DL, et al. Tumor antigen markers for the detection of solid cancers in inflammatory myopathies. Cancer Epidemiol Biomarkers Prev. 2005;14(5):1279–82.

    Article  PubMed  CAS  Google Scholar 

  34. Whitmore SE, Anhalt GJ, Provost TT, et al. Serum CA-125 screening for ovarian cancer in patients with dermatomyositis. Gynecol Oncol. 1997;65(2):241–4.

    Article  PubMed  CAS  Google Scholar 

  35. Chen YJ, Wu CY, Shen JL. Predicting factors of malignancy in dermatomyositis and polymyositis: a case-control study. Br J Dermatol. 2001;144(4):825–31.

    Article  PubMed  CAS  Google Scholar 

  36. • Marie I, Lahaxe L, Benveniste O, et al. Long-term outcome of patients with polymyositis/dermatomyositis and anti-PM-Scl antibody. Br J Dermatol. 2010;162(2):337-44. The long-term outcomes of 20 patients with PM/DM and anti–PM-Scl antibodies were analyzed. These patients had a high frequency of severe dysphagia, respiratory insufficiency, and malignancy, suggesting that this antibody is a poor prognostic factor in patients with PM/DM.

    Article  PubMed  CAS  Google Scholar 

  37. Chinoy H, Fertig N, Oddis CV, et al. The diagnostic utility of myositis autoantibody testing for predicting the risk of cancer-associated myositis. Ann Rheum Dis. 2007;66(10):1345–9.

    Article  PubMed  Google Scholar 

  38. Legault D, McDermott J, Crous-Tsanaclis AM, et al. Cancer-associated myositis in the presence of anti-Jo1 autoantibodies and the antisynthetase syndrome. J Rheumatol. 2008;35(1):169–71.

    PubMed  Google Scholar 

  39. Rozelle A, Trieu S, Chung L. Malignancy in the setting of the anti-synthetase syndrome. J Clin Rheumatol. 2008;14(5):285–8.

    Article  PubMed  Google Scholar 

  40. Targoff IN, Mamyrova G, Trieu EP, et al. A novel autoantibody to a 155-kd protein is associated with dermatomyositis. Arthritis Rheum. 2006;54(11):3682–9.

    Article  PubMed  CAS  Google Scholar 

  41. Kaji K, Fujimoto M, Hasegawa M, et al. Identification of a novel autoantibody reactive with 155 and 140 kDa nuclear proteins in patients with dermatomyositis: an association with malignancy. Rheumatology (Oxford). 2007;46(1):25–8.

    Article  CAS  Google Scholar 

  42. Gunawardena H, Wedderburn LR, North J, et al. Clinical associations of autoantibodies to a p155/140 kDa doublet protein in juvenile dermatomyositis. Rheumatology (Oxford). 2008;47(3):324–8.

    Article  CAS  Google Scholar 

  43. • Selva-O'Callaghan A, Trallero-Araguas E, Grau-Junyent JM, et al. Malignancy and myositis: novel autoantibodies and new insights. Curr Opin Rheumatol. 2010;22(6):627-32. In this review, the authors reported on a meta-analysis of the published studies on the predictive accuracy of anti-p155 antibodies for the detection of occult malignancy in IM patients. They also proposed a diagnostic algorithm for the diagnosis of occult malignancy in myositis; it includes testing for anti-p155 antibodies, a test that is not commercially available.

    Article  PubMed  Google Scholar 

  44. Targoff IN, Trieu EP, Levy-Noto M, et al. Autoantibodies to transcriptional intermediary factor 1-gamma in dermatomyositis [abstract]. Arthritis Rheum. 2006;54:S518.

    Article  Google Scholar 

  45. Gordon KJ, Blobe GC. Role of transforming growth factor-beta superfamily signaling pathways in human disease. Biochim Biophys Acta. 2008;1782(4):197–228.

    PubMed  CAS  Google Scholar 

  46. Cox NH, Lawrence CM, Langtry JA, et al. Dermatomyositis. Disease associations and an evaluation of screening investigations for malignancy. Arch Dermatol. 1990;126(1):61–5.

    Article  PubMed  CAS  Google Scholar 

  47. Hatada T, Aoki I, Ikeda H, et al. Dermatomyositis and malignancy: case report and review of the Japanese literature. Tumori. 1996;82(3):273–5.

    PubMed  CAS  Google Scholar 

  48. Schulman P, Kerr LD, Spiera H. A reexamination of the relationship between myositis and malignancy. J Rheumatol. 1991;18(11):1689–92.

    PubMed  CAS  Google Scholar 

  49. •• Selva-O'Callaghan A, Grau JM, Gamez-Cenzano C, et al. Conventional cancer screening versus PET/CT in dermatomyositis/polymyositis. Am J Med. 2010;123(6):558-62. In this prospective study of 55 consecutive patients with a recent diagnosis of myositis, whole-body fludeoxyglucose-positron emission tomography/CT scanning had a performance comparable to that of broad conventional screening (thoracoabdominal CT, mammography, gynecologic examination, ultrasonography, and tumor marker analysis) in the detection of occult malignant disease.

    Article  PubMed  Google Scholar 

  50. •• Zampieri S, Valente M, Adami N, Biral D, Ghirardello A, Rampudda ME, et al. Polymyositis, dermatomyositis and malignancy: A further intriguing link. Autoimmun Rev. 2010;9(6):449-53. Muscle biopsies from patients with newly diagnosed colon cancer but without overt myositis showed histologic abnormalities, including fiber internalization of myonuclei and immunohistochemical staining for two markers of myofiber regeneration. These results provide evidence for muscle damage occurring in the setting of distant malignancy and support the hypothesis that regenerating muscle is the source of Jo-1 and other antigen expression that underlies the autoimmune response in cancer-associated myositis.

    Article  PubMed  CAS  Google Scholar 

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Zahr, Z.A., Baer, A.N. Malignancy in Myositis. Curr Rheumatol Rep 13, 208–215 (2011). https://doi.org/10.1007/s11926-011-0169-7

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