Malignancy Risk and Myositis-Specific Autoantibodies in Dermatomyositis: A Comprehensive Review
DOI:
https://doi.org/10.12775/QS.2026.60.72808Keywords
dermatomyositis, cancer risk, dermatomyositis autoantibodies, anti-TIF1, anti-NXP2, anti-SAE, anti-Mi-2, anti-MDA5, malignancy, MSAsAbstract
Background. Dermatomyositis is an inflammatory myopathy characterized by proximal muscle weakness, cutaneous manifestations and systemic complications. DM has an association with cancer. Meta-analyses indicating an OR of 4.66 for developing malignancies. Especially within the first year of DM diagnosis. Myositis-specific autoantibodies (MSAs) have a distinct correlation with clinical phenotypes and the risk of developing a malignancy.
Aim. The aim of this review is to synthesize current data on the risk of malignancy in DM patients positive for MSAs and to determinate how the presence of those antibodies affects the course of cancer.
Material and methods. Comprehensive literature research was conducted across PubMed, Scopus, Web of Science, and Google Scholar databases focusing primary on publications from the past 20 years (2006-2026). Eligibility was restricted to systematic reviews, retrospective studies, cohort studies, and case reports, while conference abstracts and studies specifically concerning juvenile dermatomyositis were excluded.
Results. Anti-TIF1γ antibody presents the strongest correlation with malignancy. The predominant cancer types are breast, lung, ovarian and gastrointestinal cancer. Solid tumours presented at an advanced stage. The risk is increased by the presence of anti-TIF1α antibody, age over 60 and male sex. Patients with anti-NXP2 antibodies have a 2.5 to 3.68-fold increased risk of cancer. Tumours present at an advanced stage. Male sex and age over 60 are risk factors. Anti-Mi-2 and anti-SAE antibodies are highly population-dependent.The anti-MDA5 antibody presents a weak association with malignancy.
Conclusions. MSAs provide prognostic value for stratifying malignancy risk and optimizing screening timelines in adult dermatomyositis. Seropositivity for anti-TIF1γ and anti-NXP2 requires a prompt cancer screening within the first year of DM onset. Systematic malignancy surveillance should be standardized for all DM patients
References
1. Targoff IN, Mamyrova G, Trieu EP, et al; Childhood Myositis Heterogeneity Study Group; International Myositis Collaborative Study Group. A novel autoantibody to a 155-kd protein is associated with dermatomyositis. Arthritis Rheum. 2006;54(11):3682-3689. doi:10.1002/art.22164
2. Marzęcka M, Niemczyk A, Rudnicka L. Autoantibody markers of increased risk of malignancy in patients with dermatomyositis. Clin Rev Allergy Immunol. 2022;63(2):289-296. doi:10.1007/s12016-022-08922-4
3. Bax CE, Casciola-Rosen L, Fiorentino D. Autoantibodies as biomarkers of cancer risk in dermatomyositis. JID Innov. 2026;6(3):100460. doi:10.1016/j.xjidi.2026.100460
4. 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(3):256-262. doi:10.2340/00015555-3091
5. Ogawa-Momohara M, Muro Y, Mitsuma T, et al. Strong correlation between cancer progression and anti-transcription intermediary factor 1γ antibodies in dermatomyositis patients. Clin Exp Rheumatol. 2018;36(6):990-995.
6. Syrmou V, Liaskos C, Patrikious E, Alexiou I, Simopoulou T, Katsiari CG, Bogdanos DP. Clinical profile and outcomes in anti-TIF1γ positive idiopathic inflammatory myositis patients: a Greek cohort study. Mediterr J Rheumatol. 2025;36(2):200-209. doi:10.31138/mjr.300525.iao
7. 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(9250):96-100. doi:10.1016/S0140-6736(00)03540-6
8. Fiorentino DF, Kuo K, Chung L, Zaba L, Li S, Casciola-Rosen L. Distinctive cutaneous and systemic features associated with antitranscriptional intermediary factor-1γ antibodies in adults with dermatomyositis. J Am Acad Dermatol. 2015;72(3):449-455. doi:10.1016/j.jaad.2014.12.009
9. Malinić M, Reljić V, Vuković J, Tanasilović S, Živanović D. Dermatomyositis in women: a 15-year retrospective analysis of clinical patterns, malignancy risk, and long-term outcomes at a tertiary center. Acta Dermatovenerol Alp Pannonica Adriat. 2025;34(4):165-169.
10. Harada Y, Tominaga M, Iitoh E, et al. Clinical characteristics of anti-TIF-1γ antibody-positive dermatomyositis associated with malignancy. J Clin Med. 2022;11(7):1925. doi:10.3390/jcm11071925
11. Lauinger J, Ghoreschi K, Volc S. Characteristics of dermatomyositis patients with and without associated malignancy. J Dtsch Dermatol Ges. 2021;19(11):1601-1611. doi:10.1111/ddg.14566
12. Patt YS, Ben-Shabat N, David P, et al. Malignancy risk and predictors in dermatomyositis and polymyositis: a large population-based study. Medicina (Kaunas). 2025;61(11):1932. doi:10.3390/medicina61111932
13. Ono R, Kumagae T, Igasaki M, Murata T, Yoshizawa M, Kitagawa I. Anti-transcription intermediary factor 1 gamma (TIF1γ) antibody-positive dermatomyositis associated with ascending colon cancer: a case report and review of the literature. J Med Case Rep. 2021;15(1):142. doi:10.1186/s13256-021-02664-1
14. Tang ZL, Chi CC, Tang ZW, Li XW, Man XY. Malignancy in dermatomyositis: a mono-centric retrospective study of 134 patients in China and a potential predictive model. Front Med (Lausanne). 2023;10:1200804. doi:10.3389/fmed.2023.1200804
15. Mecoli CA, Igusa T, Chen M, et al. Subsets of idiopathic inflammatory myositis enriched for contemporaneous cancer relative to the general population. Arthritis Rheumatol. 2023;75(4):620-629. doi:10.1002/art.42311
16. Oldroyd A, Sergeant JC, New P, et al; UKMyoNet. The temporal relationship between cancer and adult-onset anti-transcriptional intermediary factor 1 antibody-positive dermatomyositis. Rheumatology (Oxford). 2019;58(4):650-655. doi:10.1093/rheumatology/key357
17. Qiang JK, Kim WB, Baibergenova A, Alhusayen R. Risk of malignancy in dermatomyositis and polymyositis. J Cutan Med Surg. 2017;21(2):131-136. doi:10.1177/1203475416665601
18. Fujimoto M, Hamaguchi Y, Kaji K, et al. Myositis-specific anti-155/140 autoantibodies target transcription intermediary factor 1 family proteins. Arthritis Rheum. 2012;64(2):513-522. doi:10.1002/art.33403
19. 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 1γ. Arthritis Rheum. 2013;65(11):2954-2962. doi:10.1002/art.38093
20. Fiorentino D, Mecoli CA, Igusa T, et al. Association of anti-CCAR1 autoantibodies with decreased cancer risk relative to the general population in patients with anti-transcriptional intermediary factor 1γ-positive dermatomyositis. Arthritis Rheumatol. 2023;75(7):1238-1245. doi:10.1002/art.42474
21. Mecoli CA, Fiorentino D, Albayda J, et al. The relationship between anti-cell division cycle and apoptosis regulator 1 autoantibodies, anti-Sp4 autoantibodies, and cancer in anti-transcription intermediary factor 1γ-positive dermatomyositis. ACR Open Rheumatol. 2024;6(12):912-917. doi:10.1002/acr2.11750
22. 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(11):1771-1776. doi:10.1002/acr.23188
23. Ichimura Y, Matsushita T, Hamaguchi Y, et al. Anti-NXP2 autoantibodies in adult patients with idiopathic inflammatory myopathies: possible association with malignancy. Ann Rheum Dis. 2012;71(5):710-713. doi:10.1136/annrheumdis-2011-200697
24. Betteridge Z, Gunawardena H, North J, Slinn J, McHugh N. Identification of a novel autoantibody directed against small ubiquitin-like modifier activating enzyme in dermatomyositis. Arthritis Rheum. 2007;56(9):3132-3137. doi:10.1002/art.22862
25. Zhang Y, Liu L, Duan X, et al. Longitudinal study of patients with anti-SAE antibody-positive dermatomyositis: a multicenter cohort study in China. Rheumatology (Oxford). 2025;64(3):1377-1385. doi:10.1093/rheumatology/keae232
26. Saardi KM, Zachary CM, DeWitt CA, Cardis MA. Dermatomyositis: clinical features and pathogenesis. J Am Acad Dermatol. 2020;83(1):e21-e22. doi:10.1016/j.jaad.2020.02.083
27. Albayda J, Mecoli C, Casciola-Rosen L, et al. A North American cohort of anti-SAE dermatomyositis: clinical phenotype, testing, and review of cases. ACR Open Rheumatol. 2021;3(5):287-294. doi:10.1002/acr2.11247
28. Depascale R, Ghirardello A, Zanatta E, et al. Patients with anti-SAE+ dermatomyositis display refractory and difficult-to-treat skin manifestations: case series from two Italian cohorts and review of literature. Front Immunol. 2025;16:1597282. doi:10.3389/fimmu.2025.1597282
29. Betteridge Z, Tansley S, Shaddick G, et al. Frequency, mutual exclusivity and clinical associations of myositis autoantibodies in a combined European cohort of idiopathic inflammatory myopathy patients. J Autoimmun. 2019;101:48-55. doi:10.1016/j.jaut.2019.04.001
30. Salcedo-Soto DA, Arvizu-Rivera RI, Garza-Elizondo AK, et al. Clinical characteristics of Hispanic patients with anti-SAE dermatomyositis. Clin Rheumatol. 2025;44(5):1983-1987. doi:10.1007/s10067-025-07419-7
31. 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(9):1745-1747. doi:10.1093/rheumatology/kev247
32. Demortier J, Vautier M, Chosidow O, et al. Anti-SAE autoantibody in dermatomyositis: original comparative study and review of the literature. Rheumatology (Oxford). 2023;62(12):3932-3939. doi:10.1093/rheumatology/kead154
33. Satoh M, Tanaka S, Ceribelli A, Calise SJ, Chan EK. A comprehensive overview on myositis-specific antibodies: new and old biomarkers in idiopathic inflammatory myopathy. Clin Rev Allergy Immunol. 2017;52(1):1-19. doi:10.1007/s12016-015-8510-y
34. Hamaguchi Y, Kuwana M, Hoshino K, et al. Clinical correlations with dermatomyositis-specific autoantibodies in adult Japanese patients with dermatomyositis: a multicenter cross-sectional study. Arch Dermatol. 2011;147(4):391-398. doi:10.1001/archdermatol.2011.52
35. Monseau G, Landon-Cardinal O, Stenzel W, et al. Systematic retrospective study of 64 patients with anti-Mi2 dermatomyositis: a classic skin rash with a necrotizing myositis and high risk of malignancy. J Am Acad Dermatol. 2020;83(6):1759-1763. doi:10.1016/j.jaad.2020.03.058
36. Truzzi NCC, Hoff LS, Borges IBP, de Souza FHC, Shinjo SK. Clinical manifestations, outcomes, and antibody profile of Brazilian adult patients with dermatomyositis: a single-center longitudinal study. Adv Rheumatol. 2022;62(1):41. doi:10.1186/s42358-022-00276-x
37. Gupta L, Naveen R, Gaur P, Agarwal V, Aggarwal R. Myositis-specific and myositis-associated autoantibodies in a large Indian cohort of inflammatory myositis. Semin Arthritis Rheum. 2021;51(1):113-120. doi:10.1016/j.semarthrit.2020.10.014
38. Dos Passos Carvalho MIC, Shinjo SK. Frequency and clinical relevance of anti-Mi-2 autoantibody in adult Brazilian patients with dermatomyositis. Adv Rheumatol. 2019;59(1):27. doi:10.1186/s42358-019-0071-y
39. Reich A, Lis-Święty A, Krasowska D, et al. Dermatomyositis. Diagnostic and therapeutic recommendations of the Polish Dermatological Society. Dermatology Review/Przegląd Dermatologiczny. 2021;108(2):85-104. doi:10.5114/dr.2021.107278
40. Sodsri T, Petnak T, Suwatanapongched T, et al. Clinical characteristics and outcomes of anti-MDA5 dermatomyositis: a retrospective study. BMC Rheumatol. 2025;9(1):102. doi:10.1186/s41927-025-00556-1
41. Koga T, Fujikawa K, Horai Y, et al. The diagnostic utility of anti-melanoma differentiation-associated gene 5 antibody testing for predicting the prognosis of Japanese patients with DM. Rheumatology (Oxford). 2012;51(7):1278-1284. doi:10.1093/rheumatology/ker518
42. Nombel A, Fabien N, Coutant F. Dermatomyositis with anti-MDA5 antibodies: bioclinical features, pathogenesis and emerging therapies. Front Immunol. 2021;12:773352. doi:10.3389/fimmu.2021.773352
43. Qiang JK, Kim WB, Baibergenova A, Alhusayen R. Risk of Malignancy in Dermatomyositis and Polymyositis. J Cutan Med Surg. 2017;21(2):131-136. doi:10.1177/1203475416665601
44. Chawrylak K, Kubas M, Krzemińska K, Kuszneruk J, Kłusek M. Impact of Exercise on the Course of Dermatomyositis: A Narrative Review of Recent Studies. Qual Sport [Internet]. 2024 Nov. 12 [cited 2026 May 29];31:55447. Available from: https://apcz.umk.pl/QS/article/view/55447
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