Amyopathic dermatomyositis with anti-MDA5 antibody and interstitial lung disease: A case report

Nguyen Thi Mai Huong, Cao Thi Trinh, Tran Thi Mui, Bui Van Dan, Nguyen Hoang Phuong

Main Article Content

Abstract

Anti-MDA5 dermatomyositis is a clinically heterogeneous entity, often presenting with non-specific cutaneous manifestations that can be easily mistaken for other dermatological conditions. Such diagnostic challenges frequently delay recognition and management, leading to missed opportunities for early intervention, particularly in cases complicated by rapidly progressive interstitial lung disease (RP-ILD) - a life-threatening complication. Clinicians should focus at patients with suggestive skin findings such as heliotrope rash, Gottron’s papules, V-sign, shawl sign, digital ulcers, or palmar papules, especially in those without overt muscle weakness but with unexplained interstitial lung abnormalities. Early screening for anti-MDA5 antibodies and prompt initiation of aggressive immunosuppressive therapy, including high-dose corticosteroids and additional immunosuppressants, are essential to improve clinical outcomes and survival.

Article Details

References

1. Lundberg IE, Tjärnlund A, Bottai M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis. 2017;76(12):1955-1964. doi:10.1136/annrheumdis-2017-211468
2. Gerami P, Schope JM, McDonald L,et al. A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis siné myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies. J Am Acad Dermatol. 2006;54(4):597-613. doi:10.1016/j.jaad.2005.10.041
3. Nombel A, Fabien N, Coutant F. Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies. Front Immunol. 2021;12. doi:10.3389/fimmu.2021.773352
4. Chen M, Zhao Q, Diao L, et al. Distribution of anti-melanoma differentiation associated gene 5 (MDA5) IgG subclasses in MDA5+ dermatomyositis. Rheumatology. 2022;61(1):430-439. doi:10.1093/rheumatology/keab268
5. Xu YT, Zhang YM, Yang HX, et al. Evaluation and validation of the prognostic value of anti-MDA5 IgG subclasses in dermatomyositis-associated interstitial lung disease. Rheumatology. 2023;62(1):397-406. doi:10.1093/rheumatology/keac229
6. L C, F M, A M, et al. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies. Clin Exp Rheumatol. 2022;40(2):274-283. doi:10.55563/clinexprheumatol/di1083
7. Ceribelli A, Fredi M, Taraborelli M, et al. Prevalence and clinical significance of anti-MDA5 antibodies in European patients with polymyositis/dermatomyositis. Clin Exp Rheumatol. 2014;32(6):891-897.
8. McPherson M, Economidou S, Liampas A, et al. Management of MDA-5 antibody positive clinically amyopathic dermatomyositis associated interstitial lung disease: A systematic review. Semin Arthritis Rheum. 2022;53:151959. doi:10.1016/j.semarthrit.2022.151959
9. Matsushita T, Mizumaki K, Kano M, et al. Antimelanoma differentiation-associated protein 5 antibody level is a novel tool for monitoring disease activity in rapidly progressive interstitial lung disease with dermatomyositis. Br J Dermatol. 2017;176(2):395-402. doi:10.1111/bjd.14882
10. Tsuji H, Nakashima R, Hosono Y, et al. Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy With High-Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti-Melanoma Differentiation-Associated Gene 5-Positive Dermatomyositis. Arthritis Rheumatol Hoboken NJ. 2020;72(3):488-498. doi:10.1002/art.41105