Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2025 Apr 21:2025:5560523.
doi: 10.1155/crrh/5560523. eCollection 2025.

Anifrolumab in Refractory Dermatomyositis and Antisynthetase Syndrome

Ryan Bonaventure Soares  1 , Jihad Ben Gabr  2 , Mark Ash  3 , Gregory Hosler  4
Affiliations
Case Reports

Anifrolumab in Refractory Dermatomyositis and Antisynthetase Syndrome

Ryan Bonaventure Soares et al. Case Rep Rheumatol. .

Abstract

Dermatomyositis (DM) and antisynthetase syndrome are rare autoimmune disorders within the spectrum of inflammatory myopathies, typically characterized by cutaneous and muscular inflammation along with systemic manifestations. This case report highlights the evolving treatment strategies for inflammatory myopathies, with a focus on the use of anifrolumab, a type-1 interferon receptor blocker, in a 29 year-old female with refractory DM/antisynthetase syndrome. The patient presented with classic DM features, including heliotrope rash, Gottron's papules, and malar erythema, but lacked significant myopathy. Initial therapies with methotrexate and prednisone were ineffective, and her condition worsened despite adding intravenous immunoglobulin (IVIg) and tofacitinib. Following persistent disease progression, therapy was switched to a combination of IVIg, apremilast, and anifrolumab as an off-label drug for DM. Within 5 months, the patient showed significant improvement in myopathy and skin manifestations. Anifrolumab targets the interferon (IFN) axis, which plays a crucial role in DM pathogenesis. This case underscores the potential of targeted therapies like anifrolumab in managing DM, especially in cases not responsive to conventional standard of care therapies. It also highlights the need for further research into the IFN pathway as a therapeutic target in inflammatory myopathies. Trial Registration: ClinicalTrials.gov identifier: NCT06455449.

Keywords: anifrolumab; antisynthetase syndrome; dermatomyositis; inflammatory myopathy; interferon receptor.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Left lower back demonstrating dermatomyositis associated rash and erythema.
Figure 2
Figure 2
Skin biopsy showing interface dermatitis with basement membrane thickening and sparse lymphocytic infiltrate.
Figure 3
Figure 3
Preanifrolumab—dorsal aspect on the hands showing the classical dermatomyositis rash.
Figure 4
Figure 4
Postanifrolumab—appropriate response and improvement in the dermatological signs after anifrolumab therapy.
Figure 5
Figure 5
Preanifrolumab—lateral right hip demonstrating dermatomyositis associated skin rash and erythema.
Figure 6
Figure 6
Postanifrolumab—lateral right hip demonstrating clinical response to anifrolumab with resolution of the rash.
See this image and copyright information in PMC

References

    1. Lundberg I. E., Fujimoto M., Vencovsky J., et al. Idiopathic Inflammatory Myopathies. Nature Reviews Disease Primers . 2021;7(1):p. 86. https://pubmed.ncbi.nlm.nih.gov/34857798/ - PubMed
    1. Carolina Londe A., Fernandez-Ruiz R., Rogério Julio P., et al. Type I Interferons in Autoimmunity: Implications in Clinical Phenotypes and Treatment Response. Journal of Rheumatology . 2023;50(9):1103–1113. https://www.jrheum.org/content/50/9/1103 . - PubMed
    1. Cojocaru M., Cojocaru I. M., Chicos B. New Insights into Antisynthetase Syndrome. Maedica (Bucur) . 2016;11(2):p. 130. - PMC - PubMed
    1. Hum R. M., Lilleker J. B., Lamb J. A., et al. Comparison of Clinical Features Between Patients With Anti-Synthetase Syndrome and Dermatomyositis: Results From the MYONET Registry. Rheumatology . 2024;63(8):2093–2100. https://pubmed.ncbi.nlm.nih.gov/37698987/ - PMC - PubMed
    1. Aggarwal R., Charles-Schoeman C., Schessl J., et al. Trial of Intravenous Immune Globulin in Dermatomyositis. New England Journal of Medicine . 2022;387(14):1264–1278. https://www.nejm.org/doi/full/10.1056/NEJMoa2117912 . - DOI - PubMed

Publication types

Associated data

LinkOut - more resources