International Myositis Assessment and Clinical Studies Guidelines for Risk-Based Cancer Screening: An External Validation in Patients with Dermatomyositis Seen at a Metropolitan Academic Center

Authors

• Isabel Silva, Rutgers New Jersey Medical School
• Jasper Dayanan, Icahn School of Medicine at Mount Sinai
• Alexandra Nigro, Macon & Joan Brock Virginia Health Sciences at Old Dominion UniversityFollow
• Capriana Jiang, Icahn School of Medicine at Mount Sinai
• Ma Georgina Erfe, Icahn School of Medicine at Mount Sinai
• Trixie Rivera, Icahn School of Medicine at Mount Sinai
• Saakshi Khattri, Icahn School of Medicine at Mount Sinai

ORCID

0009-0006-1247-407X (Nigro)

Document Type

Article

Publication Date

2026

DOI

10.1002/acr2.90101

Publication Title

ACR Open Rheumatology

Volume

8

Issue

7

Pages

e90101

Abstract

Objective

Dermatomyositis (DM) is an idiopathic inflammatory myopathy (IIM) characterized by muscle weakness, skin findings, and increased malignancy risk. Cancer risk is greatest in the three years before or after DM onset, termed paraneoplastic DM. The International Myositis Assessment and Clinical Studies Group (IMACS) set forth evidence-based guidelines for risk stratification and cancer screenings for newly diagnosed patients with IIM. '

Methods

We retrospectively reviewed the records of 413 patients with a known diagnosis of DM or clinically amyopathic DM at the Mount Sinai Health System (2019-2024). Demographics, clinical findings, laboratory results, myositis autoantibody panels, and cancer screening or diagnosis data were collected.

Results

Among the 413 patients with DM, 6.5% (n = 27) had paraneoplastic DM. The proportion of paraneoplastic disease was highest in the high-risk group (8.8%), compared to intermediate- (5.1%) and low-risk (2.5%) groups. 70.4% of patients with paraneoplastic DM were categorized as high risk, compared to 51% of nonparaneoplastic patients. Among the intermediate-risk patients, most patients were diagnosed with cancer more than one year after DM diagnosis. Age ≥40 at DM diagnosis was statistically significant for an increased risk of paraneoplastic DM (P = 0.001). The most common paraneoplastic malignancies were breast cancer and lung cancer. One-third of patients with paraneoplastic DM were diagnosed with cancer within three years preceding DM diagnosis.

Conclusion

The IMACS screening guidelines and risk stratification showed moderate sensitivity for identifying paraneoplastic DM, helping to identify a majority of patients at the highest risk for malignancy leading to more targeted and risk-based screening.

Rights

© 2026 The Authors.

This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

Original Publication Citation

Silva, I., Dayanan, J., Nigro, A., Jiang, C., Erfe, M. G., Rivera, T., & Khattri, S. (2026). International myositis assessment and clinical studies guidelines for risk-based cancer screening: An external validation in patients with dermatomyositis seen at a metropolitan academic center. ACR Open Rheumatology, 8(7), Article e90101. https://doi.org/10.1002/acr2.90101

Repository Citation

Silva, I., Dayanan, J., Nigro, A., Jiang, C., Erfe, M. G., Rivera, T., & Khattri, S. (2026). International myositis assessment and clinical studies guidelines for risk-based cancer screening: An external validation in patients with dermatomyositis seen at a metropolitan academic center. ACR Open Rheumatology, 8(7), Article e90101. https://doi.org/10.1002/acr2.90101