[Malignancy in idiopathic inflammatory myopathies : Risk assessment and screening strategies]
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Published on: October 31, 2025
Kastriot Kastrati1,2, Karolina Gente3, Rebecca Hasseli-Fräbel4,5
1Klinische Abteilung für Rheumatologie, Universitätsklinik für Innere Medizin III, Währinger Gürtel 18-20, 1090, Wien, Österreich.
Background:
Idiopathic inflammatory myopathies (IIM, "myositis") are rare autoimmune diseases of the skeletal musculature, which are associated with an increased risk of cancer, particularly in adults. Malignant tumors occur especially frequently in dermatomyositis (DM), emphasizing the need for systematic cancer screening.
Objective:
This review provides a practice-oriented summary of the association between IIM and malignant diseases and addresses the recommendations of the International Myositis Assessment and Clinical Studies Group (IMACS) for cancer risk stratification and screening.
Material And Methods:
A selective literature search was carried out including current studies, register data and consensus guidelines. The focus was on the IMACS recommendations on risk stratification and screening published in 2023.
Results:
Approximately 20-25% of all adult patients with IIM develop a malignant disease within 3 years after disease onset. Patients with DM are at the highest risk, showing an approximately twofold increased risk of cancer compared to other IIM subtypes. Common malignancies include lung, ovarian, colorectal, breast, thyroid and nasopharyngeal carcinomas as well as lymphomas. Risk factors for paraneoplastic myositis include the presence of a DM subtype, but also specific autoantibodies such as anti-transcription intermediary factor 1‑gamma (TIF1-γ) and anti-nuclear matrix protein 2 (NXP2) antibodies, and clinical features such as age > 40 years at disease onset, persistently high and treatment-refractory disease activity, dysphagia and cutaneous necrosis. Based on these factors, the individual cancer risk can be stratified and screening and follow-up examinations are recommended in accordance with the IMACS guidelines.
Discussion:
The systematic early detection of malignant comorbidities in IIM is essential, as malignancies represent the leading cause of death in these patients. The recently published IMACS recommendations enable a standardized, risk-adapted approach to cancer screening and are intended to facilitate early diagnosis and treatment.
