Spatial transcriptomics in immune-mediated necrotising myopathy uncovers a specific necrotic signature related to immune-driven fibrosis
Authors' conclusion
In conclusion, IMNM necrotic muscle areas are linked to a specific SFRP4-expressing fibroblast subtype interacting with macrophages, potentially driving the pathological muscle phenotype and contributing to inflammation, fibro-fatty replacement, and strength loss.
Study context
- Population
- Patients with immune-mediated necrotising myopathies (IMNM) and other myositis; muscle biopsies
- Outcome
- Signatures in IMNM muscle
Main finding
As reported in the abstract
- Inflammatory, fibrotic, regenerative, and lipid metabolism-related signatures were identified in IMNM, distinct from other myositis, with specific distribution in necrotic and peri-necrotic regions
Further findings
SFRP4-expressing immune-driven fibroblasts in necrotic areas
- A unique subtype of immune-driven fibroblasts expressing SFRP4 was enriched in IMNM necrotic areas
- These cells carried a strong proportion of IMNM-enriched pathological signatures, notably a complement-linked 'tumour-like' inflammatory fibroblast signature and a specific interferon response, characterised by the expression of metallothioneins
Peri-necrotic areas
- Peri-necrotic areas were characterised by pathological muscle signatures carrying interferon response and FABP4+ metabolically dysfunctional signatures
Original publication
Spatial transcriptomics in immune-mediated necrotising myopathy uncovers a specific necrotic signature related to immune-driven fibrosis. Ann Rheum Dis. Published 30 October 2026. doi:10.
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