Factors associated with endomysial fibrosis in anti-signal recognition particle antibody immune-mediated necrotising myopathy
Authors' conclusion
In anti-SRP-IMNM, endomysial fibrosis may be the pathological basis of fatty infiltration observed on MRI and may be associated with resistance to immunotherapy.
Study context
- Population
- Patients diagnosed with anti-SRP-IMNM (paediatric and adult)12 paediatric, 82 adult
- Participants
- 94 patients
- Exposure
- Patients with vs without increased endomysial fibrosis on muscle biopsy
- Outcome
- Relationship between endomysial fibrosis and clinical, muscle MRI and myo-pathological features
Main finding
As reported in the abstract
Factors associated with endomysial fibrosis
- Endomysial fibrosis prevalence
- 50%
- Association with early onset
- p=0.004
- Association with paediatric age
- p=0.044
- Association with muscle fatty infiltration on thigh magnetic resonance imaging (MRI)
- p=0.045
- Association with inflammatory cell infiltration on pathology
- p<0.05
Observational data: associations, not effects.
Further findings
Baseline characteristics and MRI findings
- Mean age at onset (years)
- 41.2±17.3
- Mean serum creatinine kinase (IU/L)
- 6885.8±6300.7
- Muscle oedema on MRI
- 87.3%
- Fatty infiltration on MRI, particularly severe in posterior thigh
- 77.5%
Original publication
Factors associated with endomysial fibrosis in anti-signal recognition particle antibody immune-mediated necrotising myopathy. Clin Exp Rheumatol. Published 6 July 2025. doi:10.
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