Clinicopathological profile of eosinophilic fasciitis: a retrospective cohort study from a neuromuscular disorder center in China
Authors' conclusion
Perifascicular upregulation of MHC-I and/or MHC-II but negative expression of MxA, with or without PFA, represents a unique pathological phenotype of EF.
Study context
- Population
- Patients with eosinophilic fasciitis (EF) at a neuromuscular disorder centre in China, January 2011 to August 2023
- Participants
- 17 patients
- Outcome
- Clinical, serological, MRI and myo-fascial pathological features
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
- Male predominance
- 12/17, 70.6%
- Skin thickening, always distal to elbow and knee joints
- 100%
- Limited joint mobility
- 12/17, 70.6%
- "Prayer sign"
- 7 (41.2%)
- Eosinophilia (6 in blood, 3 in tissue)
- 7 (41.2%)
- Anti-Ha antibody
- 1
- Perifascicular MHC-I and/or MHC-II upregulation without MxA expression in muscle specimens
- 56.3% (9/16)
- Typical perifascicular atrophy
- 4
Observational data: associations, not effects.
Further findings
Complete recovery on long-term follow-up
- Complete recovery
- 5
- Prednisone monotherapy
- 4
- Prednisone plus D-penicillamine
- 1
Original publication
Clinicopathological profile of eosinophilic fasciitis: a retrospective cohort study from a neuromuscular disorder center in China. Arthritis Res Ther. Published 19 May 2025. doi:10. 1186/ s13075-025-03574-z
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