Inflammatory myopathies

Clinicopathological profile of eosinophilic fasciitis: a retrospective cohort study from a neuromuscular disorder center in China

Arthritis Res TherPublished Retrospective cohort study

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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