Development of differential diagnostic models for distinguishing between limb-girdle muscular dystrophy and idiopathic inflammatory myopathy
Authors' conclusion
We developed two practical differential diagnosis models for LGMD and IIM based on the analysis of four accessible indicators, including the age of onset, cervical flexor weakness, the ratio of synchronous Mb/CK values and diffuse MHC-I expression.
Study context
- Population
- Patients with idiopathic inflammatory myopathy (IIM) or limb-girdle muscular dystrophy (LGMD) diagnosed at a neuromuscular center
- Participants
- 71 / 24 / 22
- Comparison
- IIM vs LGMD
- IIMn = 71
- LGMDR2n = 24
- LGMDR1n = 22
- Outcome
- Differential diagnosis of LGMD vs IIM
Methodological detailssingle-centre, nomogram and decision tree, five-fold cross-validation and bootstrapping
- Single-centre
- Nomogram and decision tree
- Five-fold cross-validation and bootstrapping
Performance
As reported in the abstract
Differential diagnostic models
ROC analysis: high differential diagnostic efficiency of synchronous myoglobin (Mb)/creatine kinase (CK), cutoff
0.18
Further findings
Clinical and pathological differences, IIM vs LGMD
- IIM: significantly older age of onset, higher prevalence of cervical flexor weakness, more commonly diffuse MHC-I expression
- Synchronous Mb/CK ratio before immunotherapy significantly higher in IIM than LGMD
Original publication
Development of differential diagnostic models for distinguishing between limb-girdle muscular dystrophy and idiopathic inflammatory myopathy. Arthritis Res Ther. Published 19 December 2024. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →