Can we differentiate patients with dysferlinopathies and inflammatory myopathies by ultrasound? A discriminant analysis study.
Diagnostic accuracy
Dysferlinopathies
Idiopathic inflammatory myopathies
Ultrasound
Journal
Rheumatology international
ISSN: 1437-160X
Titre abrégé: Rheumatol Int
Pays: Germany
ID NLM: 8206885
Informations de publication
Date de publication:
18 Sep 2024
18 Sep 2024
Historique:
received:
04
07
2024
accepted:
03
09
2024
medline:
18
9
2024
pubmed:
18
9
2024
entrez:
18
9
2024
Statut:
aheadofprint
Résumé
Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of diseases that are characterized by inflammation and muscle weakness. Dysferlinopathies are autosomal recessive limb-girdle muscular dystrophies caused by mutations in DYSF, which share a similar clinical presentation and histopathological inflammatory changes. To determine the sonographic differences between dysferlinopathies and IIM and whether these differences allow their classification. This observational, cross-sectional, and analytical study evaluated 20 muscles from 11 patients with dysferlinopathies and 11 patients with IIM. The patients were matched for age, sex, and disease duration. Clinical and laboratory variables were analyzed. Semi-quantitative scales were used to weigh the gray scale and power Doppler muscle abnormalities. Descriptive statistics were computed and discriminant analysis was performed to determine the ultrasound variables that best predicted the final diagnosis. Forty muscles were evaluated. Atrophy and higher Heckmatt scale scores were observed in patients with dysferlinopathies. A set of three muscles (biceps/brachialis, quadriceps, and gastrocnemius) had a diagnostic accuracy of 100% (sensitivity, 100%; specificity, 100%; canonical coefficient, 0.733 p < 0.001). A set of two formulas was used to classify both diseases correctly. In the present study, scanning of three muscle groups showed high diagnostic accuracy in differentiating dysferlinopathies from MII. Ultrasound can be used as an initial test in patients with suspected muscle disease or as an additional tool to support diagnosis in controversial cases.
Identifiants
pubmed: 39292434
doi: 10.1007/s00296-024-05721-2
pii: 10.1007/s00296-024-05721-2
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.
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