Assessment of IBM-FRS total score and specific functional domains in a large cohort of inclusion body myositis patients

Inclusion body myositis (IBM) is an acquired myopathy of older adults, associated with progressive weakness and functional limitations. The inclusion body myositis functional rating score (IBM-FRS) is a reliable tool for the assessment of disease severity, but poor data are available on its correlat...

Celý popis

Uloženo v:
Podrobná bibliografie
Vydáno v:Journal of neurology Ročník 272; číslo 12; s. 775
Hlavní autoři: Vicino, Alex, Lauletta, Antonio, Bonanno, Silvia, Barbaccia, Adele, Valentino, Maria Lucia, Risi, Barbara, Torchia, Eleonora, Cheli, Marta, Tonin, Paola, Urbano, Guido, Saccani, Elena, Faedo, Elena, Ruggieri, Alessandra, Iannibelli, Eliana, Croce, Michele Giovanni, Zoppi, Dario, Costantini, Emanuele Maria, Rigamonti, Andrea, Colacicco, Giovanni, Grandis, Marina, Coccia, Michela, Barp, Andrea, Stephan, Aurélie Marroun, Carda, Stefano, Théaudin, Marie, Ravaglia, Sabrina, Ruggiero, Lucia, Mongini, Tiziana, Verriello, Lorenzo, Vattemi, Gaetano, Bortolani, Sara, Sansone, Valeria Ada, Filosto, Massimiliano, Previtali, Stefano Carlo, Liguori, Rocco, Rodolico, Carmelo, Garibaldi, Matteo, Maggi, Lorenzo
Médium: Journal Article
Jazyk:angličtina
Vydáno: Berlin/Heidelberg Springer Berlin Heidelberg 01.12.2025
Springer Nature B.V
Témata:
Activities of Daily Living
Age
Aged
Aged, 80 and over
Biopsy
Clinical trials
Cohort Studies
Cross-Sectional Studies
Deglutition Disorders - etiology
Deglutition Disorders - physiopathology
Dysphagia
Female
Health risk assessment
Humans
Magnetic resonance imaging
Male
Medicine
Medicine & Public Health
Middle Aged
Missing data
Myopathy
Myositis
Myositis, Inclusion Body - complications
Myositis, Inclusion Body - diagnosis
Myositis, Inclusion Body - physiopathology
Neurology
Neuroradiology
Neurosciences
Original Communication
Ostomy
Patients
Phenotypes
Retrospective Studies
Severity of Illness Index
Yeast
United States > US
Inclusion body myositis
Myositis
IBM
IBM-FRS
ISSN:0340-5354, 1432-1459, 1432-1459
On-line přístup:Získat plný text
Tagy: Přidat tag
Žádné tagy, Buďte první, kdo vytvoří štítek k tomuto záznamu!
Popis
Shrnutí:Inclusion body myositis (IBM) is an acquired myopathy of older adults, associated with progressive weakness and functional limitations. The inclusion body myositis functional rating score (IBM-FRS) is a reliable tool for the assessment of disease severity, but poor data are available on its correlation with clinical features. In this cross-sectional, retrospective multicenter study, we analyzed IBM-FRS total score (TS) and its items in a cohort of 147 IBM patients, considering 4 functional domains (FD) (dysphagia, upper limb function, daily living and independence, lower limb function). We analyzed TS and FD values’ correlations with demographic and clinical variables (onset, loss of walking ability, non-invasive ventilation, percutaneous gastrostomy—PEG) as well as between each FD and the remaining TS. Lower, hence worse, scores in the dysphagia domain (FD1) correlated with female gender ( p  < 0.01), dysphagia as presenting symptom ( p  < 0.01), and PEG use ( p  < 0.0001), but interestingly without correlation with time variables IBM-FRS total score or other domains. This may define a distinct phenotype with different onset and outcome. Worse scores in the upper and lower limb and daily living domains (FD2–FD4) were strongly associated with loss of ambulation, higher age at baseline, and longer disease duration ( p  < 0.001). We identified a cutoff value of 28/40, allowing to differentiate patients with less disease burden, potentially able to show better response in future clinical trials. Our study enriches the literature by correlating IBM-FRS total scores and its items with patient characteristics, offering important implications for clinical management, subgroup analysis, and future trials design.
Bibliografie:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 14
content type line 23
ISSN:0340-5354
1432-1459
1432-1459
DOI:10.1007/s00415-025-13516-z