Muscle MRI in immune-mediated necrotizing myopathy (IMNM) : implications for clinical management and treatment strategies
Fionda, Laura (Università degli Studi di Roma La Sapienza)
Lauletta, Antonio (Università degli Studi di Roma La Sapienza)
Leonardi, Luca (Università degli Studi di Roma La Sapienza)
Perez, Jorge Alonso (Hospital de la Santa Creu i Sant Pau (Barcelona, Catalunya))
Morino, Stefania (Università degli Studi di Roma La Sapienza)
Merlonghi, Gioia (Università degli Studi di Roma La Sapienza)
Alfieri, Girolamo (Università degli Studi di Roma La Sapienza)
Costanzo, Rocco (Università degli Studi di Roma La Sapienza)
Tufano, Laura (Università degli Studi di Roma La Sapienza)
Vanoli, Fiammetta (IRCCS Istituto Neurologico Carlo Besta)
Rossini, Elena (Università degli Studi di Roma La Sapienza)
Vigo, Eduard Gallardo (Hospital de la Santa Creu i Sant Pau (Barcelona, Catalunya))
Tartaglione, Tommaso (Istituto Dermopatico dell'Immacolata (Itàlia))
Salvetti, Marco (Università degli Studi di Roma La Sapienza)
Antonini, Giovanni (Università degli Studi di Roma La Sapienza)
Diaz-Manera, Jordi (Centro de Investigación Biomédica en Red de Enfermedades Raras)
Garibaldi, Matteo (Università degli Studi di Roma La Sapienza)
Universitat Autònoma de Barcelona

Date:	2022
Abstract:	Immune-mediated necrotizing myopathy (IMNM) is the most severe idiopathic inflammatory myopathy (IIM) and early aggressive poly-immunotherapy is often required to reduce long-term disability. The aim of this study is to investigate muscle MRI in IMNM as outcome measure for disease activity, severity, progression, response to treatment, and to better characterize the pattern of muscle involvement. This is a retrospective, observational, cross-sectional, and longitudinal study including 22 IMNM patients, divided into three groups based on timing of first MRI and if performed before or under treatment. T1 score and percentage of STIR positive muscles (STIR%) were considered and analyzed also in relation to demographic, clinical and laboratory characteristics. STIR% was higher in untreated patients and in those who performed MRI earlier (p = 0. 001). Pelvic girdle and thighs were in general more affected than legs. T1 score was higher in patients with MRI performed later in disease course (p = 0. 004) with a prevalent involvement of the lumbar paraspinal muscles, gluteus medius and minimus, adductor magnus and hamstrings. 22% of STIR positive muscles showed fat replacement progression at second MRI. Higher STIR% at baseline correlated with higher risk of fat replacement at follow-up (p = 0. 003); higher T1 score correlated with clinical disability at follow-up, with late treatment start and delayed treatment with IVIG (p = 0. 03). Muscle MRI is a sensitive biomarker for monitoring disease activity and therapy response, especially when performed early in disease course and before treatment start, and could represent a supportive outcome measure and early prognostic index in IMNM.
Rights:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Language:	Anglès
Document:	Article ; recerca ; Versió publicada
Subject:	Immune mediated necrotizing myopathy (IMNM) ; Whole body muscle MRI ; Inflammatory myopathies ; Immunomodulating therapy ; Follow-up study
Published in:	Journal of Neurology, Vol. 270 (november 2022) , p. 960-974, ISSN 1432-1459

DOI: 10.1007/s00415-022-11447-7
PMID: 36329184

15 p, 2.2 MB

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