Muscle MRI in immune-mediated necrotizing myopathy (IMNM): implications for clinical management and treatment strategies

Laura Fionda, Antonio Lauletta, Luca Leonardi, Jorge Alonso Perez, Stefania Morino, Gioia Merlonghi, Girolamo Alfieri, Rocco Costanzo, Laura Tufano, Fiammetta Vanoli, Elena Rossini, Eduard Gallardo Vigo, Tommaso Tartaglione, Marco Salvetti, Giovanni Antonini, Jordi Diaz-Manera, Matteo Garibaldi

Risultato della ricerca: Contributo in rivistaArticolo in rivista

Abstract

Objectives: 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. Methods: 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. Results: 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). Interpretation: 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.
Lingua originaleEnglish
pagine (da-a)960-974
Numero di pagine15
RivistaJournal of Neurology
Volume270
DOI
Stato di pubblicazionePubblicato - 2023

Keywords

  • Follow-up study
  • Immune mediated necrotizing myopathy (IMNM)
  • Whole body muscle MRI
  • Inflammatory myopathies
  • Immunomodulating therapy

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