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.

Muscle MRI in immune-mediated necrotizing myopathy (IMNM). implications for clinical management and treatment strategies / Fionda, Laura; Lauletta, Antonio; Leonardi, Luca; Perez, Jorge Alonso; Morino, Stefania; Merlonghi, Gioia; Alfieri, Girolamo; Costanzo, Rocco; Tufano, Laura; Vanoli, Fiammetta; Rossini, Elena; Vigo, Eduard Gallardo; Tartaglione, Tommaso; Salvetti, Marco; Antonini, Giovanni; Diaz-Manera, Jordi; Garibaldi, Matteo. - In: JOURNAL OF NEUROLOGY. - ISSN 0340-5354. - (2022), pp. 1-16. [10.1007/s00415-022-11447-7]

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

Fionda, Laura;Lauletta, Antonio;Leonardi, Luca;Morino, Stefania;Merlonghi, Gioia;Alfieri, Girolamo;Costanzo, Rocco;Tufano, Laura;Vanoli, Fiammetta;Rossini, Elena;Salvetti, Marco;Antonini, Giovanni;Garibaldi, Matteo
2022

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.
2022
follow-up study; immune mediated necrotizing myopathy (imnm); immunomodulating therapy; inflammatory myopathies; whole body muscle mri
01 Pubblicazione su rivista::01a Articolo in rivista
Muscle MRI in immune-mediated necrotizing myopathy (IMNM). implications for clinical management and treatment strategies / Fionda, Laura; Lauletta, Antonio; Leonardi, Luca; Perez, Jorge Alonso; Morino, Stefania; Merlonghi, Gioia; Alfieri, Girolamo; Costanzo, Rocco; Tufano, Laura; Vanoli, Fiammetta; Rossini, Elena; Vigo, Eduard Gallardo; Tartaglione, Tommaso; Salvetti, Marco; Antonini, Giovanni; Diaz-Manera, Jordi; Garibaldi, Matteo. - In: JOURNAL OF NEUROLOGY. - ISSN 0340-5354. - (2022), pp. 1-16. [10.1007/s00415-022-11447-7]
File allegati a questo prodotto
File Dimensione Formato  
Fionda_Muscle-MRI-in-immune-mediated-necrotizing-myopathy_2022.pdf

accesso aperto

Tipologia: Versione editoriale (versione pubblicata con il layout dell'editore)
Licenza: Creative commons
Dimensione 2.17 MB
Formato Adobe PDF
2.17 MB Adobe PDF

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1659088
Citazioni
  • ???jsp.display-item.citation.pmc??? 2
  • Scopus 2
  • ???jsp.display-item.citation.isi??? 2
social impact