Quantitative whole-body muscle MRI in idiopathic inflammatory myopathies including polymyositis with mitochondrial pathology: indications for a disease spectrum.

Autor: Zierer LK; Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany.; Department of Radiology, University Medicine Halle, Halle (Saale), Germany., Naegel S; Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany.; Department of Neurology, Alfried-Krupp-Krankenhaus Essen, Essen, Germany., Schneider I; Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany.; Department of Neurology, St. Georg Hospital Leipzig, Leipzig, Germany., Kendzierski T; Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany., Kleeberg K; Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany., Koelsch AK; Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany., Scholle L; Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany., Schaefer C; Department of Internal Medicine II, Rheumatology, University Medicine Halle, Halle (Saale), Germany., Naegel A; Goethe Center for Scientific Computing (G-CSC), Goethe University, Frankfurt/Main, Germany., Zierz S; Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany., Otto M; Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany., Stoltenburg-Didinger G; Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany.; Institute of Cell and Neurobiology, Charité University Medicine Berlin, Berlin, Germany., Kraya T; Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany.; Department of Neurology, St. Georg Hospital Leipzig, Leipzig, Germany., Stoevesandt D; Department of Radiology, University Medicine Halle, Halle (Saale), Germany., Mensch A; Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. alexander.mensch@medizin.uni-halle.de.
Jazyk: angličtina
Zdroj: Journal of neurology [J Neurol] 2024 Jun; Vol. 271 (6), pp. 3186-3202. Date of Electronic Publication: 2024 Mar 05.
DOI: 10.1007/s00415-024-12191-w
Abstrakt: Objective: Inflammatory myopathies (IIM) include dermatomyositis (DM), sporadic inclusion body myositis (sIBM), immune-mediated necrotizing myopathy (IMNM), and overlap myositis (OLM)/antisynthetase syndrome (ASyS). There is also a rare variant termed polymyositis with mitochondrial pathology (PM-Mito), which is considered a sIBM precursor. There is no information regarding muscle MRI for this rare entity. The aim of this study was to compare MRI findings in IIM, including PM-Mito.
Methods: This retrospective analysis included 41 patients (7 PM-Mito, 11 sIBM, 11 PM/ASyS/OLM, 12 IMNM) and 20 healthy controls. Pattern of muscle involvement was assessed by semiquantitative evaluation, while Dixon method was used to quantify muscular fat fraction.
Results: The sIBM typical pattern affecting the lower extremities was not found in the majority of PM-Mito-patients. Intramuscular edema in sIBM and PM-Mito was limited to the lower extremities, whereas IMNM and PM/ASyS/OLM showed additional edema in the trunk. Quantitative assessment showed increased fat content in sIBM, with an intramuscular proximo-distal gradient. Similar changes were also found in a few PM-Mito- and PM/ASyS/OLM patients. In sIBM and PM-Mito, mean fat fraction of several muscles correlated with clinical involvement.
Interpretation: As MRI findings in patients with PM-Mito relevantly differed from sIBM, the attribution of PM-Mito as sIBM precursor should be critically discussed. Some patients in PM/ASyS/OLM and PM-Mito group showed MR-morphologic features predominantly observed in sIBM, indicative of a spectrum from PM/ASyS/OLM toward sIBM. In some IIM subtypes, MRI may serve as a biomarker of disease severity.
(© 2024. The Author(s).)
Databáze: MEDLINE
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