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Open Access 05-03-2024 | Polymyositis | Original Communication

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

Authors: Lea-Katharina Zierer, Steffen Naegel, Ilka Schneider, Thomas Kendzierski, Kathleen Kleeberg, Anna Katharina Koelsch, Leila Scholle, Christoph Schaefer, Arne Naegel, Stephan Zierz, Markus Otto, Gisela Stoltenburg-Didinger, Torsten Kraya, Dietrich Stoevesandt, Alexander Mensch

Published in: Journal of Neurology | Issue 6/2024

Abstract

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.

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Title: Quantitative whole-body muscle MRI in idiopathic inflammatory myopathies including polymyositis with mitochondrial pathology: indications for a disease spectrum
Authors: Lea-Katharina Zierer
Steffen Naegel
Ilka Schneider
Thomas Kendzierski
Kathleen Kleeberg
Anna Katharina Koelsch
Leila Scholle
Christoph Schaefer
Arne Naegel
Stephan Zierz
Markus Otto
Gisela Stoltenburg-Didinger
Torsten Kraya
Dietrich Stoevesandt
Alexander Mensch
Publication date: 05-03-2024
Publisher: Springer Berlin Heidelberg
Keywords: Polymyositis
Polymyositis
Edema
Myopathy
Biomarkers
Pathology
Published in: Journal of Neurology / Issue 6/2024
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-024-12191-w

Keynote webinar | Spotlight on medication adherence

Springer Medicine

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

Abstract

Objective

Methods

Results

Interpretation

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Methods

Results

Interpretation

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