PMID- 38438820 OWN - NLM STAT- Publisher LR - 20240304 IS - 1432-1459 (Electronic) IS - 0340-5354 (Linking) DP - 2024 Mar 5 TI - Quantitative whole-body muscle MRI in idiopathic inflammatory myopathies including polymyositis with mitochondrial pathology: indications for a disease spectrum. LID - 10.1007/s00415-024-12191-w [doi] AB - 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. CI - (c) 2024. The Author(s). FAU - Zierer, Lea-Katharina AU - Zierer LK AD - Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. AD - Department of Radiology, University Medicine Halle, Halle (Saale), Germany. FAU - Naegel, Steffen AU - Naegel S AD - Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. AD - Department of Neurology, Alfried-Krupp-Krankenhaus Essen, Essen, Germany. FAU - Schneider, Ilka AU - Schneider I AD - Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. AD - Department of Neurology, St. Georg Hospital Leipzig, Leipzig, Germany. FAU - Kendzierski, Thomas AU - Kendzierski T AD - Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. FAU - Kleeberg, Kathleen AU - Kleeberg K AD - Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. FAU - Koelsch, Anna Katharina AU - Koelsch AK AD - Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. FAU - Scholle, Leila AU - Scholle L AD - Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. FAU - Schaefer, Christoph AU - Schaefer C AD - Department of Internal Medicine II, Rheumatology, University Medicine Halle, Halle (Saale), Germany. FAU - Naegel, Arne AU - Naegel A AD - Goethe Center for Scientific Computing (G-CSC), Goethe University, Frankfurt/Main, Germany. FAU - Zierz, Stephan AU - Zierz S AD - Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. FAU - Otto, Markus AU - Otto M AD - Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. FAU - Stoltenburg-Didinger, Gisela AU - Stoltenburg-Didinger G AD - Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. AD - Institute of Cell and Neurobiology, Charite University Medicine Berlin, Berlin, Germany. FAU - Kraya, Torsten AU - Kraya T AD - Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. AD - Department of Neurology, St. Georg Hospital Leipzig, Leipzig, Germany. FAU - Stoevesandt, Dietrich AU - Stoevesandt D AD - Department of Radiology, University Medicine Halle, Halle (Saale), Germany. FAU - Mensch, Alexander AU - Mensch A AUID- ORCID: 0000-0003-0089-4473 AD - Department of Neurology, University Medicine Halle, Ernst-Grube-Str. 40, 06120, Halle (Saale), Germany. alexander.mensch@medizin.uni-halle.de. LA - eng PT - Journal Article DEP - 20240305 PL - Germany TA - J Neurol JT - Journal of neurology JID - 0423161 SB - IM OTO - NOTNLM OT - IBM OT - Imaging biomarker OT - Inclusion body myositis OT - Myositis OT - PM-Mito OT - qMRI EDAT- 2024/03/05 00:45 MHDA- 2024/03/05 00:45 CRDT- 2024/03/04 23:40 PHST- 2023/09/03 00:00 [received] PHST- 2024/01/04 00:00 [accepted] PHST- 2024/01/01 00:00 [revised] PHST- 2024/03/05 00:45 [medline] PHST- 2024/03/05 00:45 [pubmed] PHST- 2024/03/04 23:40 [entrez] AID - 10.1007/s00415-024-12191-w [pii] AID - 10.1007/s00415-024-12191-w [doi] PST - aheadofprint SO - J Neurol. 2024 Mar 5. doi: 10.1007/s00415-024-12191-w.