{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T02:09:51Z","timestamp":1791684591888,"version":"4.3.4"},"reference-count":41,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2024,4,13]],"date-time":"2024-04-13T00:00:00Z","timestamp":1712966400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J cachexia sarcopenia muscle"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Finding sensitive clinical outcome measures has become crucial in natural history studies and therapeutic trials of neuromuscular disorders. Here, we focus on 1\u2010year longitudinal data from quantitative magnetic resonance imaging (MRI) and phosphorus magnetic resonance spectroscopy (<jats:sup>31<\/jats:sup>P MRS) in a placebo\u2010controlled study of sirolimus for inclusion body myositis (IBM), also examining their links to functional, strength, and clinical parameters in lower limb muscles.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Quantitative MRI and <jats:sup>31<\/jats:sup>P MRS data were collected at 3\u00a0T from a single site, involving 44 patients (22 on placebo, 22 on sirolimus) at baseline and year\u20101, and 21 healthy controls. Assessments included fat fraction (FF), contractile cross\u2010sectional area (cCSA), and water T<jats:sub>2<\/jats:sub> in global leg and thigh segments, muscle groups, individual muscles, as well as <jats:sup>31<\/jats:sup>P MRS indices in <jats:italic>quadriceps<\/jats:italic> or <jats:italic>triceps surae<\/jats:italic>. Analyses covered patient\u2010control comparisons, annual change assessments via standard <jats:italic>t<\/jats:italic>\u2010tests and linear mixed models, calculation of standardized response means (SRM), and exploration of correlations between MRI, <jats:sup>31<\/jats:sup>P MRS, functional, strength, and clinical parameters.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The <jats:italic>quadriceps<\/jats:italic> and <jats:italic>gastrocnemius medialis<\/jats:italic> muscles had the highest FF values, displaying notable heterogeneity and asymmetry, particularly in the <jats:italic>quadriceps<\/jats:italic>. In the placebo group, the median 1\u2010year FF increase in the quadriceps was 3.2% (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001), whereas in the sirolimus group, it was 0.7% (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.033). Both groups experienced a significant decrease in cCSA in the <jats:italic>quadriceps<\/jats:italic> after 1\u00a0year (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001), with median changes of 12.6% for the placebo group and 5.5% for the sirolimus group. Differences in FF and cCSA changes between the two groups were significant (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001). SRM values for FF and cCSA were 1.3 and 1.4 in the placebo group and 0.5 and 0.8 in the sirolimus group, respectively. Water T<jats:sub>2<\/jats:sub> values were highest in the <jats:italic>quadriceps<\/jats:italic> muscles of both groups, significantly exceeding control values in both groups (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001) and were higher in the placebo group than in the sirolimus group. After treatment, water T<jats:sub>2<\/jats:sub> increased significantly only in the sirolimus group's <jats:italic>quadriceps<\/jats:italic> (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.01). Multiple <jats:sup>31<\/jats:sup>P MRS indices were abnormal in patients compared to controls and remained unchanged after treatment. Significant correlations were identified between baseline water T<jats:sub>2<\/jats:sub> and FF at baseline and the change in FF (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001). Additionally, significant correlations were observed between FF, cCSA, water T<jats:sub>2<\/jats:sub>, and functional and strength outcome measures.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This study has demonstrated that quantitative MRI\/<jats:sup>31<\/jats:sup>P MRS can discern measurable differences between placebo and sirolimus\u2010treated IBM patients, offering promise for future therapeutic trials in idiopathic inflammatory myopathies such as IBM.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jcsm.13451","type":"journal-article","created":{"date-parts":[[2024,4,13]],"date-time":"2024-04-13T08:04:35Z","timestamp":1712995475000},"page":"1108-1120","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Effect of sirolimus on muscle in inclusion body myositis observed with magnetic resonance imaging and spectroscopy"],"prefix":"10.1002","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0777-2711","authenticated-orcid":false,"given":"Harmen","family":"Reyngoudt","sequence":"first","affiliation":[{"name":"NMR Laboratory, Neuromuscular Investigation Center Institute of Myology  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre\u2010Yves","family":"Baudin","sequence":"additional","affiliation":[{"name":"NMR Laboratory, Neuromuscular Investigation Center Institute of Myology  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ericky","family":"Caldas de Almeida Ara\u00fajo","sequence":"additional","affiliation":[{"name":"NMR Laboratory, Neuromuscular Investigation Center Institute of Myology  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Damien","family":"Bachasson","sequence":"additional","affiliation":[{"name":"Neuromuscular Physiology and Evaluation Laboratory, Neuromuscular Investigation Center Institute of Myology  Paris France"},{"name":"INSERM, UMRS1158 Neurophysiologie Respiratoire Exp\u00e9rimentale et Clinique Sorbonne Universit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean\u2010Marc","family":"Boisserie","sequence":"additional","affiliation":[{"name":"NMR Laboratory, Neuromuscular Investigation Center Institute of Myology  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kub\u00e9raka","family":"Mariampillai","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Clinical Immunology, Inflammatory Myopathies Reference Center, Research Center in Myology UMR974, Sorbonne Universit\u00e9, Assistance Publique\u2010H\u00f4pitaux de Paris Piti\u00e9\u2010Salp\u00eatri\u00e8re University Hospital  Paris France"},{"name":"I\u2010Motion Institute of Myology  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00e9lanie","family":"Annoussamy","sequence":"additional","affiliation":[{"name":"I\u2010Motion Institute of Myology  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yves","family":"Allenbach","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Clinical Immunology, Inflammatory Myopathies Reference Center, Research Center in Myology UMR974, Sorbonne Universit\u00e9, Assistance Publique\u2010H\u00f4pitaux de Paris Piti\u00e9\u2010Salp\u00eatri\u00e8re University Hospital  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean\u2010Yves","family":"Hogrel","sequence":"additional","affiliation":[{"name":"Neuromuscular Physiology and Evaluation Laboratory, Neuromuscular Investigation Center Institute of Myology  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre 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