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Magnetic resonance imaging (MRI) provides useful biomarkers of muscle degeneration and disease activity. To investigate one-year longitudinal changes in lower leg muscles of patients with FSHD using qualitative (T1-weighted [T1w], STIR) and quantitative MRI (fat fraction [FF], water T2), and to assess their relationship with clinical function. Thirty-one patients with FSHD (21 men; age 44.2\u00b117 years) and 30 matched controls underwent 3T MRI. Twenty-seven patients completed one-year follow-up. Semi-quantitative grading and quantitative measures were analyzed muscle-wise. Longitudinal changes and correlations with clinical scores were assessed. At baseline, T1w imaging showed marked inter- and intra-individual variability, with heterogeneous proximal\u2013distal involvement in 22% of muscles and asymmetric involvement in 77% of patients. STIR hyperintensity was present in 25 patients. Over one-year, clinical decline was small (MRC: \u0394=\u20130.26, p=0.018, SRM=-0.23; mobility: \u0394=\u20130.41, p=0.031, SRM=-0.44). In contrast, quantitative MRI detected progression, with increases in FF (0.17\u00b10.13 to 0.22\u00b10.15, p&lt;0.001, SRM=0.87), FF heterogeneity, and water T2 heterogeneity, while T1w scores and mean water T2 showed limited change. Progression was greatest in muscles with intermediate baseline FF (\u0394FF\/year: 0.049 vs 0.007, p&lt;0.001). Baseline STIR and water T2 abnormalities predicted increases in FF but not T1w scores. FF correlated more strongly with clinical measures than T1w grading. Quantitative MRI is more sensitive than qualitative grading for detecting short-term disease progression in FSHD. Measures of heterogeneity and disease activity predict structural progression and represent promising biomarkers for clinical trials.<\/jats:p>","DOI":"10.1177\/22143602261491510","type":"journal-article","created":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T18:06:26Z","timestamp":1791569186000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Combined qualitative and quantitative MRI reveals short-term progression in facioscapulohumeral muscular dystrophy"],"prefix":"10.1177","author":[{"given":"Anna-Lena","family":"Mayer","sequence":"first","affiliation":[{"name":"University Hospital Erlangen"},{"name":"Friedrich-Alexander-University Erlangen-N\u00fcrnberg (FAU)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anika","family":"Starke","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsklinikum Erlangen"},{"name":"Friedrich-Alexander-University Erlangen-N\u00fcrnberg (FAU)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Schunk","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsklinikum Erlangen"},{"name":"Friedrich-Alexander-University Erlangen-N\u00fcrnberg (FAU)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"T\u00fcrk","sequence":"additional","affiliation":[{"name":"University Hospital Erlangen"},{"name":"Friedrich-Alexander-Universit\u00e4t Erlangen-N\u00fcrnberg (FAU)"},{"name":"University Hospital Erlangen"},{"name":"Friedrich-Alexander-Universit\u00e4t Erlangen-N\u00fcrnberg (FAU)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Uder","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsklinikum Erlangen"},{"name":"Friedrich-Alexander-University Erlangen-N\u00fcrnberg (FAU)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnd","family":"D\u00f6rfler","sequence":"additional","affiliation":[{"name":"University Hospital Erlangen"},{"name":"Friedrich-Alexander-University Erlangen-N\u00fcrnberg (FAU)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Armin M.","family":"Nagel","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsklinikum Erlangen"},{"name":"Friedrich-Alexander-University Erlangen-N\u00fcrnberg (FAU)"},{"name":"German Cancer Research Center (DKFZ)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre-Yves","family":"Baudin","sequence":"additional","affiliation":[{"name":"Institute of Myology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabetta","family":"Gazzerro","sequence":"additional","affiliation":[{"name":"Charit\u00e9 and Max-Delbr\u00fcck-Center for Molecular Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9734-4632","authenticated-orcid":false,"given":"Teresa","family":"Gerhalter","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsklinikum Erlangen"},{"name":"Friedrich-Alexander-University Erlangen-N\u00fcrnberg (FAU)"},{"name":"Medical University of Graz"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2026,10,9]]},"reference":[{"key":"e_1_3_8_2_2","doi-asserted-by":"publisher","DOI":"10.3233\/JND-140045"},{"key":"e_1_3_8_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.23949"},{"key":"e_1_3_8_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2454"},{"key":"e_1_3_8_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2014.05.021"},{"key":"e_1_3_8_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2022.10.005"},{"key":"e_1_3_8_7_2","first-page":"565","article-title":"MRI of normal and pathological skeletal muscle","volume":"146","author":"Murphy W","year":"1986","unstructured":"Murphy W, Totty WG, Carroll JE. 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