{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T17:48:19Z","timestamp":1792172899111,"version":"4.4.0"},"reference-count":59,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2025,7,23]],"date-time":"2025-07-23T00:00:00Z","timestamp":1753228800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Antibodies"],"abstract":"<jats:p>Background\/Objectives: Immune-mediated necrotizing myopathy (IMNM) is a severe inflammatory myopathy marked by proximal muscle weakness, elevated creatine kinase (CK), and the presence of anti-HMGCR antibodies. Statin exposure is a recognized trigger for anti-HMGCR-positive IMNM, which may persist despite statin withdrawal. This pilot case series explores, for the first time, the use of bempedoic acid\u2014a liver-specific lipid-lowering agent with minimal muscle toxicity\u2014as an alternative to statins in these patients. Methods: We report 10 anti-HMGCR-antibody-positive IMNM patients (6 females, 4 males) previously on statins for primary prevention (8 on atorvastatin, 2 on simvastatin) without prior cardiovascular events. Statins were discontinued at myositis onset. All patients received prednisone and immunosuppressants (methotrexate in 7, mycophenolate in 3), plus bempedoic acid. Anti-HMGCR antibodies were measured using a chemiluminescence method. Results: Their mean anti-HMGCR antibody levels decreased significantly from 390.93 \u00b1 275.22 to 220.89 \u00b1 113.37 CU\/L (p = 0.027) after 6 months of treatment. Their CK levels dropped from 1278.9 \u00b1 769.39 to 315.1 \u00b1 157.72 IU\/L (p = 0.001), and aldolase dropped from 11.63 \u00b1 2.18 to 6.61 \u00b1 1.22 U\/L (p = 0.0001). The mean LDL-C value was 96.1 \u00b1 8.16 mg\/dL. No disease recurrence was observed. Autoimmune panels were negative for other myositis-associated and\/or -specific antibodies. Conclusions: Bempedoic acid appears to be a safe, effective, and cost-efficient lipid-lowering alternative in statin-intolerant IMNM patients. Larger studies are warranted to confirm its efficacy across different subgroups and to optimize dyslipidemia management in this setting.<\/jats:p>","DOI":"10.3390\/antib14030063","type":"journal-article","created":{"date-parts":[[2025,7,23]],"date-time":"2025-07-23T08:02:06Z","timestamp":1753257726000},"page":"63","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Anti-HMGCR-Antibody-Positive Statin-Induced Myositis: A Pilot Case Series on Treatment with Bempedoic Acid and Immunosuppressive Therapy"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8180-6660","authenticated-orcid":false,"given":"Maurizio","family":"Benucci","sequence":"first","affiliation":[{"name":"Rheumatology Unit, S. Giovanni di Dio Hospital, Azienda USL-Toscana Centro, 50143 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9342-2445","authenticated-orcid":false,"given":"Riccardo","family":"Terenzi","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, S. Giovanni di Dio Hospital, Azienda USL-Toscana Centro, 50143 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Li Gobbi","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, S. Giovanni di Dio Hospital, Azienda USL-Toscana Centro, 50143 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5009-1161","authenticated-orcid":false,"given":"Emanuele Antonio Maria","family":"Cassar\u00e0","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, S. Giovanni di Dio Hospital, Azienda USL-Toscana Centro, 50143 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4968-1882","authenticated-orcid":false,"given":"Tommaso","family":"Picchioni","sequence":"additional","affiliation":[{"name":"Internal Medicine Unit, S. Giovanni di Dio Hospital, Azienda USL-Toscana Centro, 50143 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edda","family":"Russo","sequence":"additional","affiliation":[{"name":"Clinical Pathology S. Giuseppe Hospital, Azienda USL-Toscana Centro, 50053 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Lari","sequence":"additional","affiliation":[{"name":"Immunology and Allergology Laboratory Unit, S. Giovanni di Dio Hospital, Azienda USL-Toscana Centro, 50143 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariangela","family":"Manfredi","sequence":"additional","affiliation":[{"name":"Immunology and Allergology Laboratory Unit, S. Giovanni di Dio Hospital, Azienda USL-Toscana Centro, 50143 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6200-4467","authenticated-orcid":false,"given":"Maria","family":"Infantino","sequence":"additional","affiliation":[{"name":"Immunology and Allergology Laboratory Unit, S. Giovanni di Dio Hospital, Azienda USL-Toscana Centro, 50143 Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,7,23]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2757","DOI":"10.1002\/art.27572","article-title":"A novel autoantibody recognizing 200 kd and 100-kd proteins is associated with an immune-mediated necrotizing myopathy","volume":"62","author":"Hong","year":"2010","journal-title":"Arthritis Rheum."},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Vinci, P., Panizon, E., Tosoni, L.M., Cerrato, C., Pellicori, F., Mearelli, F., Biasinutto, C., Fiotti, N., Di Girolamo, F.G., and Biolo, G. (2021). Statin-associated myopathy: Emphasis on mechanisms and targeted therapy. Int. J. Mol. 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