{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T13:59:39Z","timestamp":1781099979616,"version":"3.54.1"},"reference-count":26,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2026,1,9]],"date-time":"2026-01-09T00:00:00Z","timestamp":1767916800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,1,9]],"date-time":"2026-01-09T00:00:00Z","timestamp":1767916800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>T\u2010cell large granular lymphocytic leukemia (T\u2010LGLL), reported in up to 58% of inclusion body myositis (IBM) patients, is a rare leukemia of cytotoxic or less commonly helper T cells. The range of myopathies in T\u2010LGLL and the impact of coexisting T\u2010LGLL in IBM are not well understood. Our objectives are to investigate the spectrum of myopathies in patients with T\u2010LGLL and compare the clinical, serological, pathological, and transcriptomic features of IBM patients with and without T\u2010LGLL.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We reviewed two Mayo Clinic cohorts: (1) T\u2010LGLL patients evaluated for myopathies (2003\u20132018), and (2) IBM patients tested for T\u2010LGLL via T\u2010cell receptor gene rearrangement or flow cytometry (2016\u20132022). We also compared transcriptomic profiles of IBM muscle biopsies with and without T\u2010LGLL.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Of 447\u2009T\u2010LGLL patients, 13 (2.9%) had myopathies, IBM being the most common (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20097). Of 43 IBM patients, 9 (20.9%) had T\u2010LGLL, with 5 diagnosed prior to the onset of weakness. Clinical and pathological features were largely similar between IBM patients with and without T\u2010LGLL, except for milder finger flexor weakness and more frequent neutropenia (55.6% vs. 0%,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001) in the T\u2010LGLL group. However, transcriptomic analysis of muscle tissues revealed a more immunologically active profile, with upregulation of T\u2010cell and macrophage markers, elevated levels of IFN\u2010\u03b3 gene and IFN\u2010\u03b3\u2013inducible genes, heightened cytokine activity, and enhanced immunoglobulin production in IBM patients with T\u2010LGLL.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>IBM is the most common myopathy in T\u2010LGLL patients. While clinico\u2010sero\u2010pathological features were largely similar, transcriptomic differences suggest IBM with T\u2010LGLL may represent a distinct, more immune\u2010driven subtype.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/acn3.70301","type":"journal-article","created":{"date-parts":[[2026,1,9]],"date-time":"2026-01-09T16:29:29Z","timestamp":1767976169000},"page":"1196-1205","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Immune\u2010Driven Expression in Inclusion Body Myositis With T\u2010Cell Large Granular Lymphocytic Leukemia"],"prefix":"10.1002","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1285-0021","authenticated-orcid":false,"given":"Pannathat","family":"Soontrapa","sequence":"first","affiliation":[{"name":"Division of Neuromuscular Medicine, Department of Neurology Mayo Clinic  Rochester Minnesota USA"},{"name":"Division of Neurology, Department of Medicine, Siriraj Hospital, Mahidol University  Bangkok Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6338-9218","authenticated-orcid":false,"given":"Iago","family":"Pinal\u2010Fernandez","sequence":"additional","affiliation":[{"name":"Muscle Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health  Bethesda Maryland USA"},{"name":"Department of Neurology Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pritikanta","family":"Paul","sequence":"additional","affiliation":[{"name":"Department of Neurology University of California San Francisco  San Francisco, California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael P.","family":"Skolka","sequence":"additional","affiliation":[{"name":"Division of Neuromuscular Medicine, Department of Neurology Mayo Clinic  Jacksonville 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USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0212-5697","authenticated-orcid":false,"given":"Teerin","family":"Liewluck","sequence":"additional","affiliation":[{"name":"Division of Neuromuscular Medicine, Department of Neurology Mayo Clinic  Rochester Minnesota 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