{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T19:33:39Z","timestamp":1789500819824,"version":"build-2803163510"},"reference-count":67,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2021,5,27]],"date-time":"2021-05-27T00:00:00Z","timestamp":1622073600000},"content-version":"vor","delay-in-days":26,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100010275","name":"Deutsche Gesellschaft f\u00fcr Muskelkranke","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100010275","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100009438","name":"National Center of Neurology and Psychiatry","doi-asserted-by":"publisher","award":["Intramural Research Grant (2\u20105) for the Neurological and Psychiatric Disorders of NCNP"],"award-info":[{"award-number":["Intramural Research Grant (2\u20105) for the Neurological and Psychiatric Disorders of NCNP"]}],"id":[{"id":"10.13039\/501100009438","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Brain Pathology"],"published-print":{"date-parts":[[2021,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Sporadic late\u2010onset nemaline myopathy (SLONM) is a rare adult\u2010onset non\u2010hereditary disease with subacute proximal muscle and often axial muscle weakness, characterized by the presence of nemaline bodies in skeletal muscle biopsies. Considering its association with concurrent monoclonal gammopathy of undetermined significance (MGUS), the disease is classified into two major subtypes (1) SLONM without MGUS (SLONM\u2010noMGUS) and (2) with MGUS (SLONM\u2010MGUS) association. SLONM associated with HIV infection (SLONM\u2010HIV) is also reported. SLONM\u2010MGUS has been shown to be associated with poorer prognosis and required aggressive treatment including high\u2010dose melphalan and autologous stem cell transplantation. The approach is currently debatable as recent reports suggested effectiveness of intravenous immunoglobulin as initial treatment with indifference of overall survival despite the presence of MGUS. Our study aimed to find an underlying basis by review of pathological features in 49 muscle biopsy proven\u2010SLONM from two large tertiary centers in Japan and Germany (n\u00a0=\u00a049: SLONM\u2010noMGUS\u00a0=\u00a034, SLONM\u2010MGUS\u00a0=\u00a013, SLONM\u2010HIV\u00a0=\u00a02). We compared pathological findings in SLONM\u2010noMGUS and SLONM\u2010MGUS and focused on the presence of any detectable inflammatory features by immunohistochemistry. The clinical and histological features in SLONM\u2010noMGUS and SLONM\u2010MGUS were not distinctively different except for more common regenerating fibers (&gt;5% of myofibers) present in SLONM\u2010MGUS (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0&lt;\u00a00.01). HLA\u2010ABC expression and fine granular p62 were observed in 66.7% and 78.3% of SLONM, respectively. The predominant inflammatory cells were CD68\n                    <jats:sup>+<\/jats:sup>\n                    cells. The inflammatory cells showed positive correlations with the percentage of nemaline\u2010containing fibers (\n                    <jats:italic>p<\/jats:italic>\n                    \u00a0&lt;\u00a00.001). In conclusion, inflammatory features are present although rather mild in SLONM. This finding contributes to the hypothesis of an acquired inflammatory disease pathogenesis and opens the possibility to offer immunotherapy in SLONM with inflammatory features regardless of the monoclonal gammopathy status.\n                  <\/jats:p>","DOI":"10.1111\/bpa.12962","type":"journal-article","created":{"date-parts":[[2021,5,27]],"date-time":"2021-05-27T09:58:54Z","timestamp":1622109534000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Inflammatory features in sporadic late\u2010onset nemaline myopathy are independent from monoclonal gammopathy"],"prefix":"10.1111","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4110-9198","authenticated-orcid":false,"given":"Jantima","family":"Tanboon","sequence":"first","affiliation":[{"name":"Department of Neuromuscular Research National Institute of Neuroscience National Center of Neurology and Psychiatry (NCNP) Tokyo 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Germany"},{"name":"Department of Neuropathology Universit\u00e4tsmedizin Mainz Mainz Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ichizo","family":"Nishino","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Research National Institute of Neuroscience National Center of Neurology and Psychiatry (NCNP) Tokyo Japan"},{"name":"Department of Genome Medicine Development Medical Genome Center National Center of Neurology and Psychiatry (NCNP) Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1143-2103","authenticated-orcid":false,"given":"Werner","family":"Stenzel","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin Corporate Member of Freie Universit\u00e4t Berlin Humboldt\u2010Universit\u00e4t zu Berlin Berlin Germany"},{"name":"Leibniz ScienceCampus Chronic Inflammation Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,5,27]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0000000000000477"},{"key":"e_1_2_7_3_1","first-page":"713","article-title":"Late\u2010onset rod myopathy (a new syndrome?): light and electron microscopic observations in two cases","volume":"41","author":"Engel AG","year":"1966","journal-title":"Mayo Clin Proc"},{"key":"e_1_2_7_4_1","first-page":"308","article-title":"Late\u2010onset rod myopathy: a newly recognized acquired and progressive disease","volume":"16","author":"Engel 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