{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,6]],"date-time":"2026-11-06T09:35:40Z","timestamp":1793957740776,"version":"4.8.0"},"reference-count":32,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2021,10,13]],"date-time":"2021-10-13T00:00:00Z","timestamp":1634083200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,11,19]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>To review our diagnostic and treatment approaches concerning sporadic inclusion body myositis (sIBM) and polymyositis with mitochondrial pathology (PM-Mito), we conducted a retrospective analysis of clinical and histological data of 32 patients diagnosed as sIBM and 7 patients diagnosed as PM-Mito by muscle biopsy. Of 32 patients identified histologically as sIBM, 19 fulfilled the 2011 European Neuromuscular Center (ENMC) diagnostic criteria for \u201cclinico-pathologically defined sIBM\u201d at the time of biopsy. Among these, 2 patients developed sIBM after years of immunosuppressive treatment for organ transplantation. Of 11 patients fulfilling the histological but not the clinical criteria, including 3 cases with duration &amp;lt;12\u2009months, 8 later fulfilled the criteria for clinico-pathologically defined sIBM. Of 7 PM-Mito patients, 4 received immunosuppression with clinical improvement in 3. One of these later developed clinico-pathologically defined sIBM; 1 untreated patient progressed to clinically defined sIBM. Thus, muscle histology remains important for this differential diagnosis to identify sIBM patients not matching the ENMC criteria and the PM-Mito group. In the latter, we report at least 50% positive, if occasionally transient, response to immunosuppressive treatments and progression to sIBM in a minority. The mitochondrial abnormalities defining PM-Mito do not seem to define the threshold to immunosuppression unresponsiveness.<\/jats:p>","DOI":"10.1093\/jnen\/nlab101","type":"journal-article","created":{"date-parts":[[2021,10,18]],"date-time":"2021-10-18T03:28:57Z","timestamp":1634527737000},"page":"1060-1067","source":"Crossref","is-referenced-by-count":14,"title":["Diagnosis and Clinical Development of Sporadic Inclusion Body Myositis and Polymyositis With Mitochondrial Pathology: A Single-Center Retrospective Analysis"],"prefix":"10.1093","volume":"80","author":[{"given":"Maren","family":"Winkler","sequence":"first","affiliation":[{"name":"Neuromuscular Diseases, Department of Neurology, University Hospital of Bonn From the Section of , Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina","family":"von Landenberg","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases, Department of Neurology, University Hospital of Bonn From the Section of , Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karin","family":"Kappes-Horn","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases, Department of Neurology, University Hospital of Bonn From the Section of , Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephan","family":"Neudecker","sequence":"additional","affiliation":[{"name":"Group Practice for Neurology , Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0111-7281","authenticated-orcid":false,"given":"Cornelia","family":"Kornblum","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases, Department of Neurology, University Hospital of Bonn From the Section of , Bonn, Germany"},{"name":"Center for Rare Diseases, University Hospital of Bonn , Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3349-6877","authenticated-orcid":false,"given":"Jens","family":"Reimann","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases, Department of Neurology, University Hospital of Bonn From the Section of , Bonn, Germany"},{"name":"Neuromuscular Diseases, Department of Neurology, University Hospital of Bonn From the Section of , Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,10,13]]},"reference":[{"key":"2024032523425010800_nlab101-B1","doi-asserted-by":"crossref","first-page":"1240","DOI":"10.1136\/jnnp-2013-305690","article-title":"Clinical assessment determines the diagnosis of inclusion body myositis independently of pathological features","volume":"84","author":"Brady","year":"2013","journal-title":"J Neurol Neurosurg 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