{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,5]],"date-time":"2026-03-05T15:55:27Z","timestamp":1772726127022,"version":"3.50.1"},"reference-count":34,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2021,5,10]],"date-time":"2021-05-10T00:00:00Z","timestamp":1620604800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005743","name":"Universit\u00e0 Cattolica del Sacro Cuore","doi-asserted-by":"publisher","award":["Linea D1 2017"],"award-info":[{"award-number":["Linea D1 2017"]}],"id":[{"id":"10.13039\/501100005743","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>In recent years, an autoantibody directed against the 5\u2032-citosolic nucleotidase1A (cN1A) was identified in the sera of sporadic inclusion body myositis (s-IBM) patients with widely variable sensitivity (33%\u201376%) and specificity (87%\u2013100%). We assessed the sensitivity\/specificity of anti-cN1A antibodies in an Italian cohort of s-IBM patients, searching for a potential correlation with clinical data. We collected clinical data and sera from 62 consecutive s-IBM patients and 62 other inflammatory myopathies patients. Testing for anti-cN1A antibodies was performed using a commercial ELISA. Anti-cN1A antibodies were detected in 23 s-IBM patients, resulting in a sensitivity of 37.1% with a specificity of 96.8%. Positive and negative predictive values were 92.0% and 60.6%, respectively. We did not find significant difference regarding demographic variables, nor quadriceps or finger flexor weakness. Nevertheless, we found that anti-cN1A-positive patients presented significantly lower scores in IBMFRS item 1 (swallowing, p = 0.045) and more frequently reported more severe swallowing problems, expressed as an IBMFRS item 1 score \u2264 2 (p &lt; 0.001). We confirmed the low sensitivity and high specificity of anti-cN1A Ab in s-IBM patients with a high positive predictive value. The presence of anti-CN1A antibodies identified patients with a greater risk of more severe dysphagia.<\/jats:p>","DOI":"10.3390\/cells10051146","type":"journal-article","created":{"date-parts":[[2021,5,10]],"date-time":"2021-05-10T10:49:51Z","timestamp":1620643791000},"page":"1146","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Anti-cN1A Antibodies Are Associated with More Severe Dysphagia in Sporadic Inclusion Body Myositis"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0447-2297","authenticated-orcid":false,"given":"Matteo","family":"Lucchini","sequence":"first","affiliation":[{"name":"Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Roma, Italy"},{"name":"Department of Neurosciences, Section of Neurology, Universit\u00e0 Cattolica del Sacro Cuore, 00168 Roma, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0932-5173","authenticated-orcid":false,"given":"Lorenzo","family":"Maggi","sequence":"additional","affiliation":[{"name":"Neuroimmunology and Neuromuscular Diseases Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, 20133 Milano, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elena","family":"Pegoraro","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, University of Padova, 35122 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2852-7512","authenticated-orcid":false,"given":"Massimiliano","family":"Filosto","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Sciences, University of Brescia, NeMO-Brescia Clinical Center for Neuromuscular Diseases, 25121 Brescia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2543-6356","authenticated-orcid":false,"given":"Carmelo","family":"Rodolico","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, University of Messina, 98122 Messina, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanni","family":"Antonini","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Mental Health and Sensory Organs (NESMOS), School of Medicine and Psychology, Sant\u2019Andrea Hospital, Sapienza University of Rome, 00189 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1830-2886","authenticated-orcid":false,"given":"Matteo","family":"Garibaldi","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Mental Health and Sensory Organs (NESMOS), School of Medicine and Psychology, Sant\u2019Andrea Hospital, Sapienza University of Rome, 00189 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Lucia","family":"Valentino","sequence":"additional","affiliation":[{"name":"IRCCS Istituto delle Scienze Neurologiche di Bologna, 40139 Bologna, Italy"},{"name":"Department of Biomedical and Neuromotor Sciences, University of Bologna, 40126 Bologna, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gabriele","family":"Siciliano","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, University of Pisa, 56126 Pisa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0849-9144","authenticated-orcid":false,"given":"Giorgio","family":"Tasca","sequence":"additional","affiliation":[{"name":"Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Roma, Italy"},{"name":"Department of Neurosciences, Section of Neurology, Universit\u00e0 Cattolica del Sacro Cuore, 00168 Roma, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Valeria","family":"De Arcangelis","sequence":"additional","affiliation":[{"name":"Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Roma, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chiara","family":"De Fino","sequence":"additional","affiliation":[{"name":"Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Roma, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7783-114X","authenticated-orcid":false,"given":"Massimiliano","family":"Mirabella","sequence":"additional","affiliation":[{"name":"Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Roma, Italy"},{"name":"Department of Neurosciences, Section of Neurology, Universit\u00e0 Cattolica del Sacro Cuore, 00168 Roma, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,5,10]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1002\/ana.410380504","article-title":"Inclusion body myositis and myopathies","volume":"38","author":"Griggs","year":"1995","journal-title":"Ann. Neurol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1002\/ana.410390318","article-title":"Use of anti-neurofilament antibody to identify paired-helical filaments in inclusion-body myositis","volume":"39","author":"Askanas","year":"1996","journal-title":"Ann. Neurol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1350","DOI":"10.1016\/j.jocn.2008.01.011","article-title":"Prevalence of sporadic inclusion body myositis and factors contributing to delayed diagnosis","volume":"15","author":"Needham","year":"2008","journal-title":"J. Clin. Neurosci."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1044","DOI":"10.1016\/j.nmd.2013.08.007","article-title":"188th ENMC International Workshop: Inclusion Body Myositis, 2\u20134 December 2011, Naarden, The Netherlands","volume":"23","author":"Rose","year":"2013","journal-title":"Neuromuscul. Disord."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"956","DOI":"10.1002\/mus.24661","article-title":"Magnetic resonance imaging pattern recognition in sporadic inclusion-body myositis","volume":"52","author":"Tasca","year":"2015","journal-title":"Muscle Nerve"},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Salajegheh, M., Lam, T., and Greenberg, S.A. (2011). Autoantibodies against a 43 KDa Muscle Protein in Inclusion Body Myositis. PLoS ONE, 6.","DOI":"10.1371\/journal.pone.0020266"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1002\/ana.23822","article-title":"Autoantibodies to cytosolic 5\u2032-nucleotidase 1A in inclusion body myositis","volume":"73","author":"Pluk","year":"2012","journal-title":"Ann. Neurol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1002\/ana.23840","article-title":"Cytosolic 5\u2032-nucleotidase 1A autoimmunity in sporadic inclusion body myositis","volume":"73","author":"Larman","year":"2013","journal-title":"Ann. Neurol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1002\/mus.24199","article-title":"Cytoplasmic 5\u2032-nucleotidase autoantibodies in inclusion body myositis: Isotypes and diagnostic utility","volume":"50","author":"Greenberg","year":"2014","journal-title":"Muscle Nerve"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"654","DOI":"10.1002\/mus.24989","article-title":"Anti- C N1A antibodies in South Australian patients with inclusion body myositis","volume":"53","author":"Limaye","year":"2016","journal-title":"Muscle Nerve"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1136\/jnnp-2014-310008","article-title":"Seropositivity for NT5c1A antibody in sporadic inclusion body myositis predicts more severe motor, bulbar and respiratory involvement","volume":"87","author":"Goyal","year":"2016","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1136\/annrheumdis-2014-206691","article-title":"Disease specificity of autoantibodies to cytosolic 5\u2032-nucleotidase 1A in sporadic inclusion body myositis versus known autoimmune diseases","volume":"75","author":"Herbert","year":"2016","journal-title":"Ann. Rheum. Dis."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"512","DOI":"10.1002\/ana.24919","article-title":"Pathomechanisms of anti-cytosolic 5\u2032-nucleotidase 1A autoantibodies in sporadic inclusion body myositis","volume":"81","author":"Tawara","year":"2017","journal-title":"Ann. Neurol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"862","DOI":"10.1136\/annrheumdis-2016-210282","article-title":"Cytosolic 5\u2032-nucleotidase 1A autoantibody profile and clinical characteristics in inclusion body myositis","volume":"76","author":"Lilleker","year":"2017","journal-title":"Ann. Rheum. Dis."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"660","DOI":"10.1016\/j.nmd.2018.06.005","article-title":"Sensitivity and clinical utility of the anti-cytosolic 5\u2032-nucleotidase 1A (cN1A) antibody test in sporadic inclusion body myositis: Report of 40 patients from a single neuromuscular center","volume":"28","author":"Felice","year":"2018","journal-title":"Neuromuscul. Disord."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1097\/BOR.0000000000000216","article-title":"Novel serology testing for sporadic inclusion body myositis","volume":"27","author":"Herbert","year":"2015","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"745","DOI":"10.3389\/fimmu.2019.00745","article-title":"Anti-NT5c1A Autoantibodies as Biomarkers in Inclusion Body Myositis","volume":"10","author":"Amlani","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1002\/acr.22600","article-title":"Cytosolic 5\u2032-Nucleotidase 1A As a Target of Circulating Autoantibodies in Autoimmune Diseases","volume":"68","author":"Lloyd","year":"2016","journal-title":"Arthritis Rheum."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1002\/mus.20958","article-title":"The Muscle Study Group (MSG) Inclusion body myositis functional rating scale: A reliable and valid measure of disease severity","volume":"37","author":"Jackson","year":"2008","journal-title":"Muscle Nerve"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s13317-016-0088-8","article-title":"Development and evaluation of a standardized ELISA for the determination of autoantibodies against cN-1A (Mup44, NT5C1A) in sporadic inclusion body myositis","volume":"7","author":"Kramp","year":"2016","journal-title":"Autoimmun. Highlights"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1002\/mus.27157","article-title":"Anti-cN1A antibodies do not correlate with specific clinical, electromyographic, or pathological findings in sporadic inclusion body myositis","volume":"63","author":"Paul","year":"2021","journal-title":"Muscle Nerve"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1002\/ana.10121","article-title":"Comparison of weakness progression in inclusion body myositis during treatment with methotrexate or placebo","volume":"51","author":"Badrising","year":"2002","journal-title":"Ann. Neurol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1007\/s004150050005","article-title":"High-dose immunoglobulin therapy in sporadic inclusion body myositis: A double-blind, placebo-controlled study","volume":"247","author":"Walter","year":"2000","journal-title":"J. Neurol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1212\/WNL.56.3.323","article-title":"A controlled study of intravenous immunoglobulin combined with prednisone in the treatment of IBM","volume":"56","author":"Dalakas","year":"2001","journal-title":"Neurology"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1097\/BOR.0000000000000327","article-title":"Epidemiology of sporadic inclusion body myositis","volume":"28","author":"Molberg","year":"2016","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1007\/s00455-011-9338-0","article-title":"Dysphagia in Inflammatory Myopathy: Self-report, Incidence, and Prevalence","volume":"27","author":"Mulcahy","year":"2011","journal-title":"Dysphagia"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1093\/brain\/112.3.727","article-title":"INCLUSION BODY MYOSITIS","volume":"112","author":"Lotz","year":"1989","journal-title":"Brain"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"2009","DOI":"10.1007\/s00415-009-5229-9","article-title":"Detecting dysphagia in inclusion body myositis","volume":"256","author":"Cox","year":"2009","journal-title":"J. Neurol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1016\/j.jns.2017.09.014","article-title":"Five-year history of dysphagia as a sole initial symptom in inclusion body myositis","volume":"381","author":"Shibata","year":"2017","journal-title":"J. Neurol. Sci."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1007\/s00455-011-9384-7","article-title":"Pharyngeal Dysphagia in Inflammatory Muscle Diseases Resulting from Impaired Suprahyoid Musculature","volume":"27","author":"Langdon","year":"2011","journal-title":"Dysphagia"},{"key":"ref_31","first-page":"CD004303","article-title":"Interventions for dysphagia in long-term, progressive muscle disease","volume":"2","author":"Jones","year":"2016","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"465","DOI":"10.2147\/IJGM.S198031","article-title":"Dysphagia in Patients with Sporadic Inclusion Body Myositis: Management Challenges","volume":"12","author":"Mohannak","year":"2019","journal-title":"Int. J. Gen. Med."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"67","DOI":"10.3233\/JND-150138","article-title":"Mortality and Causes of Death in Patients with Sporadic Inclusion Body Myositis: Survey Study Based on the Clinical Experience of Specialists in Australia, Europe and the USA","volume":"3","author":"Price","year":"2016","journal-title":"J. Neuromuscul. Dis."},{"key":"ref_34","doi-asserted-by":"crossref","unstructured":"Shelly, S., Mielke, M.M., Mandrekar, J., Milone, M., Ernste, F.C., Naddaf, E., and Liewluck, T. (2021). Epidemiology and Natural History of Inclusion Body Myositis: A 40-Year Population-Based Study. Neurology, 20.","DOI":"10.1212\/WNL.0000000000012004"}],"container-title":["Cells"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4409\/10\/5\/1146\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T05:58:43Z","timestamp":1760162323000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4409\/10\/5\/1146"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,5,10]]},"references-count":34,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2021,5]]}},"alternative-id":["cells10051146"],"URL":"https:\/\/doi.org\/10.3390\/cells10051146","relation":{},"ISSN":["2073-4409"],"issn-type":[{"value":"2073-4409","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,5,10]]}}}