{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T13:25:17Z","timestamp":1787145917420,"version":"build-2736575974"},"reference-count":28,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T00:00:00Z","timestamp":1787097600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T00:00:00Z","timestamp":1787097600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"DOI":"10.13039\/501100004332","name":"Seoul National University Hospital","doi-asserted-by":"publisher","award":["SNUH Lee Kun-hee Child Cancer & Rare Disease Proje"],"award-info":[{"award-number":["SNUH Lee Kun-hee Child Cancer & Rare Disease Proje"]}],"id":[{"id":"10.13039\/501100004332","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Neuromuscular Diseases"],"abstract":"<jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Anti-signal recognition particle (SRP) myositis is a rare subset of immune-mediated necrotizing myopathy (IMNM). It is characterized by proximal muscle weakness, markedly elevated serum creatine kinase (CK) levels, and poor response to conventional therapies. Evidence for optimal management remains limited, particularly regarding the long-term outcomes of rituximab treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Method<\/jats:title>\n                    <jats:p>We retrospectively reviewed anti-SRP myositis patients who received rituximab at Seoul National University Hospital. Diagnosis was based on clinical features, elevated CK levels, and positivity for anti-SRP antibodies. Demographic, clinical, laboratory, radiological, histopathological, and treatment data were collected.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Five patients, aged between 4 and 71 years, exhibited proximal muscle weakness, elevated CK levels, and variable disease severity. Rituximab induction followed by maintenance therapy led to a significant improvement in muscle strength and normalization of CK and aldolase levels. In patients with mild or subclinical phenotypes, aldolase elevations often preceded clinical relapses. Relapses during follow-up were managed with rituximab re-administration, which restored muscle strength and biochemical markers. MRI, EMG, and muscle biopsy findings were consistent with myopathic changes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This case series highlights the heterogeneity in age, disease severity, and clinical course of anti-SRP myositis and the efficacy of rituximab in refractory and relapsing cases. Multimodal monitoring, including CK, aldolase, CD19+ B-cell counts, and clinical assessment, is essential for the early detection of relapses. Our findings support rituximab as a valuable component of individualized treatment strategies for anti-SRP myositis, though larger studies are warranted to establish standardized protocols and clarify long-term outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/22143602261478703","type":"journal-article","created":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T12:43:32Z","timestamp":1787143412000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Anti-SRP immune-mediated necrotizing myopathy from childhood to adulthood: A case series highlighting rituximab-responsive disease"],"prefix":"10.1177","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0520-7812","authenticated-orcid":false,"given":"Seon-Jae","family":"Ahn","sequence":"first","affiliation":[{"name":"Seoul National University Hospital"},{"name":"Seoul National University Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1583-2224","authenticated-orcid":false,"given":"Jee Min","family":"Kim","sequence":"additional","affiliation":[{"name":"Seoul National University Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Han Sang","family":"Lee","sequence":"additional","affiliation":[{"name":"Seoul National University Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3145-8714","authenticated-orcid":false,"given":"Seungbok","family":"Lee","sequence":"additional","affiliation":[{"name":"Seoul National University Hospital"},{"name":"Seoul National University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9173-922X","authenticated-orcid":false,"given":"Narae","family":"Kim","sequence":"additional","affiliation":[{"name":"Seoul National University Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Soo Yeon","family":"Kim","sequence":"additional","affiliation":[{"name":"Seoul National University Hospital"},{"name":"Seoul National University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9704-7870","authenticated-orcid":false,"given":"Hye Jin","family":"Yoo","sequence":"additional","affiliation":[{"name":"Seoul National University Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2992-7881","authenticated-orcid":false,"given":"Eun Na","family":"Kim","sequence":"additional","affiliation":[{"name":"Seoul National University Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1459-8093","authenticated-orcid":false,"given":"Jae-Kyung","family":"Won","sequence":"additional","affiliation":[{"name":"Seoul National University Hospital"},{"name":"Neuroscience Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin Sook","family":"Lee","sequence":"additional","affiliation":[{"name":"Seoul National University Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1282-4528","authenticated-orcid":false,"given":"Jangsup","family":"Moon","sequence":"additional","affiliation":[{"name":"Seoul National University Hospital"},{"name":"Seoul National University Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jong-Hee","family":"Chae","sequence":"additional","affiliation":[{"name":"Seoul National University Hospital"},{"name":"Seoul National University"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2026,8,19]]},"reference":[{"key":"e_1_3_4_2_2","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keaf183"},{"key":"e_1_3_4_3_2","unstructured":"Amir H Sabouri M PhD; Lisa Christopher-Stine M MPH; Jafar Kafaie M. Inflammatory Myopathies: Clinicopathologic classfication can aid understanding and guide treatment. 2021; (40\u201353)P: e41\u2013e45. Available at. https:\/\/practicalneurology.com\/diseases-diagnoses\/ms-immune-disorders\/inflammatory-myopathies\/31812\/. Accessed Aug 2nd 2025."},{"key":"e_1_3_4_4_2","first-page":"364","article-title":"An update on myositis autoantibodies and insights into pathogenesis","volume":"43","author":"Wang G","year":"2025","unstructured":"Wang G, McHugh NJ. An update on myositis autoantibodies and insights into pathogenesis. Clin Exp Rheumatol 2025; 43: 364\u2013371. https:\/\/doi.org\/10.55563\/clinexprheumatol\/kyj2cy","journal-title":"Clin Exp Rheumatol"},{"key":"e_1_3_4_5_2","doi-asserted-by":"publisher","DOI":"10.21037\/atm-21-475"},{"key":"e_1_3_4_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.biopha.2022.113936"},{"key":"e_1_3_4_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2017.09.016"},{"key":"e_1_3_4_8_2","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-015-0277-y"},{"key":"e_1_3_4_9_2","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2024.1439807"},{"key":"e_1_3_4_10_2","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.1019972"},{"key":"e_1_3_4_11_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41584-020-00515-9"},{"key":"e_1_3_4_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/acr.22920"},{"key":"e_1_3_4_13_2","doi-asserted-by":"publisher","DOI":"10.2478\/rir-2022-0001"},{"key":"e_1_3_4_14_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25575"},{"key":"e_1_3_4_15_2","doi-asserted-by":"publisher","DOI":"10.3988\/jcn.2022.0374"},{"key":"e_1_3_4_16_2","doi-asserted-by":"publisher","DOI":"10.3988\/jcn.2024.0007"},{"key":"e_1_3_4_17_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.11484"},{"key":"e_1_3_4_18_2","doi-asserted-by":"publisher","DOI":"10.1007\/s42399-020-00269-1"},{"key":"e_1_3_4_19_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.28114"},{"key":"e_1_3_4_20_2","doi-asserted-by":"publisher","DOI":"10.1002\/acr.25045"},{"key":"e_1_3_4_21_2","first-page":"766","article-title":"Necrotising myopathy associated with anti-signal recognition particle (anti-SRP) antibody","volume":"35","author":"De Souza FHC","year":"2017","unstructured":"De Souza FHC, Miossi R, Shinjo SK. Necrotising myopathy associated with anti-signal recognition particle (anti-SRP) antibody. Clin Exp Rheumatol 2017; 35: 766\u2013771.","journal-title":"Clin Exp Rheumatol"},{"key":"e_1_3_4_22_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-024-74940-1"},{"key":"e_1_3_4_23_2","first-page":"1076","article-title":"Case Report Anti-SRP positive immune-mediated necrotic myopathy with two ulnar fingers on the right hand: A case report","volume":"12","author":"Chen J","year":"2019","unstructured":"Chen J, Huang W, Jing F, et al. Case Report Anti-SRP positive immune-mediated necrotic myopathy with two ulnar fingers on the right hand: A case report. Int J Clin Exp Med 2019; 12: 1076\u20131085.","journal-title":"Int J Clin Exp Med"},{"key":"e_1_3_4_24_2","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2025.1455867"},{"key":"e_1_3_4_25_2","doi-asserted-by":"publisher","DOI":"10.1186\/s41927-025-00500-3"},{"key":"e_1_3_4_26_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41379-018-0164-x"},{"key":"e_1_3_4_27_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-023-02669-8"},{"key":"e_1_3_4_28_2","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-020-01431-7"},{"key":"e_1_3_4_29_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-020-04973-0"}],"container-title":["Journal of Neuromuscular Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/22143602261478703","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/22143602261478703","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/22143602261478703","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T12:43:34Z","timestamp":1787143414000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/22143602261478703"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,19]]},"references-count":28,"alternative-id":["10.1177\/22143602261478703"],"URL":"https:\/\/doi.org\/10.1177\/22143602261478703","relation":{},"ISSN":["2214-3599","2214-3602"],"issn-type":[{"value":"2214-3599","type":"print"},{"value":"2214-3602","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,19]]},"article-number":"22143602261478703"}}