{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T05:46:50Z","timestamp":1792388810324,"version":"4.4.0"},"reference-count":14,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,10,18]],"date-time":"2026-10-18T00:00:00Z","timestamp":1792281600000},"content-version":"vor","delay-in-days":290,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Case Reports in Rheumatology"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:p>Immune\u2010mediated necrotizing myopathy (IMNM) associated with 3\u2010hydroxy\u20103\u2010methylglutaryl\u2010coenzyme A reductase (HMGCR) antibodies is a rare condition often associated with statin use. It typically presents with proximal weakness and markedly elevated creatine phosphokinase (CPK) levels and is often refractory, requiring long\u2010term immunotherapy. Respiratory involvement is uncommon but can be life\u2010threatening. Herein, we report the case of a 73\u2010year\u2010old woman with long\u2010standing anti\u2010HMGCR antibody\u2013positive IMNM, refractory to glucocorticoids, methotrexate, azathioprine, intravenous immunoglobulin (IVIg), rituximab, and plasma exchange (PLEX), who presented with progressive hypercapnic respiratory failure. Despite ongoing treatment with IVIg, PLEX, and high\u2010dose glucocorticoids, the patient\u2019s respiratory function worsened, and she required mechanical ventilation (MV). The addition of tofacitinib, a Janus kinase (JAK) inhibitor, coincided with marked clinical improvement, discontinuation of MV, and successful transition to room air. The patient continued to do well on this therapy, with improvement in muscle strength until outpatient discharge. The clinical benefit of JAK inhibition has been reported in dermatomyositis, overlap myositis, and anti\u2013MDA5\u2010positive disease, particularly in refractory cases with pulmonary or systemic involvement\u2014but has not been described in the setting of refractory IMNM. In our patient, who had exhausted multiple conventional therapies and PLEX, the rapid clinical improvement after JAK inhibition strongly suggests a therapeutic effect and highlights the need for future studies of JAK inhibitors in IMNM.<\/jats:p>","DOI":"10.1155\/crrh\/7268721","type":"journal-article","created":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T05:19:11Z","timestamp":1792387151000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Refractory Anti\u2010HMGCR Necrotizing Myopathy With Progressive Hypercapnic Respiratory Failure Responsive to a JAK Inhibitor"],"prefix":"10.1155","volume":"2026","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6649-5419","authenticated-orcid":false,"given":"N.","family":"Huang","sequence":"first","affiliation":[{"name":"University of Pittsburgh Myositis Center Division of Rheumatology and Clinical Immunology University of Pittsburgh School of Medicine  Pittsburgh Pennsylvania USA  pitt.edu"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1275-2723","authenticated-orcid":false,"given":"T. B.","family":"George","sequence":"additional","affiliation":[{"name":"University of Pittsburgh Myositis Center Division of Rheumatology and Clinical Immunology University of Pittsburgh School of Medicine  Pittsburgh Pennsylvania USA  pitt.edu"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7150-7306","authenticated-orcid":false,"given":"L.","family":"Christopher-Stine","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine Division of Rheumatology Johns Hopkins University School of Medicine  Baltimore Maryland USA  jhu.edu"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1194-8820","authenticated-orcid":false,"given":"A.","family":"Nicolas","sequence":"additional","affiliation":[{"name":"University of Pittsburgh Myositis Center Division of Rheumatology and Clinical Immunology University of Pittsburgh School of Medicine  Pittsburgh Pennsylvania USA  pitt.edu"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9529-3111","authenticated-orcid":false,"given":"C.","family":"Oddis","sequence":"additional","affiliation":[{"name":"University of Pittsburgh Myositis Center Division of Rheumatology and Clinical Immunology University of Pittsburgh School of Medicine  Pittsburgh Pennsylvania USA  pitt.edu"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1387-0860","authenticated-orcid":false,"given":"D. P.","family":"Ascherman","sequence":"additional","affiliation":[{"name":"University of Pittsburgh Myositis Center Division of Rheumatology and Clinical Immunology University of Pittsburgh School of Medicine  Pittsburgh Pennsylvania USA  pitt.edu"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7533-0491","authenticated-orcid":false,"given":"V. M.","family":"Liarski","sequence":"additional","affiliation":[{"name":"University of Pittsburgh Myositis Center Division of Rheumatology and Clinical Immunology University of Pittsburgh School of Medicine  Pittsburgh Pennsylvania USA  pitt.edu"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,10,18]]},"reference":[{"key":"e_1_2_9_1_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41584-020-00515-9"},{"key":"e_1_2_9_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2021.102993"},{"key":"e_1_2_9_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2023.103468"},{"key":"e_1_2_9_4_2","doi-asserted-by":"publisher","DOI":"10.3233\/jnd-170282"},{"key":"e_1_2_9_5_2","doi-asserted-by":"publisher","DOI":"10.1080\/03009742.2019.1672782"},{"key":"e_1_2_9_6_2","doi-asserted-by":"publisher","DOI":"10.3109\/03009742.2015.1132761"},{"key":"e_1_2_9_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.28333"},{"key":"e_1_2_9_8_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.41602"},{"key":"e_1_2_9_9_2","doi-asserted-by":"publisher","DOI":"10.23937\/2469-5726\/1510025"},{"key":"e_1_2_9_10_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-015-2891-4"},{"key":"e_1_2_9_11_2","doi-asserted-by":"publisher","DOI":"10.1136\/ard-2024-225732"},{"key":"e_1_2_9_12_2","doi-asserted-by":"publisher","DOI":"10.1212\/wnl.0000000000008128"},{"key":"e_1_2_9_13_2","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12955"},{"key":"e_1_2_9_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2023.103276"}],"container-title":["Case Reports in Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1155\/crrh\/7268721","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1155\/crrh\/7268721","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1155\/crrh\/7268721","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T05:19:12Z","timestamp":1792387152000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1155\/crrh\/7268721"}},"subtitle":[],"editor":[{"given":"Ankit","family":"Dhoundiyal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2026,1]]},"references-count":14,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026,1]]}},"alternative-id":["10.1155\/crrh\/7268721"],"URL":"https:\/\/doi.org\/10.1155\/crrh\/7268721","archive":["Portico"],"relation":{},"ISSN":["2090-6889","2090-6897"],"issn-type":[{"value":"2090-6889","type":"print"},{"value":"2090-6897","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1]]},"assertion":[{"value":"2025-11-12","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-10-08","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-10-18","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"7268721"}}