{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T16:38:37Z","timestamp":1773419917842,"version":"3.50.1"},"reference-count":25,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T00:00:00Z","timestamp":1773360000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Efgartigimod, a neonatal Fc receptor blocker, has received regulatory approval for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in Japan in December 2024.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>To investigate the effectiveness and safety of efgartigimod in real-world clinical setting immediately after its approval.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a prospective, single-center, case series study to evaluate the switch from standard therapy to efgartigimod in five patients with typical or variant CIDP in Japan. Effectiveness was assessed with clinical responses defined by changes in the Inflammatory Rasch-built Overall Disability Scale, Inflammatory Neuropathy Cause and Treatment, Medical Research Council sum scores, and grip strength. The occurrence of adverse events (AEs) was also monitored.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Three patients had typical CIDP, one had variant CIDP of the motor type, and one had distal type CIDP. All patients exhibited a clinical response evaluated using at least one effectiveness endpoint after switching to efgartigimod treatment. Especially, the three patients with typical CIDP experienced an significant effectiveness of efgartigimod, even in those with an inadequate response to intravenous or subcutaneous immunoglobulin. Conversely, one patient with distal CIDP did not exhibit a response to efgartigimod treatment. One patient experienced a severe headache after efgartigimod treatment; however, the AE was manageable.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Efgartigimod is a useful treatment option for CIDP in real-world clinical practice. However, its effectiveness was different between the patients with typical CIDP and CIDP variant in our study, possibly due to variances in the immune pathophysiology of each disease subtype. Further validation is warranted in our exploratory findings.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fneur.2026.1748826","type":"journal-article","created":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T14:38:07Z","timestamp":1773412687000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Real-world outcomes after switching from standard therapy to efgartigimod in five patients with chronic inflammatory demyelinating polyradiculoneuropathy: a case series study in Japan"],"prefix":"10.3389","volume":"17","author":[{"given":"Tatsuya","family":"Imai","sequence":"first","affiliation":[{"name":"Division of Respirology, Neurology and Rheumatology, Department of Medicine, Kurume University School of Medicine","place":["Kurume, Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kenichi","family":"Irie","sequence":"additional","affiliation":[{"name":"Division of Respirology, Neurology and Rheumatology, Department of Medicine, Kurume University School of Medicine","place":["Kurume, Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shinichiro","family":"Mori","sequence":"additional","affiliation":[{"name":"Division of Respirology, Neurology and Rheumatology, Department of Medicine, Kurume University School of Medicine","place":["Kurume, Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomoaki","family":"Hoshino","sequence":"additional","affiliation":[{"name":"Division of Respirology, Neurology and Rheumatology, Department of Medicine, Kurume University School of Medicine","place":["Kurume, Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshihiro","family":"Ide","sequence":"additional","affiliation":[{"name":"Department of Neurology, Saga University","place":["Saga, Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takahisa","family":"Tateishi","sequence":"additional","affiliation":[{"name":"Division of Respirology, Neurology and Rheumatology, Department of Medicine, Kurume University School of Medicine","place":["Kurume, Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,3,13]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"242","DOI":"10.1111\/jns.12455","article-title":"European Academy of Neurology\/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint Task Force-Second revision","volume":"26","author":"den Van Bergh","year":"2021","journal-title":"J Peripher Nerv Syst"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"981","DOI":"10.1136\/jnnp-2019-320314","article-title":"Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment","volume":"90","author":"Lehmann","year":"2019","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"973","DOI":"10.1136\/jnnp-2014-309697","article-title":"Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype","volume":"86","author":"Mathey","year":"2015","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"3706","DOI":"10.1007\/s00415-020-09998-8","article-title":"Systematic literature review of burden of illness in chronic inflammatory demyelinating polyneuropathy (CIDP)","volume":"268","author":"Querol","year":"2021","journal-title":"J Neurol"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"38","DOI":"10.1136\/jnnp-2024-334165","article-title":"Novel therapies in CIDP","volume":"96","author":"Mair","year":"2024","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"1575464","DOI":"10.3389\/fimmu.2025.1575464","article-title":"A pathophysiological and mechanistic review of chronic inflammatory demyelinating polyradiculoneuropathy therapy","volume":"16","author":"Caballero-\u00c1vila","year":"2025","journal-title":"Front Immunol"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"715","DOI":"10.1038\/nri2155","article-title":"FcRn: the neonatal Fc receptor comes of age","volume":"7","author":"Roopenian","year":"2007","journal-title":"Nat Rev Immunol"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"415","DOI":"10.1038\/s41577-022-00821-1","article-title":"The therapeutic age of the neonatal Fc receptor","volume":"23","author":"Pyzik","year":"2023","journal-title":"Nat Rev Immunol"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"1013","DOI":"10.1016\/S1474-4422(24)00309-0","article-title":"Safety, tolerability, and efficacy of subcutaneous efgartigimod in patients with chronic inflammatory demyelinating polyradiculoneuropathy (ADHERE): a multicentre, randomised-withdrawal, double-blind, placebo-controlled, phase 2 trial","volume":"23","author":"Allen","year":"2024","journal-title":"Lancet Neurol"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"123313","DOI":"10.1016\/j.jns.2024.123313","article-title":"Early deterioration of CIDP following transition from IVIG to FcRn inhibitor treatment","volume":"468","author":"Levine","year":"2025","journal-title":"J Neurol Sci"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"1533167","DOI":"10.3389\/fimmu.2025.1533167","article-title":"Short-term treatment of CIDP with efgartigimod: a case series in China","volume":"16","author":"Sun","year":"2025","journal-title":"Front Immunol"},{"key":"ref12","article-title":"Combination subcutaneous injection","year":"2025"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"1159","DOI":"10.1001\/jamaneurol.2020.0781","article-title":"Monitoring clinical course and treatment response in chronic inflammatory demyelinating polyneuropathy during routine care: a review of clinical and laboratory assessment measures","volume":"77","author":"Allen","year":"2020","journal-title":"JAMA Neurol"},{"key":"ref14","doi-asserted-by":"publisher","first-page":"653734","DOI":"10.3389\/fneur.2021.653734","article-title":"Treatment approaches for atypical CIDP","volume":"12","author":"Menon","year":"2021","journal-title":"Front Neurol"},{"key":"ref15","doi-asserted-by":"publisher","first-page":"1471","DOI":"10.1212\/01.WNL.0000158680.89323.F8","article-title":"Clinical and electrophysiologic correlates of IVIg responsiveness in CIDP","volume":"64","author":"Iijima","year":"2005","journal-title":"Neurology"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"e1876","DOI":"10.1212\/WNL.0000000000011703","article-title":"Quantifying treatment-related fluctuations in CIDP: results of the GRIPPER study","volume":"96","author":"Allen","year":"2021","journal-title":"Neurology"},{"key":"ref17","doi-asserted-by":"publisher","first-page":"148","DOI":"10.1186\/s12974-015-0361-1","article-title":"Chronic inflammatory demyelinating polyneuropathy (CIDP): change of serum IgG dimer levels during treatment with intravenous immunoglobulins","volume":"12","author":"Ritter","year":"2015","journal-title":"J Neuroinflammation"},{"key":"ref18","doi-asserted-by":"publisher","first-page":"24","DOI":"10.3390\/antib9020024","article-title":"Considerations for optimizing dosing of immunoglobulins based on pharmacokinetic evidence","volume":"9","author":"Mahmood","year":"2020","journal-title":"Antibodies"},{"key":"ref19","doi-asserted-by":"publisher","first-page":"638816","DOI":"10.3389\/fneur.2021.638816","article-title":"Individualizing therapy in CIDP: a mini-review comparing the pharmacokinetics of Ig with SCIg and IVIg","volume":"12","author":"Beydoun","year":"2021","journal-title":"Front Neurol"},{"key":"ref20","doi-asserted-by":"publisher","first-page":"6961","DOI":"10.3390\/jcm12226961","article-title":"Subcutaneous immunoglobulin in chronic inflammatory demyelinating polyneuropathy: a historical perspective","volume":"12","author":"Cocito","year":"2023","journal-title":"J Clin Med"},{"key":"ref21","doi-asserted-by":"publisher","first-page":"116497","DOI":"10.1016\/j.jns.2019.116497","article-title":"Immunoglobulin administration for the treatment of CIDP: IVIG or SCIG?","volume":"408","author":"Allen","year":"2020","journal-title":"J Neurol Sci"},{"key":"ref22","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1002\/mus.27746","article-title":"CIDP prognosis in patients with IVIG treatment-related fluctuations","volume":"67","author":"Cook","year":"2023","journal-title":"Muscle Nerve"},{"key":"ref23","doi-asserted-by":"publisher","first-page":"836","DOI":"10.1111\/ene.12080","article-title":"Subcutaneous immunoglobulin in responders to intravenous therapy with chronic inflammatory demyelinating polyradiculoneuropathy","volume":"20","author":"Markvardsen","year":"2013","journal-title":"Eur J Neurol"},{"key":"ref24","doi-asserted-by":"publisher","first-page":"682","DOI":"10.3389\/fimmu.2014.00682","article-title":"Unraveling the interaction between FcRn and albumin: opportunities for design of albumin-based therapeutics","volume":"5","author":"Sand","year":"2015","journal-title":"Front Immunol"},{"key":"ref25","doi-asserted-by":"publisher","first-page":"4372","DOI":"10.1172\/JCI97911","article-title":"Neonatal Fc receptor antagonist efgartigimod safely and sustainably reduces IgGs in humans","volume":"128","author":"Ulrichts","year":"2018","journal-title":"J Clin Invest"}],"container-title":["Frontiers in Neurology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2026.1748826\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T14:38:08Z","timestamp":1773412688000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2026.1748826\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,13]]},"references-count":25,"alternative-id":["10.3389\/fneur.2026.1748826"],"URL":"https:\/\/doi.org\/10.3389\/fneur.2026.1748826","relation":{},"ISSN":["1664-2295"],"issn-type":[{"value":"1664-2295","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,13]]},"article-number":"1748826"}}