{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,19]],"date-time":"2026-02-19T06:14:47Z","timestamp":1771481687602,"version":"3.50.1"},"reference-count":23,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,2,19]],"date-time":"2026-02-19T00:00:00Z","timestamp":1771459200000},"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>IgG4-related disease (Immunoglobulin G4-related disease) is an immune-mediated condition characterized by elevated serum IgG4 levels, clinically presenting as multi-organ enlargement. However, only case reports exist of IgG4-related disease presenting initially with peripheral neuropathy. This study aims to elucidate the clinical features of IgG4-related disease with prominent peripheral neuropathy manifestations.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We reviewed 16 cases of IgG4-related disease with peripheral neuropathy as the initial presentation, including 15 cases from the literature and one case from our institution. Patient data were extracted and analyzed, encompassing clinical characteristics, pathological features, electrophysiological findings, and treatment information.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The median age of onset was 64.5\u202fyears, with 3 female and 13 male patients. Initially, 15 patients presented with limb numbness and weakness, while 1 exhibited hoarseness. All 16 patients demonstrated organ enlargement in addition to peripheral nerve damage. Peripheral nerve electrophysiology revealed demyelination and\/or axonal lesions. All cases received oral corticosteroids, with immunosuppressants added in 3 cases. All patients responded well to corticosteroid therapy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Peripheral neuropathy may present as the initial manifestation of IgG4-related disease. Such neuropathy demonstrates favorable response to corticosteroid therapy, and the indication for adding immunosuppressive agents should be evaluated based on individual circumstances.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fneur.2026.1776740","type":"journal-article","created":{"date-parts":[[2026,2,19]],"date-time":"2026-02-19T05:24:08Z","timestamp":1771478648000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["When the nerve speaks first: IgG4-related disease unmasked by peripheral neuropathy"],"prefix":"10.3389","volume":"17","author":[{"given":"Yi","family":"Zhou","sequence":"first","affiliation":[{"name":"Department of Neurology, Peking University Third Hospital","place":["Beijing, China"]},{"name":"Department of Neurology, The Second Affiliated Hospital of Chengdu Medical College, China National Nuclear Corporation 416 Hospital","place":["Chengdu, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xinran","family":"Ma","sequence":"additional","affiliation":[{"name":"Department of Neurology, Peking University Third Hospital","place":["Beijing, China"]},{"name":"Beijing Key Laboratory of Biomarker and Translational Research in Neurodegenerative Diseases","place":["Beijing, China"]},{"name":"Key Laboratory for Neuroscience, National Health Commission\/Ministry of Education, Peking University","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jinxia","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Peking University Third Hospital","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kun","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Peking University Third Hospital","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Danfeng","family":"Zheng","sequence":"additional","affiliation":[{"name":"Department of Pathology, School of Basic Medical Sciences, Peking University Third Hospital, Peking University Health Science Center","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hongsong","family":"Song","sequence":"additional","affiliation":[{"name":"Department of Neurology, Peking University Third Hospital","place":["Beijing, China"]},{"name":"Beijing Key Laboratory of Biomarker and Translational Research in Neurodegenerative Diseases","place":["Beijing, China"]},{"name":"Key Laboratory for Neuroscience, National Health Commission\/Ministry of Education, Peking University","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dongsheng","family":"Fan","sequence":"additional","affiliation":[{"name":"Department of Neurology, Peking University Third Hospital","place":["Beijing, China"]},{"name":"Beijing Key Laboratory of Biomarker and Translational Research in Neurodegenerative Diseases","place":["Beijing, China"]},{"name":"Key Laboratory for Neuroscience, National Health Commission\/Ministry of Education, Peking University","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,2,19]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"172","DOI":"10.1111\/cns.13747","article-title":"Chronic inflammatory demyelinating polyneuropathy-like neuropathy in IgG4-related disease","volume":"28","author":"Wang","year":"2022","journal-title":"CNS Neurosci Ther"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"907","DOI":"10.1017\/cjn.2022.321","article-title":"IgG4-related disease of the central nervous system: a case series","volume":"50","author":"Abbas","year":"2023","journal-title":"Can J Neurol 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Neuroimmunol"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"175","DOI":"10.5414\/CN110547","article-title":"Vasculitic neuropathy associated with IgG4-related kidney disease: a case report and literature review","volume":"96","author":"Jiang","year":"2021","journal-title":"Clin Nephrol"},{"key":"ref17","doi-asserted-by":"publisher","first-page":"502","DOI":"10.1001\/jamaneurol.2013.658","article-title":"IgG4-related neuropathy: a case report","volume":"70","author":"Ohyama","year":"2013","journal-title":"JAMA Neurol"},{"key":"ref18","doi-asserted-by":"publisher","first-page":"427","DOI":"10.1016\/j.humpath.2013.07.047","article-title":"Mononeuritis multiplex with tumefactive cellular infiltration in a patient with reactive lymphoid hyperplasia with increased immunoglobulin G4\u2013positive cells","volume":"45","author":"Yokoi","year":"2014","journal-title":"Hum Pathol"},{"key":"ref19","doi-asserted-by":"publisher","first-page":"490","DOI":"10.5114\/ceji.2018.81349","article-title":"A case of recurrent Mikulicz\u2019s disease with mononeuritis multiplex","volume":"43","author":"Mizuma","year":"2018","journal-title":"CEJOI"},{"key":"ref20","doi-asserted-by":"publisher","first-page":"e70133","DOI":"10.1111\/1756-185X.70133","article-title":"Immunoglobulin G4 -related disease presenting with peripheral neuropathy, membranous nephropathy and interstitial lung disease: a case report","volume":"28","author":"Yu","year":"2025","journal-title":"Int J Rheum Dis"},{"key":"ref21","doi-asserted-by":"publisher","first-page":"326","DOI":"10.1159\/000525908","article-title":"IgG4-related peripheral neuropathy with unilateral cervical nerve root and brachial plexus swelling: a case report","volume":"14","author":"Tarisawa","year":"2022","journal-title":"Case Rep 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