{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,7]],"date-time":"2026-01-07T07:02:49Z","timestamp":1767769369507,"version":"3.48.0"},"reference-count":17,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,1,7]],"date-time":"2026-01-07T00:00:00Z","timestamp":1767744000000},"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. Immunol."],"abstract":"<jats:p>Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated chronic fibrotic inflammatory disorder. The coexistence of IgG4-RD with monoclonal gammopathy of undetermined significance (MGUS) is relatively rare, with only a few cases reported in the literature. This article reports a case of a 79-year-old male patient with clinical manifestations of persistent upper abdominal pain of unknown cause and recurrent pleural effusion. Auxiliary examinations indicated positive serum antinuclear antibodies and persistently positive IgG4; three-dimensional imaging reconstruction showed mild pancreatic swelling and the peripancreatic fat planes appeared blurred, which was consistent with the manifestations of pancreatitis. According to the \u201cInternational Consensus Diagnostic Criteria for Autoimmune Pancreatitis (AIP)\u201dof the diagnostic criteria of the International Pancreatic Disease Association, the patient was diagnosed with IgG4-RD AIP. At the same time, the patient\u2019s serum protein electrophoresis M protein was 2.34 g\/L, and immunofixation electrophoresis confirmed it to be IgG-\u03bb-type M protein. Combined with bone marrow smear and biopsy results, it was diagnosed as having MGUS. To our knowledge, this represents a rare case of IgG4-RD AIP concomitant with MGUS, and we provide an in-depth discussion of its clinical management and potential pathogenic association.<\/jats:p>","DOI":"10.3389\/fimmu.2025.1729602","type":"journal-article","created":{"date-parts":[[2026,1,7]],"date-time":"2026-01-07T06:59:50Z","timestamp":1767769190000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: IgG4-RD-related autoimmune pancreatitis combined with monoclonal gammopathy of undetermined significance"],"prefix":"10.3389","volume":"16","author":[{"given":"Jinhu","family":"Wei","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiang","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Li","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yao","family":"Xie","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wanman","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jiyuan","family":"Liang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,1,7]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1097\/MPA.0b013e3182142fd2","article-title":"International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology","volume":"40","author":"Shimosegawa","year":"2011","journal-title":"Pancreas"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1016\/j.clml.2023.02.004","article-title":"Monoclonal gammopathy of undetermined significance: a comprehensive review","volume":"23","author":"Kaur","year":"2023","journal-title":"Clin Lymphoma Myeloma Leuk"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1002\/art.41380","article-title":"2020 International consensus guidance statement on the management and treatment of IgG4-Related disease","volume":"72","author":"Khosroshahi","year":"2020","journal-title":"Arthritis Rheumatol"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2013.05.018","article-title":"Mycophenolate mofetil for treatment of IgG4-related disease","volume":"11","author":"Hart","year":"2013","journal-title":"Clin Gastroenterol Hepatol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.3760\/cma.j.cn112138-20221105-00830","article-title":"Recommendations for the diagnosis and treatment of IgG(4)-related disease in China","volume":"62","author":"Fei","year":"2023","journal-title":"Chin J Internal Med"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.3969\/j.issn.1000-484X.2025.04.029","article-title":"Application of serum IgG4 detection in the diagnosis, treatment and outcome of IgG4-RD","volume":"41","author":"Yang","year":"2025","journal-title":"Chin J Immunol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.3870\/j.issn.1672-0741.2009.06.032","article-title":"Determination of serum CA125 in patients with systemic lupus erythematosus and its clinical significance","volume":"38","author":"Zhang","year":"2009","journal-title":"Acta Medicinae Universitatis Scientiae Technologiae Huazhong"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.3969\/j.issn.1671-6450.2014.07.032","article-title":"A case of systemic lupus erythematosus characterized by elevated CA125","author":"Xu","year":"2014","journal-title":"Chin J Difficult Complicated Cases"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(14)70442-5","article-title":"International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma","volume":"15","author":"Rajkumar","year":"2014","journal-title":"Lancet Oncol"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1709974","article-title":"Long-term follow-up of monoclonal gammopathy of undetermined significance","volume":"378","author":"Kyle","year":"2018","journal-title":"N Engl J Med"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa054494","article-title":"Prevalence of monoclonal gammopathy of undetermined significance","volume":"354","author":"Kyle","year":"2006","journal-title":"N Engl J Med"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.3390\/cancers12061554","article-title":"Monoclonal gammopathy of undetermined significance (MGUS)-not so asymptomatic after all","volume":"12","author":"Lomas","year":"2020","journal-title":"Cancers"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-024-07062-8","article-title":"Elevated polyclonal IgG4 mimicking a monoclonal gammopathy in IgG4-related disease-a case-based review","volume":"43","author":"Lu","year":"2024","journal-title":"Clin Rheumatol"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2025.1565388","article-title":"Case Report: IgG4-RD-related ophthalmopathy combined with monoclonal gammopathy of undetermined significance","volume":"16","author":"Liang","year":"2025","journal-title":"Front Immunol"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1016\/j.hoc.2023.12.011","article-title":"Immune-pathogenesis of myeloma","volume":"38","author":"Dhodapkar","year":"2024","journal-title":"Hematol Oncol Clin North Am"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1016\/j.wneu.2020.07.212","article-title":"IgG4-related disease in the frontal convexity concomitant with smoldering multiple myeloma: a case report and review of the literature regarding therapeutic implications","volume":"143","author":"Tanaka","year":"2020","journal-title":"World Neurosurg"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2018.205526","article-title":"IgG4-related disease: what a hematologist needs to know","volume":"104","author":"Chen","year":"2019","journal-title":"Haematologica"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2025.1729602\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,7]],"date-time":"2026-01-07T07:00:05Z","timestamp":1767769205000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2025.1729602\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1,7]]},"references-count":17,"alternative-id":["10.3389\/fimmu.2025.1729602"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2025.1729602","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1,7]]},"article-number":"1729602"}}