Clinicopathological features of type 1 autoimmune pancreatitis without elevated serum IgG4 level
Abstract Elevated serum IgG4 is a key diagnostic marker for type 1 autoimmune pancreatitis (AIP), but some patients lack IgG4 elevation, complicating diagnosis. This study investigated the clinicopathological features of AIP without elevated serum IgG4 levels. A total of 323 patients diagnosed with...
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Nature Portfolio
2025-07-01
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| Online Access: | https://doi.org/10.1038/s41598-025-10478-0 |
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| author | Yumiko Yamashita Yasutaka Ishii Keiji Hanada Tamito Sasaki Yoshifumi Fujimoto Atsushi Yamaguchi Ken Hirao Masahiro Serikawa Bunjiro Noma Tomoyuki Minami Akihito Okazaki Masanobu Yukutake Teruo Mouri Yumiko Tatsukawa Shinya Nakamura Juri Ikemoto Shiro Oka |
| author_facet | Yumiko Yamashita Yasutaka Ishii Keiji Hanada Tamito Sasaki Yoshifumi Fujimoto Atsushi Yamaguchi Ken Hirao Masahiro Serikawa Bunjiro Noma Tomoyuki Minami Akihito Okazaki Masanobu Yukutake Teruo Mouri Yumiko Tatsukawa Shinya Nakamura Juri Ikemoto Shiro Oka |
| author_sort | Yumiko Yamashita |
| collection | DOAJ |
| description | Abstract Elevated serum IgG4 is a key diagnostic marker for type 1 autoimmune pancreatitis (AIP), but some patients lack IgG4 elevation, complicating diagnosis. This study investigated the clinicopathological features of AIP without elevated serum IgG4 levels. A total of 323 patients diagnosed with AIP at Hiroshima University Hospital and affiliated institutions were analyzed. Patients were categorized into IgG4-positive (≥ 135 mg/dL), IgG4-negative (< 135 mg/dL with “definite” or “probable” diagnosis), and possible (with “possible” diagnosis) groups. Comparative analysis was performed between the IgG4-positive (n = 281) and IgG4-negative (n = 20) groups. Segmental or focal narrowing of the main pancreatic duct (MPD) and retroperitoneal fibrosis were significantly more common in the IgG4-negative group (90.0% vs. 58.7%, P = 0.011; and 35.0% vs. 12.5%, P = 0.012, respectively). Although the number of pathological findings was comparable, the rate of surgical intervention was significantly higher in the IgG4-negative group (P < 0.001). No significant differences were observed in relapse rates or relapse sites between the two groups. These findings suggest that MPD narrowing and retroperitoneal fibrosis may be characteristic of AIP without elevated IgG4. Vigilant monitoring for relapse is warranted, regardless of serum IgG4 levels. |
| format | Article |
| id | doaj-art-227749f30bf64c279cc27cf3ec80b329 |
| institution | Kabale University |
| issn | 2045-2322 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | Nature Portfolio |
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| series | Scientific Reports |
| spelling | doaj-art-227749f30bf64c279cc27cf3ec80b3292025-08-20T04:02:51ZengNature PortfolioScientific Reports2045-23222025-07-011511910.1038/s41598-025-10478-0Clinicopathological features of type 1 autoimmune pancreatitis without elevated serum IgG4 levelYumiko Yamashita0Yasutaka Ishii1Keiji Hanada2Tamito Sasaki3Yoshifumi Fujimoto4Atsushi Yamaguchi5Ken Hirao6Masahiro Serikawa7Bunjiro Noma8Tomoyuki Minami9Akihito Okazaki10Masanobu Yukutake11Teruo Mouri12Yumiko Tatsukawa13Shinya Nakamura14Juri Ikemoto15Shiro Oka16Department of Gastroenterology, Graduate School of Biomedical & Health Sciences, Hiroshima UniversityDepartment of Gastroenterology, Graduate School of Biomedical & Health Sciences, Hiroshima UniversityDepartment of Gastroenterology, Onomichi General HospitalDepartment of Gastroenterology, Hiroshima Prefectural HospitalDepartment of Gastroenterology, Hiroshima General HospitalDepartment of Gastroenterology, National Hospital Organization Kure Medical Center, Chugoku Cancer CenterDepartment of Internal Medicine, Hiroshima City Hiroshima Citizens HospitalDepartment of Gastroenterology, Hiroshima Prefectural HospitalDepartment of Gastroenterology, Kure Kyosai HospitalDepartment of Gastroenterology, Hiroshima Red Cross & Atomic-bomb Survivors HospitalDepartment of Gastroenterology, National Hospital Organization Higashihiroshima Medical CenterDepartment of Gastroenterology, Hiroshima City Asa Citizens HospitalDepartment of Gastroenterology, Chugoku Rosai HospitalDepartment of Gastroenterology, Graduate School of Biomedical & Health Sciences, Hiroshima UniversityDepartment of Gastroenterology, Graduate School of Biomedical & Health Sciences, Hiroshima UniversityDepartment of Gastroenterology, Graduate School of Biomedical & Health Sciences, Hiroshima UniversityDepartment of Gastroenterology, Graduate School of Biomedical & Health Sciences, Hiroshima UniversityAbstract Elevated serum IgG4 is a key diagnostic marker for type 1 autoimmune pancreatitis (AIP), but some patients lack IgG4 elevation, complicating diagnosis. This study investigated the clinicopathological features of AIP without elevated serum IgG4 levels. A total of 323 patients diagnosed with AIP at Hiroshima University Hospital and affiliated institutions were analyzed. Patients were categorized into IgG4-positive (≥ 135 mg/dL), IgG4-negative (< 135 mg/dL with “definite” or “probable” diagnosis), and possible (with “possible” diagnosis) groups. Comparative analysis was performed between the IgG4-positive (n = 281) and IgG4-negative (n = 20) groups. Segmental or focal narrowing of the main pancreatic duct (MPD) and retroperitoneal fibrosis were significantly more common in the IgG4-negative group (90.0% vs. 58.7%, P = 0.011; and 35.0% vs. 12.5%, P = 0.012, respectively). Although the number of pathological findings was comparable, the rate of surgical intervention was significantly higher in the IgG4-negative group (P < 0.001). No significant differences were observed in relapse rates or relapse sites between the two groups. These findings suggest that MPD narrowing and retroperitoneal fibrosis may be characteristic of AIP without elevated IgG4. Vigilant monitoring for relapse is warranted, regardless of serum IgG4 levels.https://doi.org/10.1038/s41598-025-10478-0Autoimmune pancreatitisIgG4Retroperitoneal fibrosisLymphoplasmacytic sclerosing pancreatitisRelapse |
| spellingShingle | Yumiko Yamashita Yasutaka Ishii Keiji Hanada Tamito Sasaki Yoshifumi Fujimoto Atsushi Yamaguchi Ken Hirao Masahiro Serikawa Bunjiro Noma Tomoyuki Minami Akihito Okazaki Masanobu Yukutake Teruo Mouri Yumiko Tatsukawa Shinya Nakamura Juri Ikemoto Shiro Oka Clinicopathological features of type 1 autoimmune pancreatitis without elevated serum IgG4 level Scientific Reports Autoimmune pancreatitis IgG4 Retroperitoneal fibrosis Lymphoplasmacytic sclerosing pancreatitis Relapse |
| title | Clinicopathological features of type 1 autoimmune pancreatitis without elevated serum IgG4 level |
| title_full | Clinicopathological features of type 1 autoimmune pancreatitis without elevated serum IgG4 level |
| title_fullStr | Clinicopathological features of type 1 autoimmune pancreatitis without elevated serum IgG4 level |
| title_full_unstemmed | Clinicopathological features of type 1 autoimmune pancreatitis without elevated serum IgG4 level |
| title_short | Clinicopathological features of type 1 autoimmune pancreatitis without elevated serum IgG4 level |
| title_sort | clinicopathological features of type 1 autoimmune pancreatitis without elevated serum igg4 level |
| topic | Autoimmune pancreatitis IgG4 Retroperitoneal fibrosis Lymphoplasmacytic sclerosing pancreatitis Relapse |
| url | https://doi.org/10.1038/s41598-025-10478-0 |
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