IgG4-related cholangitis – distinguishing from other benign biliary strictures: clinical features, imaging, and response to treatment: a retrospective study
Abstract Background IgG4-related cholangitis (IRC) is a rare systemic fibroinflammatory disorder that can affect multiple secretory organs, posing diagnostic challenges. It mimics both benign biliary strictures (BBS) and malignant biliary strictures (MBS). A hallmark feature of IRC is its dramatic r...
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BMC
2025-07-01
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| Online Access: | https://doi.org/10.1186/s12876-025-04114-4 |
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| author | Thanaput Kunlayawutipong Tanawat Pattarapuntakul Thanawin Wong Tanawat Jongraksak Nisa Netinatsunton Jaksin Sottisuporn Thanapon Yaowmaneerat Siriboon Attasaranya Pimsiri Sripongpun |
| author_facet | Thanaput Kunlayawutipong Tanawat Pattarapuntakul Thanawin Wong Tanawat Jongraksak Nisa Netinatsunton Jaksin Sottisuporn Thanapon Yaowmaneerat Siriboon Attasaranya Pimsiri Sripongpun |
| author_sort | Thanaput Kunlayawutipong |
| collection | DOAJ |
| description | Abstract Background IgG4-related cholangitis (IRC) is a rare systemic fibroinflammatory disorder that can affect multiple secretory organs, posing diagnostic challenges. It mimics both benign biliary strictures (BBS) and malignant biliary strictures (MBS). A hallmark feature of IRC is its dramatic response to systemic corticosteroids. Misdiagnosis may lead to incorrect treatment or unwarranted surgical procedures. This study aimed to compare the baseline characteristics, laboratory test results, imaging findings, and treatment responses between IRC and other BBS groups. Methods We reviewed all patients with a definitive diagnosis of BBS between January 2013 and January 2023. Data on serum bilirubin, serum IgG4 level, stent type, and response to treatment were collected. The baseline characteristics, biliary stricture treatment, and stent indwelling time were compared between the IRC and other BBS groups. Results A total of 158 patients with BBS were included (IRC, n = 19; other BBS, n = 139). The mean age was 59 years, and 62% were male. No significant difference in comorbidities or initial laboratory results was observed between the two groups. The IRC group had significantly higher rates of jaundice (73.7% vs. 29.7%, p < 0.001) and coexisting autoimmune pancreatitis (52.6% vs. 0%, p < 0.001). Patients with IRC had significantly higher serum IgG4 levels (5.384 g/L vs. 0.838 g/L, p < 0.001) and longer stricture lengths (23 mm vs. 7 mm, p < 0.001). Patients with IRC achieved complete responses to medication without requiring prolonged endoscopic stenting or surgery. Key diagnostic factors for IRC included being male (odds ratio [OR] 3.71, 95% confidence interval [CI] 1.03–13.32, p = 0.045), uniform circumferential bile duct thickening (OR 5.00, 95% CI 1.82–13.69, p = 0.002), long stricture length (> 15 mm) (OR 5.72, 95% CI 2.02–16.19, p = 0.001), The multivariate analysis demonstrated tissue lymphoplasmacytic infiltration (OR 88.38, 95% CI 7.98-978.53, P < 0.001) and Cholangiography type I (OR 22.47, 95% CI 2.63-192.26, p = 0.004). Conclusions IRC can be distinguished from other BBS through specific clinical and imaging features. Elevated serum IgG4 levels and tissue staining for IgG4-positive cells aid in accurate diagnosis. Recognising IRC, even in low-prevalence areas, guides appropriate treatment and avoids unnecessary surgery. |
| format | Article |
| id | doaj-art-7c551645d5cc4293b30abfab32d5263a |
| institution | Kabale University |
| issn | 1471-230X |
| language | English |
| publishDate | 2025-07-01 |
| publisher | BMC |
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| series | BMC Gastroenterology |
| spelling | doaj-art-7c551645d5cc4293b30abfab32d5263a2025-08-20T03:45:52ZengBMCBMC Gastroenterology1471-230X2025-07-0125111410.1186/s12876-025-04114-4IgG4-related cholangitis – distinguishing from other benign biliary strictures: clinical features, imaging, and response to treatment: a retrospective studyThanaput Kunlayawutipong0Tanawat Pattarapuntakul1Thanawin Wong2Tanawat Jongraksak3Nisa Netinatsunton4Jaksin Sottisuporn5Thanapon Yaowmaneerat6Siriboon Attasaranya7Pimsiri Sripongpun8Gastroenterology and Hepatology Unit, Division of Internal Medicine, Faculty of Medicine, Prince of Songkla UniversityGastroenterology and Hepatology Unit, Division of Internal Medicine, Faculty of Medicine, Prince of Songkla UniversityGastroenterology and Hepatology Unit, Division of Internal Medicine, Faculty of Medicine, Prince of Songkla UniversityGastroenterology and Hepatology Unit, Division of Internal Medicine, Faculty of Medicine, Prince of Songkla UniversityFaculty of Medicine, Nanthana-Kriangkrai Chotiwattanaphan Institute of Gastroenterology and Hepatology, Prince of Songkla UniversityFaculty of Medicine, Nanthana-Kriangkrai Chotiwattanaphan Institute of Gastroenterology and Hepatology, Prince of Songkla UniversityFaculty of Medicine, Nanthana-Kriangkrai Chotiwattanaphan Institute of Gastroenterology and Hepatology, Prince of Songkla UniversityFaculty of Medicine, Nanthana-Kriangkrai Chotiwattanaphan Institute of Gastroenterology and Hepatology, Prince of Songkla UniversityGastroenterology and Hepatology Unit, Division of Internal Medicine, Faculty of Medicine, Prince of Songkla UniversityAbstract Background IgG4-related cholangitis (IRC) is a rare systemic fibroinflammatory disorder that can affect multiple secretory organs, posing diagnostic challenges. It mimics both benign biliary strictures (BBS) and malignant biliary strictures (MBS). A hallmark feature of IRC is its dramatic response to systemic corticosteroids. Misdiagnosis may lead to incorrect treatment or unwarranted surgical procedures. This study aimed to compare the baseline characteristics, laboratory test results, imaging findings, and treatment responses between IRC and other BBS groups. Methods We reviewed all patients with a definitive diagnosis of BBS between January 2013 and January 2023. Data on serum bilirubin, serum IgG4 level, stent type, and response to treatment were collected. The baseline characteristics, biliary stricture treatment, and stent indwelling time were compared between the IRC and other BBS groups. Results A total of 158 patients with BBS were included (IRC, n = 19; other BBS, n = 139). The mean age was 59 years, and 62% were male. No significant difference in comorbidities or initial laboratory results was observed between the two groups. The IRC group had significantly higher rates of jaundice (73.7% vs. 29.7%, p < 0.001) and coexisting autoimmune pancreatitis (52.6% vs. 0%, p < 0.001). Patients with IRC had significantly higher serum IgG4 levels (5.384 g/L vs. 0.838 g/L, p < 0.001) and longer stricture lengths (23 mm vs. 7 mm, p < 0.001). Patients with IRC achieved complete responses to medication without requiring prolonged endoscopic stenting or surgery. Key diagnostic factors for IRC included being male (odds ratio [OR] 3.71, 95% confidence interval [CI] 1.03–13.32, p = 0.045), uniform circumferential bile duct thickening (OR 5.00, 95% CI 1.82–13.69, p = 0.002), long stricture length (> 15 mm) (OR 5.72, 95% CI 2.02–16.19, p = 0.001), The multivariate analysis demonstrated tissue lymphoplasmacytic infiltration (OR 88.38, 95% CI 7.98-978.53, P < 0.001) and Cholangiography type I (OR 22.47, 95% CI 2.63-192.26, p = 0.004). Conclusions IRC can be distinguished from other BBS through specific clinical and imaging features. Elevated serum IgG4 levels and tissue staining for IgG4-positive cells aid in accurate diagnosis. Recognising IRC, even in low-prevalence areas, guides appropriate treatment and avoids unnecessary surgery.https://doi.org/10.1186/s12876-025-04114-4IgG4-related cholangitisBenign biliary strictureObstructive jaundiceStent indwelling |
| spellingShingle | Thanaput Kunlayawutipong Tanawat Pattarapuntakul Thanawin Wong Tanawat Jongraksak Nisa Netinatsunton Jaksin Sottisuporn Thanapon Yaowmaneerat Siriboon Attasaranya Pimsiri Sripongpun IgG4-related cholangitis – distinguishing from other benign biliary strictures: clinical features, imaging, and response to treatment: a retrospective study BMC Gastroenterology IgG4-related cholangitis Benign biliary stricture Obstructive jaundice Stent indwelling |
| title | IgG4-related cholangitis – distinguishing from other benign biliary strictures: clinical features, imaging, and response to treatment: a retrospective study |
| title_full | IgG4-related cholangitis – distinguishing from other benign biliary strictures: clinical features, imaging, and response to treatment: a retrospective study |
| title_fullStr | IgG4-related cholangitis – distinguishing from other benign biliary strictures: clinical features, imaging, and response to treatment: a retrospective study |
| title_full_unstemmed | IgG4-related cholangitis – distinguishing from other benign biliary strictures: clinical features, imaging, and response to treatment: a retrospective study |
| title_short | IgG4-related cholangitis – distinguishing from other benign biliary strictures: clinical features, imaging, and response to treatment: a retrospective study |
| title_sort | igg4 related cholangitis distinguishing from other benign biliary strictures clinical features imaging and response to treatment a retrospective study |
| topic | IgG4-related cholangitis Benign biliary stricture Obstructive jaundice Stent indwelling |
| url | https://doi.org/10.1186/s12876-025-04114-4 |
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