Research Article

Diagnostic delay and harm from malignancy misclassification in autoimmune pancreatitis and IgG4-related sclerosing cholangitis: a real-world cohort study

Volume: 9 Number: 3 May 19, 2026
Journal of Health Sciences and Medicine Cover
Journal of Health Sciences and Medicine
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Diagnostic delay and harm from malignancy misclassification in autoimmune pancreatitis and IgG4-related sclerosing cholangitis: a real-world cohort study

Abstract

Aims: Autoimmune pancreatitis (AIP) and immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) can mimic pancreatobiliary malignancies, leading to diagnostic delay and malignancy misclassification. We evaluated phenotype-specific diagnostic delay and associated clinical consequences in a real-world cohort. Methods: In this retrospective cohort study (2019-2024), 68 adults with type 1 AIP and/or IgG4-SC were classified as combined AIP with IgG4-SC, isolated AIP, or isolated IgG4-SC. Diagnostic evaluation included cross-sectional imaging, cholangiography, endoscopic ultrasonography, histopathology, and corticosteroid response. Diagnostic delay, malignancy misclassification, and three harm domains-major iatrogenic harm, healthcare utilization burden, and documented psychosocial harm-were assessed. Results: Among 68 patients, 45.6% had combined AIP with IgG4-SC, 41.2% had isolated AIP, and 13.2% had isolated IgG4-SC. Diagnostic delay was longest in isolated IgG4-SC (median 210 days) compared with combined disease (85 days) and isolated AIP (70 days) (p<0.001). Malignancy misclassification occurred in 35.3% and was most frequent in isolated IgG4-SC (77.8%; p=0.012). Unnecessary major surgery occurred in 4.4% and only in isolated phenotypes. Misclassified patients underwent more endoscopic retrograde cholangiopancreatography procedures (median 3 vs. 1; p<0.001) and biliary stent placements (median 3 vs. 1; p<0.001). Documented psychosocial harm occurred in 26.5% overall and 75.0% of misclassified patients. Conclusion: Isolated IgG4-SC was associated with the longest diagnostic delay, the highest risk of malignancy misclassification, and the greatest burden of patient harm. These findings highlight isolated IgG4-SC as the highest-risk phenotype and support harm-aware diagnostic pathways to reduce unnecessary interventions and improve patient-centered outcomes in IgG4-related pancreatobiliary disease.

Keywords

Supporting Institution

No financial or institutional support was received for this study.

Ethical Statement

Not applicable

Thanks

The authors would like to thank the staff of the Gastroenterology Department of Kayseri City Hospital for their support during data collection.

References

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  6. Detlefsen S, Klöppel G. IgG4-related disease: with emphasis on the biopsy diagnosis of autoimmune pancreatitis and sclerosing cholangitis. Virchows Arch. 2018;472(4):545-556. doi:10.1007/s00428-017-2275-z
  7. Kamisawa T, Nakazawa T, Tazuma S, et al. Clinical practice guidelines for IgG4-related sclerosing cholangitis. J Hepatobiliary Pancreat Sci. 2019;26(1):9-42. doi:10.1002/jhbp.596
  8. Nakazawa T, Naitoh I, Hayashi K, et al. Diagnostic criteria for IgG4-related sclerosing cholangitis based on cholangiographic classification. J Gastroenterol. 2012;47(1):79-87. doi:10.1007/s00535-011-0465-z

Details

Primary Language

English

Subjects

Gastroenterology and Hepatology

Journal Section

Research Article

Authors

Publication Date

May 19, 2026

Submission Date

January 29, 2026

Acceptance Date

April 5, 2026

Published in Issue

Year 2026 Volume: 9 Number: 3

IZ

https://izlik.org/JA78WJ87PM

Cite

RIS / Bibtex
APA
Özden, Y., & Büyükberber, N. M. (2026). Diagnostic delay and harm from malignancy misclassification in autoimmune pancreatitis and IgG4-related sclerosing cholangitis: a real-world cohort study. Journal of Health Sciences and Medicine, 9(3), 652-657. https://izlik.org/JA78WJ87PM
AMA
1.Özden Y, Büyükberber NM. Diagnostic delay and harm from malignancy misclassification in autoimmune pancreatitis and IgG4-related sclerosing cholangitis: a real-world cohort study. J Health Sci Med / JHSM. 2026;9(3):652-657. https://izlik.org/JA78WJ87PM
Chicago
Özden, Yavuz, and Nuh Mehmet Büyükberber. 2026. “Diagnostic Delay and Harm from Malignancy Misclassification in Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis: A Real-World Cohort Study”. Journal of Health Sciences and Medicine 9 (3): 652-57. https://izlik.org/JA78WJ87PM.
EndNote
Özden Y, Büyükberber NM (May 1, 2026) Diagnostic delay and harm from malignancy misclassification in autoimmune pancreatitis and IgG4-related sclerosing cholangitis: a real-world cohort study. Journal of Health Sciences and Medicine 9 3 652–657.
IEEE
[1]Y. Özden and N. M. Büyükberber, “Diagnostic delay and harm from malignancy misclassification in autoimmune pancreatitis and IgG4-related sclerosing cholangitis: a real-world cohort study”, J Health Sci Med / JHSM, vol. 9, no. 3, pp. 652–657, May 2026, [Online]. Available: https://izlik.org/JA78WJ87PM
ISNAD
Özden, Yavuz - Büyükberber, Nuh Mehmet. “Diagnostic Delay and Harm from Malignancy Misclassification in Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis: A Real-World Cohort Study”. Journal of Health Sciences and Medicine 9/3 (May 1, 2026): 652-657. https://izlik.org/JA78WJ87PM.
JAMA
1.Özden Y, Büyükberber NM. Diagnostic delay and harm from malignancy misclassification in autoimmune pancreatitis and IgG4-related sclerosing cholangitis: a real-world cohort study. J Health Sci Med / JHSM. 2026;9:652–657.
MLA
Özden, Yavuz, and Nuh Mehmet Büyükberber. “Diagnostic Delay and Harm from Malignancy Misclassification in Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis: A Real-World Cohort Study”. Journal of Health Sciences and Medicine, vol. 9, no. 3, May 2026, pp. 652-7, https://izlik.org/JA78WJ87PM.
Vancouver
1.Yavuz Özden, Nuh Mehmet Büyükberber. Diagnostic delay and harm from malignancy misclassification in autoimmune pancreatitis and IgG4-related sclerosing cholangitis: a real-world cohort study. J Health Sci Med / JHSM [Internet]. 2026 May 1;9(3):652-7. Available from: https://izlik.org/JA78WJ87PM

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