Year 2025 / Volume 117 / Number 12
Letter
Unexpected diagnosis: IgG4-related sclerosing cholangitis with normal serum IgG4 levels - Unraveling diagnostic and therapeutic challenges

761-762

DOI: 10.17235/reed.2024.10711/2024

Sónia Barros, Luís Relvas, Isabel Malta Carvalho, Ana Margarida Vaz, Bruno Peixe,

Abstract
Diagnosing IgG4-related sclerosing cholangitis (IgG4-SC) presents significant challenges, especially when serum IgG4 levels are normal and other organs are not involved. We report a case of a 51-year-old patient with typical symptoms of biliary obstruction, including jaundice and abdominal pain. Despite normal serum IgG4 levels, imaging suggested cholangiocarcinoma, leading to extensive surgical intervention. Histological analysis of the surgical specimen revealed extensive lymphoplasmacytic infiltration and numerous IgG4-positive plasma cells, confirming IgG4-SC. This case underscores the difficulty in differentiating IgG4-SC from other biliary diseases such as primary sclerosing cholangitis and cholangiocarcinoma, highlighting the need for thorough histological examination in atypical presentations.
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References
1. Okazaki K, Uchida K. Current perspectives on autoimmune pancreatitis and IgG4-related disease. Proc Jpn Acad Ser B Phys Biol Sci. 2018;94(10):412-27.
2. Hori Y, Chari ST, Tsuji Y, Takahashi N, Inoue D, Hart PA, et al. Diagnosing Biliary Strictures: Distinguishing IgG4-Related Sclerosing Cholangitis From Cholangiocarcinoma and Primary Sclerosing Cholangitis. Mayo Clin Proc Innov Qual Outcomes. 2021;5(3):535-41.
3. Naitoh I, Nakazawa T. Classification and Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis. Gut Liver. 2022;16(1):28-36.
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Citation tools
Barros S, Relvas L, Carvalho I, Vaz A, Peixe B. Unexpected diagnosis: IgG4-related sclerosing cholangitis with normal serum IgG4 levels - Unraveling diagnostic and therapeutic challenges. 10711/2024


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Publication history

Received: 06/08/2024

Accepted: 28/08/2024

Online First: 05/09/2024

Published: 12/12/2025

Article Online First time: 30 days

Article editing time: 493 days


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