DOI: 10.12890/2025_005376

Autoimmune polyglandular syndrome type 3B: a key to diagnosing autoimmune gastritis and asymptomatic primary biliary cholangitis

Authors

  • Sayaka Mabuchi Department of General Medicine, Iinan Hospital, Shimane, Japan
  • Hiroya Mabuchi Department of General Medicine, Iinan Hospital, Shimane, Japan
  • Takashi Watari Integrated Clinical Education Center, Kyoto University Hospital, Kyoto, Japan; General Medicine Center, Shimane University Hospital, Shimane, Japan

wataritari@gmail.com

Keywords

Autoimmune polyglandular syndrome type 3, diagnosis, autoimmune gastritis, primary biliary cholangitis

Abstract

Background: Autoimmune polyglandular syndrome type 3 (APS-3) encompasses autoimmune thyroid disease and other autoimmune disorders. APS-3 is further categorized into four subtypes, with APS-3B involving gastrointestinal autoimmune diseases. In this case, we diagnosed autoimmune gastritis, a condition challenging to identify based on endoscopic findings alone, and asymptomatic primary biliary cholangitis (PBC) through the recognition of APS-3B.
Case report: An 84-year-old female patient presented with forgetfulness and a history of Hashimoto’s disease. Although endoscopy revealed pan-atrophic gastritis, autoimmune gastritis was suspected rather than Helicobacter pylori infection due to consideration of the APS-3 framework. Specific observations, including adherent mucus and remnants of oxyntic mucosa, guided tests for anti-endogenous antibodies, confirming autoimmune gastritis. Recognizing APS-3B prompted further evaluation for PBC, including measurement of anti-mitochondrial M2 antibody, which led to the diagnosis of asymptomatic PBC.
Conclusion: Recognition of APS-3 offers a valuable framework for the differential diagnosis of autoimmune diseases.

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References

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Endoscopy shows pan-atrophy, remnants of oxyntic mucosa (blue arrow), and sticky, adherent, dense mucous (yellow arrows)

Published

2025-05-06

Issue

Vol. 12 No. 6 (2025)

Categories

How to Cite

1.
Mabuchi S, Mabuchi H, Watari T. Autoimmune polyglandular syndrome type 3B: a key to diagnosing autoimmune gastritis and asymptomatic primary biliary cholangitis. EJCRIM [Internet]. 2025 May 6 [cited 2026 Nov. 3];12(6). Available from: https://ejcrim.com/index.php/ejcrim/article/view/5376

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