Autoimmune Polyglandular Syndrome Type 3 Complicated with IgG4-related Disease

Intern Med. 2024 Feb 1;63(3):425-431. doi: 10.2169/internalmedicine.1270-22. Epub 2023 Jun 21.


A 52-year-old Japanese woman developed type 1 diabetes mellitus (type 1 DM) at 41 years old. She became complicated with Hashimoto's disease and showed swelling of both submandibular glands, which was diagnosed as IgG4-related disease (IgG4-RD). This is a rare case of a Japanese patient with autoimmune polyglandular syndrome type 3A (APS-3A) coexisting with autoimmune thyroid disease (AITD) and type 1 DM complicated by IgG4-RD. Bilateral submandibular gland resection was successfully performed without steroid therapy. We discuss the possibility that the immunological pathogenic mechanisms of APS-3A and IgG4-RD are related.

Keywords: IgG4-related disease; autoimmune pancreatitis; autoimmune polyglandular syndrome; type 1 diabetes mellitus.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Autoimmune Diseases* / complications
  • Autoimmune Diseases* / diagnosis
  • Diabetes Mellitus, Type 1* / complications
  • Female
  • Hashimoto Disease* / complications
  • Hashimoto Disease* / diagnosis
  • Humans
  • Immunoglobulin G4-Related Disease* / complications
  • Immunoglobulin G4-Related Disease* / diagnosis
  • Middle Aged
  • Polyendocrinopathies, Autoimmune* / complications
  • Polyendocrinopathies, Autoimmune* / diagnosis