• Keisuke Ishigami, Masahiro Shitani, Yasutoshi Kimura, Tadashi Hasegawa, Yoshiharu Masaki, Ayako Ito, Noriyuki Akutsu, Motohisa Yamamoto, Masayo Motoya, Shigeru Sasaki, Hiroki Takahashi, Ichiro Takemasa, and Hiroshi Nakase.
• Department of Gastroenterology and Hepatology.
• Medicine (Baltimore). 2018 Dec 1; 97 (52): e13868.

RationaleImmunoglobulin (Ig) G4-related disease (IgG4-RD) is a chronic inflammatory disorder characterized by high levels of serum IgG4, swollen organs with fibrosis and abundant infiltration of IgG4-positive plasmacytes.Patient ConcernsAn 82-year-old male visited our hospital for an evaluation of a pancreatic enlargement and a bilateral submandibular adenopathy. Further investigation revealed elevation of serum IgG4 and bilateral lacrimal submandibular adenopathy. We diagnosed him with IgG4-related disease (IgG4-RD) and started administration of corticosteroid (CS) therapy. Both pancreatic enlargement and adenopathy rapidly improved; however, there was a new occurrence of diffuse wall thickening of the gallbladder during CS treatment.DiagnosisRadiological examination revealed diffuse wall thickening of the gallbladder, and its inner layer was smooth and homogenous. These findings suggested an inflammatory change, but the possibility of malignancy could not be excluded.InterventionsThe patient underwent laparoscopic cholecystectomy for a pathological diagnosis.OutcomesHistological examination revealed a transmural infiltration of IgG4 positive plasma cells and dense fibrosis. The patient was pathologically diagnosed with IgG4 related cholecystitis presenting as an ectopic relapse.LessonsThere are 2 major types of IgG4-related cholecystitis, a diffuse wall thickening type and a mass formation type. It is sometimes difficult to differentiate IgG4-related cholecystitis with gallbladder cancer.Corticosteroid (CS) is effective for induction of remission; however, we sometimes encounter disease relapse after reduction of CS dose. We should be mindful that some patients may relapse with new organ involvements even if the primary site and serum IgG4 level are well controlled.

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