Ectopic relapse of IgG4-related disease presenting as IgG4-related sclerosing cholecystitis: A case report and review of literature

Abstract

Rationale: Immunoglobulin (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 concerns: An 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.

Diagnosis: Radiological 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.

Interventions: The patient underwent laparoscopic cholecystectomy for a pathological diagnosis.

Outcomes: Histological 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.

Lessons: There 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.

Figure 1

An 82-year-old man with autoimmune pancreatitis. (A) Contrast-enhanced CT showed diffuse pancreatic swelling with capsule-like rim (white arrowhead). (B) Gallbladder enlargement and intrahepatic bile duct dilation caused by constriction of the common bile duct.

Figure 2

(A) Sonogram showed symmetrical wall thickening with echogenic foci (white arrow). (B) After intravenous Perflubutane injection, the inner layer of the gallbladder was enhanced (red arrow).

Figure 3

T2-weighted MR imaging revealed homogeneous thickening wall structure with hyposignal intensity. Gallbadder stones were not observed.

Figure 4

(A) Microscopically, the thickened gallbladder wall showed diffuse fibrosis. (H-E staining, ×25) (B) (C) Transmural lymphoplasmacytic infiltration and abundant IgG4 positive plasma cells were observed (B: H-E staining, ×200, C: immunostaining for IgG4, ×200). No evidence of malignancy was observed.