Multivisceral IgG4-related disease presenting as recurrent massive gastrointestinal bleeding: a case report and literature review

Abstract

Background: IgG4-related disease (IgG4-RD) is a newly recognized autoimmune systemic disorder characterized by elevated levels of serum IgG4 and abundant infiltration of IgG4-positive plasmacytes in the affected organs. The liver, biliary system and pancreas are the most commonly affected organs. However, involvement of the digestive tract is very rare. To date, only a few cases of isolated gastric IgG4-RD have been reported.

Case presentation: We present a case of IgG4-RD of the liver, gallbladder, pancreas and duodenum, which was clinically misinterpreted and thereafter over-treated. A 52-year-old male presented with obstructive jaundice for 3 years, melena for 5 months and hematemesis for 10 days. Three years prior, the patient had undergone biopsies of pancreatic lesions, liver lesions, cholecystectomy and choledochojejunostomy. Histopathology showed chronic inflammatory changes. Endoscopy at admission revealed a duodenal ulcer with active bleeding. Despite medical management, the patient presented with repeated gastrointestinal bleeding. Upon evaluation, serum IgG4 levels were found to be elevated. Histopathology of the duodenal ulcer biopsy and repeated examination of the gallbladder and pancreatic and liver biopsies confirmed IgG4 positive plasma cell infiltration. A definitive diagnosis of IgG4-RD was made and steroid administration was initiated. At last follow up, 11 months to-the-day after initiating steroid treatment, the patient was asymptomatic.

Conclusions: Notably, IgG4-RD of multiple digestive organs is still very rare. As a systemic disease, it is characterized by the infiltration of IgG4-bearing plasma cells and raised IgG4 levels. Histopathology findings remain the diagnostic gold standard for this disorder.

Keywords: Duodenal; Gallbladder; Gastrointestinal bleeding; IgG4-related disease; Liver; Pancreatic; Plasma cells.

Fig. 1

Abdominal computed tomography. Images of the liver showed numerous hypodense nodules with mild contrast enhancement, ascites and intrahepatic bile duct dilatation (a). There were multiple enlarged lymph nodes in the hepatoduodenal ligament around the abdominal aorta (b). Mesenteric lymph nodes with bilateral pleural effusion (c). Multiple nodular dense shadows with dilatation of the main pancreatic duct in the tail region (d)

Fig. 2

Gastroscopy findings. An ulcer (1.5 cm × 1.2 cm) in the anterior wall of the duodenal bulb and a lot of white mosses in the fundus of stomach (a, b)

Fig. 3

Pathologic findings. a Diaminobenzidine staining of liver specimens revealed focal necrosis of the liver surrounded by fibrous tissue. The focal necrosis area revealed infiltration by lymphocytes, plasma cells and eosinophils. Peripheral hepatocytes were partially silt. b Immunoglobulin G (IgG) immunohistochemical staining showing increased numbers of IgG-positive plasma cells in the liver lesions (30–60/ high power field [HPF], × 400). c Diaminobenzidine staining of pancreas tissues revealed focal areas of glandular atrophy accompanied by lymphocyte, plasma cell and eosinophil infiltration and fibrous tissue hyperplasia. d IgG immunohistochemical staining showing increased numbers of IgG-positive plasma cells in the pancreas (30–80/HPF, × 400). e Diaminobenzidine staining of gallbladder tissue revealed chronic cholecystitis, focal lymphoid hyperplasia, formation of lymph follicles and infiltration of the gallbladder wall by the lymphocytes, plasma cells and eosinophils. f IgG immunohistochemical staining showing increased numbers of IgG-positive plasma cells in the gallbladder (30–80/HPF, × 400). g Hematin and yepin staining of duodenum tissue revealed lymphocytic, plasmacytic and neutrophilic infiltration of the inner layer of the duodenal mucosa with congestion and dilatation of the blood vessels. h IgG immunohistochemical staining showing increased numbers of IgG-positive plasma cells in the duodenum (> 100 /HPF, × 400)