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

Abstract

BackgroundIgG4-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 presentationWe 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.ConclusionsNotably, 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.

Utility of gastric biopsy in diagnosing IgG4‐related gastrointestinal disease

“…IgG4‐related gastrointestinal (GI) disease (IgG4‐GID) is rare, but it has been reported anecdotally. In fact, various clinical manifestations, such as ulcerations, 1–5 polyps, 6–8 wall thickening, 9–16 submucosal tumors (SMTs), 17–27 GI obstruction, 28–30 vasculitis, 31 fistulas, 32,33 and the infiltration of IgG4‐positive plasma cells without morphologic abnormalities, 34 have been reported in IgG4‐GID. As a matter of fact, most cases of IgG4‐GID do not exhibit other organ involvement as observed in IgG4‐RD cases; thus, it is unlikely that all these cases represent genuine IgG4‐GID.…”

IgG4-Related Oesophageal Disease with Cytomegalovirus Infection: A Case Report

“…Thus, there is no relevant treatment experience to use for a reference. The first-line treatment strategy for IgG4-RD management consists of corticosteroid administration [ 29 ]. Ganciclovir, delivered by intravenous injection, was the first line of anti-CMV therapy, and the duration of the anti-CMV medication can vary from 3 to 16 weeks [ 30 ].…”