IgG4-related disease presenting as a gastric mass necessitating total gastrectomy: a case report

IgG4-related disease (IgG4-RD) is a multi-system disorder that rarely presents with gastric manifestations. A 26 year old woman presented with post-prandial symptoms of dyspepsia, dysphagia and reflux with a proximal submucosal gastric mass on imaging and endoscopy. She underwent a number of endoscopic and laparoscopic biopsies, which were inconclusive. Suspecting IgG4-RD, steroids were trialled empirically, but did not result in radiological or symptom improvement. Ultimately, she underwent a total gastrectomy, D2 lymphadenectomy and Roux-en-Y oesophago-jejunostomy. On final histopathological analysis of this resection, the diagnosis of gastric IgG4-RD was favoured. Most reports of resection for IgG4-RD in the stomach suggest that it can be treated medically if the level of clinical suspicion is sufficiently high. However, in this case despite clinical suspicion, the initial biopsies were non-diagnostic and empirical steroids failed, demonstrating that these approaches do not always allow avoidance of major surgery for diagnosis and treatment.