IgG4-related disease as a rare cause of gastric outlet obstruction: a case report and literature review.

ABSTRACT

BACKGROUND: IgG4-related disease involvement of the digestive tract is very rare. In few reported cases of isolated gastric/duodenal IgG4-related disease, none of which resulted in luminal obstruction. CASE PRESENTATION A 59 years old female presented with longstanding gastrointestinal symptoms. CT showed mural thickening of the proximal duodenum. Gastroscopy showed antral ulcer extending into the duodenum with outlet obstruction and biopsy showed acute on chronic duodenitis. Whipple's procedure was performed and IgG4-related disease was diagnosed on final pathology. Symptoms were revolved on mycophenolate mofetil and prednisone with no recurrence. CONCLUSIONS: Our case is the only reported case with gastric outlet obstruction secondary to gastroduodenal IgG4-related disease. The diagnosis should be considered in the differential diagnosis of unexplained duodenal stricture, gastric outlet obstruction or gastrointestinal ulceration. IgG4-related disease usually responds to steroids but long-term response rates to steroid-sparing agents, especially in the subset of patients with luminal IgG4-related disease remains to be determined.