Ileocecal IgG4-Related Disease: A Case Report Mimicking Malignancy

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a chronic mass-forming inflammatory disease characterized by fibroblastic proliferation and mixed inflammatory cell infiltration. IgG4-RD can involve one or multiple organs, and the most commonly affected organs include the pancreas, salivary glands, and the orbit. We present a case of a 66-year-old man, with a history of sarcoidosis, who presented with an obstructing ileocecal mass highly suspicious for malignancy. After surgical resection and pathological and serological evaluation, a diagnosis of IgG4-RD was rendered. In the absence of other manifestations, preoperative diagnosis is challenging, and IgG4-RD may continue to be a diagnostic pitfall.

Imaging findings of immunoglobulin G4-related disease: from the head to the pelvis

“…Gastrointestinal tract lesions in IgG4-RD have been reported sporadically in the stomach and duodenum, and small bowel lesions are extremely rare. (15) The lesions can cause wall thickening and ulcer formation; therefore, they often mimic malignancy (Fig. 15a).…”

“…(15) IgG4-related gastritis causes epigastric pain, anorexia, nausea or abdominal discomfort, while IgG4related enteritis may cause small bowel obstruction. (15) IgG4-related sclerosing mesenteritis is a rare entity characterised by focal or diffuse fibrosis and inflammation in the small bowel mesentery that can appear as a soft tissue mass. On Fig.…”