Immunoglobulin G4-related disease (IgG4-RD) affecting the esophagus, stomach, and liver.

A 60-year-old man, with occasional acid reflux, was found on computed tomography (CT) to have multiple masses in his esophagus (●" Fig.1a), stomach (●" Fig.1 d), and liver. He had a history of partial hepatectomy for inflammatory liver pseudotumor on two occasions, 22 and 9 years earlier. Physical examination revealed no superficial lymph node. Blood test showed that hemoglobin was 105g/L, and the tumor biomarkers CEA, AFP and CA199 were normal. Gastroscopy revealed a hard, fixed mass in the lower esophagus covered with normal appearing mucosa (●" Fig.1b), and a giant stomach ulcer with a clean andhard base (●" Fig.1e). Histological examination showed abundant lymphocytes, plasma cell infiltration, and fibrosis in biopsies from the esophagus (●" Fig.1c), and stomach (●" Fig.1 f). The number of IgG4 positive cells was greater than 50 per high-power field (hpf) (●" Fig. 2a,b), and the ratio of IgG4-positive to IgG-positive cells was greater than 30% in both specimens. Meanwhile, similar results were found in the inflammatory liver pseudotumor excised 9 years earlier (●" Fig.2c,d). Moreover, the concentration of serum IgG4 was 1590mg/L (normal range: 80–1400mg/L). Therefore, the patient was diagnosed as having immunoglobulin G4-related disease (IgG4-RD). After a 3-month period of steroid therapy and anti-ulcer treatment, fresh epithelial tissue had regenerated in the margin of the stomach ulcer and the stomach wall had become softer (●" Fig.3b). Neither regression nor progression of the mass in the esophagus were observed by endoscopy (●" Fig.3a), or CTscanning. IgG4-RD is an autoimmune disorder characterized by IgG4 positive plasma cell infiltration, fibrosis, phlebitis, and increased serum IgG4 [1]. IgG4-RD always mimics malignancy clinically and responds to steroids [2]. Many cases of IgG4-RD affecting extrapancreatic organs have been reported [3]. To the best of our knowledge, this is the first documented case of IgG4RD affecting the esophagus, stomach, and liver. It is highlighted that IgG4-RD should be considered as a possible diagnosis for multiple masses in the gastrointestinal tract, which may avoid unnecessary surgery or chemotherapy.