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

 

 Permissions and Reprints

A 60-year-old man, with occasional acid reflux, was found on computed tomography (CT) to have multiple masses in his esophagus ([Fig. 1 a]), 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 105 g/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. 1 b]), and a giant stomach ulcer with a clean and hard base ([Fig. 1 e]). Histological examination showed abundant lymphocytes, plasma cell infiltration, and fibrosis in biopsies from the esophagus ([Fig. 1 c]), and stomach ([Fig. 1 f]). The number of IgG4 positive cells was greater than 50 per high-power field (hpf) ([Fig. 2 a, 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. 2 c, d]). Moreover, the concentration of serum IgG4 was 1590 mg/L (normal range: 80 – 1400 mg/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. 3 b]). Neither regression nor progression of the mass in the esophagus were observed by endoscopy ([Fig. 3 a]), or CT scanning.

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 IgG4-RD 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.

Endoscopy_UCTN_Code_CCL_1AB_2AC_3AB

• References

• 1 Smyrk TC. Pathological features of IgG4-related sclerosing disease. Curr Opin Rheumatol 2011; 23: 74-79
  CrossrefPubMedGoogle Scholar
• 2 Morse B, Centeno B, Vignesh S. Autoimmune pancreatitis: updated concepts of a challenging diagnosis. Am J Med 2014; 127: 1010.e1-1010.e9
  CrossrefPubMedGoogle Scholar
• 3 Kim JH, Byun JH, Lee SS et al. Atypical manifestations of IgG4-related sclerosing disease in the abdomen: imaging findings and pathologic correlations. AJR Am J Roentgenol 2013; 200: 102-112
  CrossrefPubMedGoogle Scholar