Pathomorphological features of ІgG4-releated sclerosing disease of the stomach: case report

Abstract

Since the first description in the middle of the 1990’s of last century, it has been steadily increasing the number of literary data concerning IgG4-related disease. The process involved more organs and tissues with predominance of pancreas, salivary glands, and  lymph nodes affection. There are several documented cases arising in the gastrointestinal tract. The salient clinical features include a predisposition to affect middle-age men who present with the only symptoms referable to a mass lesion at one or more sites, without inflammation, hyperemia and other constitutional symptoms. Common laboratory features are elevated serum globulin, IgG, IgG4, and IgE subtypes and low titers of autoantibodies such as rheumatoid factor and ANA. Histological features vary depending on the affected organ. But there are some common features of extranodal sites: lymphoplasmacytic infiltration with lymphoid follicle formation, sclerosis, phlebitis, and significant tissue IgG4+ -plasmocytosis. The arteries usually are not involved in the inflammatory process. The most common features in the lymph nodes are follicular hyperplasia, interfollicular expansion and Castelman-like appearance. There are proposed by Cheuk and Chan criteria for the diagnosis such as >50 IgG4 plasma cells per HPF and IgG4+ / IgG+>40 % ratio. The calculation is based on the assessment of the 3 fields of view with the greatest color intensity and correlates with the area of 0.196 mm2 . The pancreas, salivary glands, hepatobiliary tract, orbit and lymph nodes are cites most frequently, although other sites such as the aorta, skin, central nervous system, breast, kidney, prostate, lung, and thyroid have also been occasionally reported. There are 6 cases reports of involving the GIT, 2 of them were described in the stomach, one case in esophagus, jejune, cecum and sigmoid colon. Most cases took the form of a circumscribed nodule, variably affecting the mucosa, submucosa, muscularis propria, and subserosa. Microscopically it was formed by paucicellular hyalinized fibrous tissue with patchy chronic inflammatory infiltrate compound with plasma cells and lymphocytes. The proposed by Cheuk and Chan criteria were present in 4 of these cases. In the case with involving the jejune there were diagnosed serum IgG4 elevation at 800 mg/dL, phlebitis and necrotizing mesenteric arteritis. IgG4+ plasma cells also were seen within the interfollicular region of mesenteric lymph nodes. In this report, we present the case of IgG4-related disease involving stomach, omentum and mesenteric lymph nodes. A previously well 29-year-old man presented with a 1-year history of intermittent epigastric pain, nausea and sometimes vomiting. Because of worsening and suspicion of cancer resection of stomach was made. Morphological investigation of stomach tissue revealed a chronic ulcer associated with transmural inflammatory infiltrate rich in IgG4-positiv plasma sells. Abundant IgG4+ interfollicular plasma cell were also identified in a lymph node in stomach serosa, lymph nodes of omentum and mesenteric lymph node. In view of sensitivity to steroids, this condition should be considered in the differential diagnosis of stomach ulcers and gastritis with severe plasma cell infiltration, especially in case of negative H. Pylori and absence of response to ordinary anti-inflammatory treatment. Our observation of IgG4-releated sclerosing disease of the stomach in 29-year-old male was carried out in context of modern information about IgG4-releated pathology of different organs. Data of clinical, laboratory, and pathomorphological studies which help to substantiate the diagnosis were presented.