IgG4-Related Gastrointestinal Lesion
IgG4-related disease has recently been recognized as a systemic condition that is characterized by increased serum IgG4 levels and responsiveness to steroid therapy. Patients with IgG4-RD show organ enlargement or nodular lesions with abundant lymphocyte
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Satomi Koizumi, Terumi Kamisawa, and Sawako Kuruma
Introduction IgG4-related disease has recently been recognized as a systemic condition that is characterized by increased serum IgG4 levels and responsiveness to steroid therapy. Patients with IgG4-RD show organ enlargement or nodular lesions with abundant lymphocyte and IgG4-positive plasma cell infiltration and fibrosis, and they frequently present both clinically and radiologically with findings suggestive of malignancy, which may result in unnecessary resection [1– 3]. According to the comprehensive clinical diagnostic criteria for IgG4-RD [4], the diagnosis of IgG4-RD is made in the presence of a characteristic diffuse/localized swelling or mass in one or more organs with increased serum IgG4 levels or histological findings of abundant IgG4-positive plasma cell and lymphocyte infiltration associated with fibrosis. Although there have been reports of infiltration of many IgG4-positive plasma cells in the gastric mucosa, colonic mucosa, and major duodenal papilla of some AIP patients [5–11], whether they are the lesions involved in IgG4-RD is unclear. This chapter reviews the published literature about the relationships between IgG4 and gastrointestinal diseases such as esophagitis, gastritis, colitis, and duodenal papillitis with abundant IgG4-positive plasma cell infiltration to clarify IgG4-related gastrointestinal disease.
IgG4-Related Esophageal Lesions There have been two case reports of IgG4-related esophageal disease, which presented with dysphagia, weight loss, and recurrent esophageal strictures [12, 13]. On endoscopy, wall thickening was observed, and endoscopic ultrasound showed a submucosal lesion mass in one case. Since the S. Koizumi, MD • T. Kamisawa, MD, PhD (*) • S. Kuruma, MD Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan e-mail: [email protected]; [email protected]
possibility of malignancy and gastrointestinal stromal tumor could not be ruled out, esophageal resection was performed in both cases. Gross examination showed esophageal submucosal strictures with wall thickening. On histological examinations, transmural chronic fibrotic inflammation with abundant IgG4-positive plasma cell and lymphocyte infiltration and phlebitis were observed. The postoperative serum IgG4 level was 138 mg/dL in one case. Both cases showed no evidence of other IgG4-RD, including AIP, but they met the criteria of IgG4-RD. Thus, both cases were considered to show esophageal manifestations of IgG4-RD and could be called IgG4-related esophagitis. If unexplained esophageal stricture mimicking malignancy is seen, IgG4-related esophagitis must be considered in the differential diagnosis to avoid unnecessary surgery.
IgG4-Related Gastric Lesions There have been nine cases in seven reports of IgG4-related gastric disease [14–20]. Seven cases were presented with abdominal symptoms like discomfort (n = 2), abdominal pain (n = 1), appetite loss (n = 1), anorexia (n = 1), weight loss (
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