Frydman James, Grunner Shahar, Kluger Yoram
James Frydman, Shahar Grunner, Yoram Kluger, Department of General Surgery, Rambam Health Care Campus, Haifa 31096, Israel.
World J Gastroenterol. 2014 Nov 28;20(44):16782-5. doi: 10.3748/wjg.v20.i44.16782.
IgG4-related disease is a recently recognized entity linked initially to autoimmune pancreatitis and has been subsequently described in nearly every organ system. Men over the age of 50 represent the most affected demographic group and a comprehensive set of diagnostic criteria has been developed to aid treating clinicians. Though elevated levels of IgG4 in the serum are suggestive of the disease, definitive diagnosis is made on histopathology. Treatment is tailored to the clinical presentation with corticosteroid therapy known to have proven efficacy. Gastric manifestations of the IgG4-related disease primarily come in two varieties, notably chronic ulceration or pseudotumor formation. Autoimmune pancreatitis conveys increased risk for IgG4-related disease of the stomach, which is independent of Helicobacter pylori status. In this case report, we present an acute gastric-pericardial fistula secondary to IgG4-related disease that required urgent operative management. To our knowledge, this is the first report in the medical literature describing this complication of IgG4-related disease.
IgG4相关性疾病是一种最近才被认识的疾病实体，最初与自身免疫性胰腺炎相关，随后几乎在每个器官系统中都有描述。50岁以上的男性是受影响最严重的人群，已经制定了一套全面的诊断标准来帮助临床医生进行治疗。虽然血清中IgG4水平升高提示该疾病，但确诊需依靠组织病理学检查。治疗方案根据临床表现进行调整，已知皮质类固醇疗法具有确切疗效。IgG4相关性疾病的胃部表现主要有两种类型，即慢性溃疡或假瘤形成。自身免疫性胰腺炎会增加胃部发生IgG4相关性疾病的风险，这与幽门螺杆菌感染状态无关。在本病例报告中，我们呈现了一例继发于IgG4相关性疾病的急性胃心包瘘，需要紧急手术治疗。据我们所知，这是医学文献中首次描述IgG4相关性疾病的这种并发症。
