IgG4相关性胆胰疾病的诊断和治疗

近年来发现,IgG4相关性疾病可累及全身多系统多器官,目前研究提示IgG4相关性胆胰疾病主要有IgG4相关性硬化性胆管炎和自身免疫性胰腺炎（AIP）.前者是一种免疫发病机制未明、生化学特点以及胆管影像学表现与原发性硬化性胆管炎（PSC）相似,对糖皮质激素应答良好的疾病.后者临床表现为复发性胰腺炎、梗阻性黄疸,伴胆管、涎腺等其他器官受累.实验室检查血清和组织学IgG4明显升高.影像学表现为胰腺呈“腊肠样”外观,伴主胰管弥漫不规则狭窄,同样对激素治疗敏感.二者常同时受累、关系密切,易误诊为胆管癌、硬化性胆管炎或胰腺癌,本文将当前国内外研究现状做一介绍,供临床诊治参考.

Diagnosis and Treatment of IgG4-related Biliary and Pancreatic Diseases

In recent years, IgG4-related systemic disease has been found involving multiple organ and systems. And current studies suggest that IgG4-related biliary and pancreatic diseases are mainly IgG4-related sclerosing cholangitis and autoimmune pancreatitis （AIP）. The former is an immune pathogenesis unknown, its biochemical characteristics and bile duct imaging similar to primary sclerosing cholangitis （PSC）, but it has good response to glucocorticoids. The latter clinical manifestations of recurrent pancreatitis, obstructive jaundice, bile duct, salivary gland and other organs involvement. Laboratory and histological examination of serum IgG4 increased significantly. Pancreatic imaging was sausage-like appearance, with diffuse irregular main pancreatic duct stenosis, also sensitive to hormone therapy. These two diseases often closely involved, can be misdiagnosed as cholangiocarcinoma, sclerosing cholangitis, or pancreatic cancer, this article will make an introduction research status at home and abroad for the clinical diagnosis and treatment information.