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自身免疫性胰腺炎(AIP)是逐渐被认识的慢性胰腺炎的一种类型,与其他类型的慢性胰腺炎有明显的不同。随着2001年发现AIP患者血清IgG4含量明显增高,这个现实被广泛的接受。1型AIP与2型AIP不同。2%的慢性胰腺炎为1-AIP,成人男性患者居多。患者通常由于胰头的增大或胆管壁增厚所致梗阻性黄疸,通过血清学、影像学和组织学与胰腺癌鉴别。血清IgG4水平的增高是最为敏感和特异性的表现。影像学的表现为胰管的不规则狭窄,胰腺弥散性或局灶性的扩大及胰周可见环形的囊壳样影,以及影像延迟期强化。组织学显示大量的小叶间质表现为致密的纤维化伴席纹状、炎细胞浸润,闭塞性静脉炎和IgG4阳性细胞数量增加。激素治疗疗效明显是另外的特征,在初治的2~3周血清学和影像学明显改善。基于同步或不同步的伴有多器官的损伤,1-AIP也被认为是一种IgG4相关全身性疾病。至今有一些与1-AIP相关的自身免疫性抗原被鉴定,但IgG4在此病中的作用是肯定的。
Autoimmune pancreatitis (AIP) is a type of chronic pancreatitis that is gradually recognized and is significantly different from other types of chronic pancreatitis. With the 2001 discovery of serum IgG4 levels in patients with AIP increased significantly, this reality is widely accepted. Type 1 AIP differs from Type 2 AIP. 2% of chronic pancreatitis is 1-AIP, the majority of adult male patients. Patients with obstructive jaundice, usually due to enlargement of the head of the pancreas or thickening of the bile duct wall, are differentiated from pancreatic cancer by serology, radiology and histology. Increased serum IgG4 levels are the most sensitive and specific manifestation. Radiographic manifestations of irregular narrow pancreatic duct, pancreatic diffuse or focal expansion and pancreatic peripancreatular cysts can be seen, and delayed imaging enhancement. Histology showed a large number of interlobular interstitial showed dense fibrotic striated pattern, inflammatory cell infiltration, occlusive phlebitis and IgG4-positive cells increased. Obviously, the curative effect of hormonal therapy is another characteristic. Serum and radiology have been obviously improved 2 to 3 weeks after initial treatment. 1-AIP is also considered to be an IgG4-related systemic disease based on simultaneous or unsynchronized damage with multiple organs. To date, a number of autoimmune antigens associated with 1-AIP have been identified, but the role of IgG4 in this disease is confirmed.