Ohara Hirotaka, Nakazawa Takahiro, Ando Tomoaki, Joh Takashi
Department of Internal Medicine and Bioregulation, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, Aichi, 467-8601, Japan.
J Gastroenterol. 2007 May;42 Suppl 18:15-21. doi: 10.1007/s00535-007-2045-9.
Autoimmune pancreatitis (AIP) is frequently associated with sclerosing cholangitis (SC). SC with AIP has a cholangiographic appearance that is often confused with primary sclerosing cholangitis (PSC) but only the former responds well to corticosteroid therapy. Detailed study of cholangiographic findings allows discrimination of SC with AIP from PSC. Band-like strictures, a beaded or pruned-tree appearance, and diverticulum-like outpouching were significantly more frequently observed in cases of PSC. In contrast, segmental strictures, dilation after confluent stricture, and strictures of the lower common bile duct were significantly more common in SC with AIP. The other systemic extrapancreatic lesions associated with AIP found in the literature were Sjögren's syndrome, ulcerative colitis, retroperitoneal fibrosis, sialadenitis, thyroiditis, and idiopathic thrombocytopenic purpura. In a comparison of the clinical course and laboratory data of our cases, gamma-globulin, IgG, and IgG4 levels were significantly higher in patients with AIP with systemic extrapancreatic lesions than those without them. In our immunohistochemical study, marked infiltration of IgG4+ plasma cells was frequently observed in the pancreas, liver, bile duct, and salivary glands of the AIP patients examined. In contrast, the degree of infiltration of IgG4+ plasma cells around the bile duct in the portal areas and the extrahepatic bile duct with PSC was significantly lower than with AIP. These results also suggest that AIP is a disease state clearly different from PSC. In addition, the normal epithelia of the pancreatic ducts, bile ducts, gallbladder, and salivary gland ducts reacting with the patients' sera was detectable by the anti-IgG4 antibody. Therefore, AIP may also affect extrapancreatic organs, and the sera of AIP patients may contain an IgG4 autoantibody to various organs.
自身免疫性胰腺炎（AIP）常与硬化性胆管炎（SC）相关。AIP合并的SC在胆管造影上的表现常与原发性硬化性胆管炎（PSC）混淆，但只有前者对皮质类固醇治疗反应良好。对胆管造影结果的详细研究有助于区分AIP合并的SC与PSC。PSC病例中更常观察到带状狭窄、串珠状或修剪树枝状外观以及憩室样突出。相比之下，节段性狭窄、汇合性狭窄后的扩张以及胆总管下段狭窄在AIP合并的SC中更为常见。文献中发现的与AIP相关的其他全身性胰腺外病变有干燥综合征、溃疡性结肠炎、腹膜后纤维化、涎腺炎、甲状腺炎和特发性血小板减少性紫癜。在对我们病例的临床病程和实验室数据进行比较时，有全身性胰腺外病变的AIP患者的γ-球蛋白、IgG和IgG4水平显著高于无此类病变的患者。在我们的免疫组织化学研究中，在所检查的AIP患者的胰腺、肝脏、胆管和唾液腺中经常观察到IgG4 +浆细胞的明显浸润。相比之下，PSC患者门静脉区胆管周围和肝外胆管中IgG4 +浆细胞的浸润程度明显低于AIP患者。这些结果也表明AIP是一种与PSC明显不同的疾病状态。此外，通过抗IgG4抗体可检测到胰腺导管、胆管、胆囊和唾液腺导管的正常上皮与患者血清发生反应。因此，AIP也可能影响胰腺外器官，AIP患者的血清可能含有针对各种器官的IgG4自身抗体。
