Umemura Takeji, Zen Yoh, Hamano Hideaki, Kawa Shigeyuki, Nakanuma Yasuni, Kiyosawa Kendo
Department of Internal Medicine, Division of Hepatology and Gastroenterology, Shinshu University School of Medicine, Matsumoto, Japan.
Hepatology. 2007 Aug;46(2):463-71. doi: 10.1002/hep.21700.
Autoimmune pancreatitis (AIP) is characterized by high serum immunoglobin (Ig) G4 concentrations, lymphoplasmacytic inflammation, and a favorable response to corticosteroid treatment. Since liver dysfunction is frequently seen in AIP patients, we investigated hepatic histopathology and its clinical significance in patients with AIP. We examined the clinical features, histology, and immunoglobin G (IgG)4-bearing plasma cell infiltration of liver biopsies from 17 patients with AIP and 63 patients with either autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis, or chronic viral hepatitis and histological changes in the 7 of 17 livers before and after glucocorticoid therapy. The liver histology of AIP was classified into 5 patterns: evident portal inflammation with or without interface hepatitis (6 cases), large bile-duct obstructive features (8 cases), portal sclerosis (8 cases), lobular hepatitis (5 cases), and canalicular cholestasis (4 cases); some of the histological features coexisted in the same liver. The number of IgG4-bearing plasma cells was significantly higher in AIP patients than controls (P < 0.01), and was significantly correlated with serum IgG4 concentration (P = 0.0014, r = 0.709). Glucocorticoid therapy reduced IgG4-bearing plasma cell infiltration in the liver (P = 0.031) and ameliorated other histological findings. In conclusion, virtually all AIP liver biopsies showed evidence of various pathological changes and infiltration of IgG4-bearing plasma cells. These features were ameliorated by steroid therapy, suggesting that the liver is concurrently affected in AIP, and that liver biopsies can provide significant information in the clinical evaluation and diagnosis of AIP.
自身免疫性胰腺炎（AIP）的特征为血清免疫球蛋白（Ig）G4浓度升高、淋巴浆细胞炎症以及对皮质类固醇治疗反应良好。由于AIP患者常出现肝功能障碍，我们对AIP患者的肝脏组织病理学及其临床意义进行了研究。我们检查了17例AIP患者以及63例自身免疫性肝炎、原发性胆汁性肝硬化、原发性硬化性胆管炎或慢性病毒性肝炎患者肝脏活检的临床特征、组织学及IgG4阳性浆细胞浸润情况，并观察了17例患者中7例在糖皮质激素治疗前后肝脏的组织学变化。AIP的肝脏组织学分为5种类型：有或无界面性肝炎的明显门管区炎症（6例）、大胆管阻塞特征（8例）、门管区硬化（8例）、小叶性肝炎（5例）和胆小管胆汁淤积（4例）；部分组织学特征可在同一肝脏中共存。AIP患者IgG4阳性浆细胞数量显著高于对照组（P<0.01），且与血清IgG4浓度显著相关（P = 0.0014，r = 0.709）。糖皮质激素治疗可减少肝脏中IgG4阳性浆细胞浸润（P = 0.031），并改善其他组织学表现。总之，几乎所有AIP肝脏活检均显示有各种病理变化及IgG4阳性浆细胞浸润的证据。这些特征经类固醇治疗后得到改善，提示AIP中肝脏同时受累，且肝脏活检可为AIP的临床评估和诊断提供重要信息。
