Immunoglobin G4-hepatopathy: Association of immunoglobin G4-bearing plasma cells in liver with autoimmune pancreatitis
Immunoglobin G4-hepatopathy: Association of immunoglobin G4-bearing plasma cells in liver with autoimmune pancreatitis
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DOI：
10.1002/hep.21700
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发表时间：
2007-08-01
期刊：
影响因子：
13.5
通讯作者：
Kiyosawa, Kendo
中科院分区：
文献类型：
--
作者：
Umemura, Takeji;Zen, Yoh;Kiyosawa, Kendo
自身免疫性胰腺炎 (AIP) 的特点是血清免疫球蛋白 (1g) G4 浓度高、淋巴浆细胞炎症以及对皮质类固醇治疗的良好反应。由于肝功能障碍在 AIP 患者中常见，因此我们研究了 AIR 患者的肝脏组织病理学及其临床意义。我们检查了 17 名 AIP 患者和 63 名患有自身免疫性肝炎、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的患者的临床特征、组织学和肝活检中携带免疫球蛋白 G (IgG)4 的浆细胞浸润，以及 17 名患者中 7 名的组织学变化糖皮质激素治疗前后的肝脏。 AIP的肝脏组织学分为5种类型：明显的门静脉炎症伴或不伴界面性肝炎（6例）、大胆管梗阻特征（8例）、门静脉硬化（8例）、小叶性肝炎（5例）和小管胆汁淤积（4例）；一些组织学特征同时存在于同一肝脏中。 AIP患者携带IgG4的浆细胞数量显着高于对照组（P < 0.01），且与血清IgG4浓度显着相关（P = 0.0014，r = 0.709）。糖皮质激素治疗减少了肝脏中携带 IgG4 的浆细胞浸润（P = 0.031），并改善了其他组织学结果。总之，几乎所有 AIP 肝活检均显示各种病理变化和 IgG4 浆细胞浸润的证据。这些特征通过类固醇治疗得到改善，表明 Alp 患者的肝脏同时受到影响，肝活检可以为 AIP 的临床评估和诊断提供重要信息。
Autoinumme pancreatitis (AIP) is characterized by high serum immunoglobin (1g) 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 AIR 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 Alp, and that liver biopsies can provide significant information in the clinical evaluation and diagnosis of AIP.