Erdogan D, Kloek J J, ten Kate F J W, Rauws E A J, Busch O R C, Gouma D J, van Gulik T M
Department of Surgery, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
Br J Surg. 2008 Jun;95(6):727-34. doi: 10.1002/bjs.6057.
Immunoglobulin (Ig) G(4)-related lymphoplasmacytic sclerosing pancreatitis has been described in the context of autoimmune pancreatitis mimicking distal cholangiocarcinoma. The aim of this study was to assess the occurrence of this entity in benign bile duct strictures in patients resected for presumed hilar cholangiocarcinoma.
Of 185 patients who had undergone resection of proximal bile ducts on suspicion of hilar cholangiocarcinoma between January 1984 and June 2005, 32 (17.3 per cent) had a benign bile duct stricture on histopathological examination. After re-evaluation, further immunohistochemical analysis was performed on specimens from patients with features of autoimmune-like disease.
The periductal stroma in 15 patients showed features of autoimmune-like disease (diffuse, moderate to severe lymphoplasmacytic infiltration with marked fibrosis). Abundant IgG(4)-positive plasma cell infiltration around the bile duct lesions was seen in two of these. Although not significant, patients with features of autoimmune-like disease on histological changes showed a higher incidence of recurrent biliary complications than those without (P = 0.250).
Features of autoimmune-like bile duct disease were seen in almost half (15 of 32) of patients with benign hilar strictures resected for presumed hilar cholangiocarcinoma. Frank IgG(4)-related sclerosing disease was found in only two of the 15 patients with autoimmune-like bile duct disease.
免疫球蛋白（Ig）G4相关淋巴浆细胞性硬化性胰腺炎已在模仿肝门部胆管癌的自身免疫性胰腺炎背景下被描述。本研究的目的是评估在因疑似肝门部胆管癌而接受手术切除的患者的良性胆管狭窄中该实体的发生率。
在1984年1月至2005年6月期间因疑似肝门部胆管癌而接受近端胆管切除的185例患者中，32例（17.3%）在组织病理学检查中发现有良性胆管狭窄。重新评估后，对具有自身免疫样疾病特征的患者的标本进行了进一步的免疫组织化学分析。
15例患者的导管周围间质显示出自身免疫样疾病的特征（弥漫性、中度至重度淋巴浆细胞浸润伴明显纤维化）。其中两例在胆管病变周围可见大量IgG4阳性浆细胞浸润。尽管无统计学意义，但组织学改变具有自身免疫样疾病特征的患者复发性胆道并发症的发生率高于无此特征的患者（P = 0.250）。
在因疑似肝门部胆管癌而接受手术切除的良性肝门部狭窄患者中，几乎一半（32例中的15例）可见自身免疫样胆管疾病的特征。在15例具有自身免疫样胆管疾病特征的患者中，仅两例发现有明确的IgG4相关硬化性疾病。
