Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan.
J Gastroenterol. 2011 Feb;46(2):269-76. doi: 10.1007/s00535-010-0319-0. Epub 2010 Sep 7.
IgG4-related sclerosing cholangitis (IgG4-SC) needs to be differentiated from primary sclerosing cholangitis (PSC). In this study, we performed a retrospective study to reveal cases in which liver needle biopsy was useful for differential diagnosis.
Nineteen patients with IgG4-SC and 22 patients with PSC were studied. All patients underwent endoscopic retrograde cholangiography and liver needle biopsy. We defined small bile duct involvement of IgG4-SC histologically as damage to the small bile duct associated with infiltration of ≥10 IgG4+ plasma cells per high power field (HPF). Clinicopathological characteristics were compared between IgG4-SC patients with and without small bile duct involvement.
Small bile duct involvement was observed in 5 (26%) of the patients with IgG4-SC. Patients with small bile duct involvement showed a higher incidence of intrahepatic biliary strictures on cholangiography (80 vs. 21%, p = 0.038). Conversely, 4 of 7 (57%) patients with intrahepatic biliary strictures on cholangiography had histologically evident small duct involvement. The number of IgG4+ plasma cells was significantly correlated with the site of the most proximal stricture on cholangiograms (p = 0.021). The number of IgG4+ plasma cells per HPF was significantly higher in IgG4-SC patients with intrahepatic biliary strictures than in those with PSC (13.4 vs. 0.4 cells/HPF, p < 0.001).
Involvement of small bile ducts is more frequent in patients with intrahepatic biliary strictures on cholangiography, and liver needle biopsy is especially useful for these patients.
IgG4 相关硬化性胆管炎（IgG4-SC）需要与原发性硬化性胆管炎（PSC）相鉴别。本研究通过回顾性研究揭示了肝穿刺活检有助于鉴别诊断的病例。
研究纳入 19 例 IgG4-SC 患者和 22 例 PSC 患者。所有患者均接受了内镜逆行胰胆管造影和肝穿刺活检。我们将 IgG4-SC 的小胆管受累定义为小胆管损伤伴有≥10 个高倍镜视野（HPF）的 IgG4+浆细胞浸润。比较了 IgG4-SC 患者中小胆管受累与无小胆管受累患者的临床病理特征。
5 例（26%）IgG4-SC 患者存在小胆管受累。小胆管受累患者胆管造影显示肝内胆管狭窄的发生率更高（80% vs. 21%，p = 0.038）。相反，胆管造影显示肝内胆管狭窄的 7 例患者中有 4 例存在组织学上明显的小胆管受累。胆管造影最狭窄部位的 IgG4+浆细胞数量与胆管造影最狭窄部位的 IgG4+浆细胞数量显著相关（p = 0.021）。肝内胆管狭窄的 IgG4-SC 患者的 IgG4+浆细胞/HPF 计数明显高于 PSC 患者（13.4 对 0.4 细胞/HPF，p < 0.001）。
胆管造影显示肝内胆管狭窄的患者小胆管受累更为常见，肝穿刺活检对这些患者尤其有用。
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