Department of Internal Medicine I, University of Bonn, Bonn, Germany.
Institute of Pathology, University of Bonn, Bonn, Germany.
J Dig Dis. 2024 Jun;25(6):394-403. doi: 10.1111/1751-2980.13295. Epub 2024 Jul 15.
Patients diagnosed with primary sclerosing cholangitis (PSC) but with characteristics of immunoglobulin G4 (IgG4)-associated cholangitis (IAC) have been described. IAC often presents with biliary IgG4-positive plasma cell (IgG4+ PC) infiltration and responds to corticosteroids. In PSC, the frequencies or implications of biliary IgG4+ PC are unknown. We aimed to characterize the phenomenon of biliary IgG4+ PC in patients with an established PSC diagnosis.
Bile duct biopsies from 191 surveillance or therapeutic endoscopic retrograde cholangiography of 58 PSC patients were retrospectively analyzed for IgG4+ PC infiltration. Patients with ≥10 IgG4+ PC per high-power field (HPF) were identified and characterized by clinical parameters, including serum IgG4 and cholangiographic presentations.
Altogether 39.7% of the PSC patients showed ≥10 IgG4+ PC/HPF in bile duct biopsies. Patients with biliary IgG4+ PC infiltration were significantly younger at diagnosis of PSC (P = 0.023). There was no association between biliary IgG4+ PC infiltration and transplant-free survival (P = 0.618). Patients with IgG4+ PC infiltration in bile duct biopsies showed significantly higher baseline (P = 0.002) and maximum (P = 0.001) serum IgG4 compared to those without. Biliary IgG4+ PC infiltration was associated with high-grade bile duct strictures (P = 0.05). IgG4-positive plasma cell infiltrations were found multifocally in 72.7% of this subgroup of PSC patients.
IgG4+ PC ≥10/HPF can be found abundantly in bile duct biopsies in PSC. Histological findings correlated with serum IgG4, age, and high-grade bile duct strictures. IgG4+ PC was located multifocally, hinting at a systemic biliary phenotype.
已描述了诊断为原发性硬化性胆管炎（PSC）但具有免疫球蛋白 G4（IgG4）相关胆管炎（IAC）特征的患者。IAC 常表现为胆管 IgG4 阳性浆细胞（IgG4+ PC）浸润，并对皮质类固醇有反应。在 PSC 中，胆管 IgG4+ PC 的频率或意义尚不清楚。我们旨在描述已确诊 PSC 患者中胆管 IgG4+ PC 的现象。
回顾性分析了 58 例 PSC 患者的 191 例监测或治疗性内镜逆行胰胆管造影术的胆管活检标本，以分析 IgG4+ PC 浸润。通过临床参数（包括血清 IgG4 和胆管影像学表现）鉴定和描述每高倍视野（HPF）≥10 个 IgG4+ PC 的患者。
共有 39.7%的 PSC 患者的胆管活检标本中存在≥10 个 IgG4+ PC/HPF。在诊断为 PSC 时，有胆管 IgG4+ PC 浸润的患者明显更年轻（P=0.023）。胆管 IgG4+ PC 浸润与无移植生存之间无关联（P=0.618）。与无 IgG4+ PC 浸润的患者相比，胆管活检标本中存在 IgG4+ PC 浸润的患者基线（P=0.002）和最大（P=0.001）血清 IgG4 水平显著更高。胆管 IgG4+ PC 浸润与高级别胆管狭窄相关（P=0.05）。在这组 PSC 患者中，72.7%的患者 IgG4 阳性浆细胞浸润呈多灶性分布。
PSC 患者的胆管活检标本中可大量发现 IgG4+ PC≥10/HPF。组织学发现与血清 IgG4、年龄和高级别胆管狭窄相关。IgG4+ PC 呈多灶性分布，提示存在系统性胆管表型。
