Primary sclerosing cholangitis with IgG4-positive plasma cells in bile duct biopsies ― Frequency and characterization

IF 2.3 3区医学 Q3 GASTROENTEROLOGY & HEPATOLOGY

Journal of Digestive Diseases Pub Date : 2024-07-15 DOI:10.1111/1751-2980.13295

Taotao Zhou, Florian Fronhoffs, Glen Kristiansen, Leona Dold, Dominik J. Kaczmarek, Christian P. Strassburg, Tobias J. Weismüller

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引用次数: 0

Abstract

Objectives

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.

Methods

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.

Results

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.

Conclusions

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.

Abstract Image

查看原文本刊更多论文

胆管活检中出现 IgG4 阳性浆细胞的原发性硬化性胆管炎--频率和特征。

目的：有患者被诊断为原发性硬化性胆管炎（PSC），但却具有免疫球蛋白 G4（IgG4）相关性胆管炎（IAC）的特征。IAC 通常表现为胆道 IgG4 阳性浆细胞（IgG4+ PC）浸润，并对皮质类固醇有反应。在 PSC 中，胆汁 IgG4+ PC 的频率或影响尚不清楚。我们的目的是描述已确诊为 PSC 患者的胆道 IgG4+ PC 现象：我们对 58 例 PSC 患者的 191 例监测性或治疗性内镜逆行胆管造影的胆管活检进行了回顾性分析，以确定是否存在 IgG4+ PC 浸润。对每个高倍视野（HPF）IgG4+ PC ≥10 例的患者进行鉴定，并根据临床参数（包括血清 IgG4 和胆管造影表现）对其进行特征描述：结果：总共有 39.7% 的 PSC 患者在胆管活检中发现 IgG4+ PC≥10 个/HPF。有胆道 IgG4+ PC 浸润的患者在确诊为 PSC 时明显更年轻（P = 0.023）。胆道 IgG4+ PC 浸润与无移植生存率之间没有关联（P = 0.618）。胆管活检中出现 IgG4+ PC 浸润的患者的血清 IgG4 基线（P = 0.002）和最高值（P = 0.001）均明显高于未出现 IgG4 浸润的患者。胆道 IgG4+ PC 浸润与高级别胆管狭窄有关（P = 0.05）。在这一亚组PSC患者中，72.7%的患者发现IgG4阳性浆细胞多灶性浸润：结论：在 PSC 患者的胆管活检中可发现大量 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分布在多个部位，提示存在系统性胆道表型。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Digestive Diseases 医学-胃肠肝病学

CiteScore

5.40

自引率

2.90%

发文量

审稿时长

6-12 weeks

期刊介绍： The Journal of Digestive Diseases is the official English-language journal of the Chinese Society of Gastroenterology. The journal is published twelve times per year and includes peer-reviewed original papers, review articles and commentaries concerned with research relating to the esophagus, stomach, small intestine, colon, liver, biliary tract and pancreas.