自身免疫性胰腺炎的病理学诊断

孙柯

doi:10.12449/JCH240606

自身免疫性胰腺炎的病理学诊断

DOI: 10.12449/JCH240606

孙柯^, ,

浙江大学医学院附属第一医院病理科，杭州 310003

作者贡献声明：本文不存在任何利益冲突。

详细信息

通信作者:
孙柯， 1200035@zju.edu.cn （ORCID： 0000-0002-3789-3143）

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出版历程
- 收稿日期: 2024-04-07
- 录用日期: 2024-05-27
- 出版日期: 2024-06-25

Pathological diagnosis of autoimmune pancreatitis

Ke SUN^{,
,}

Department of Pathology，The First Affiliated Hospital，Zhejiang University School of Medicine，Hangzhou 310003，China

More Information

Corresponding author: SUN ke， 1200035@zju.edu.cn （ORCID： 0000-0002-3789-3143）

摘要

摘要: 自身免疫性胰腺炎（AIP）是一种少见疾病，其诊断应基于对临床、影像学、血清学和病理学结果全面评估。目前将AIP分为两型：1型AIP被认为是与IgG4相关疾病的胰腺表现；2型AIP被视为与IgG4无关的胰腺特异性疾病。尽管1型和2型AIP的发病机制似乎不同，但二者的影像学表现相似，且均对类固醇激素有良好反应。本综述主要关注于AIP 2个亚型的病理组织学特征，尤其是内镜超声引导的细针穿刺活检组织的病理诊断难点，以提高AIP临床诊断的准确性。
- 自身免疫性胰腺炎 /
- 活组织检查 /
- 病理学 /
- 诊断
Abstract: Autoimmune pancreatitis （AIP） is a rare disease， and its diagnosis should be made based on a comprehensive evaluation of clinical， radiological， serological， and pathological findings. At present， AIP is classified into two subtypes of type 1 （identified as the pancreatic manifestation of IgG4-related disease） and type 2 （identified as the pancreas-specific disorder independent of IgG4）. Although type 1 and type 2 AIP seem to have different pathogeneses， they tend to have similar radiological findings and exhibit a good response to corticosteroid therapy. This article mainly reviews the histopathological features of the two subtypes of AIP， especially the diagnostic challenges encountered in the interpretation of specimens obtained through endoscopic ultrasound-guided fine needle aspiration/biopsy， to as to help pathologists enhance the accuracy of the diagnosis of AIP.
- Autoimmune Pancreatitis /
- Biopsy /
- Pathology /
- Diagnosis

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图 1 1型AIP病理学特征

注： a，胰腺导管周围厚袖套状淋巴浆细胞浸润，导管上皮保存完好（HE染色，×200）；b，席纹状纤维化（HE染色，×200）；c，闭塞性静脉炎呈炎性结节样改变（HE染色，×200）；d，闭塞性静脉炎（弹力纤维染色，×200）；e，胰腺导管周围大量IgG4阳性的浆细胞（IgG4免疫组化染色，×200）；f，胰腺导管周围大量IgG阳性的浆细胞（IgG免疫组化染色，×200）。

Figure 1. Pathological features of type 1 AIP

下载: 全尺寸图片幻灯片

图 2 2型AIP病理学特征（HE染色，×200）

注： a，中性粒细胞浸润导管上皮，管腔内脓肿形成（粒细胞上皮病变）；b，经皮胰腺穿刺组织中性粒细胞浸润导管上皮（粒细胞上皮病变）；c，经皮胰腺穿刺组织胰腺腺泡导管化生伴多量中性粒细胞浸润。

Figure 2. Pathological features of type 2 AIP （HE staining，×200）

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表 1 1型AIP和2型AIP的临床特征对比

Table 1. Comparison of clinical features between type 1 AIP and type 2 AIP

项目	1型AIP	2型AIP
平均发病年龄	60岁（一般>40岁）	30岁（包括儿童）
男性占比（%）	80	50
血清IgG4水平升高（%）	80～90	10
其他器官累及（%）	50	0
合并发生炎症性肠病（%）	<5	40
主要临床表现
腹痛/急性胰腺炎（%）	10	60
无痛性黄疸（%）	80	30
对激素的反应（%）	～100	～100
复发（%）	30～50	<10

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表 2 ICDC中1型和2型AIP的组织学诊断标准

Table 2. Histological diagnostic criteria for type 1 and type 2 AIP in ICDC

项目	诊断标准
1型AIP
1级	LPSP（空芯针活检/切除），至少符合以下3条：（1）导管周围淋巴浆细胞浸润，无粒细胞；（2）闭塞性静脉炎；（3）席纹状纤维化；（4）（>10个/HPF）IgG4阳性浆细胞
2级	LPSP（空芯针活检），符合以下任何2条：（1）导管周围淋巴浆细胞浸润，无粒细胞浸润；（2）闭塞性静脉炎；（3）席纹状纤维化；（4）丰富（>10个/HPF）IgG4阳性浆细胞
2型AIP
1级	IDCP（空芯针活检/切除），包括以下2条：（1）中性粒细胞浸润导管壁伴或不伴腺泡内中性粒细胞浸润；（2）IgG4阳性浆细胞缺乏或少量（0～10个/HPF）
2级	IDCP（空芯针活检/切除），包括以下2条：（1）腺泡内中性粒细胞和淋巴浆细胞浸润；（2）IgG4阳性浆细胞缺乏或少量（0～10个/HPF）
注：HPF，高倍镜。

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表 3 ADM和高分化导管腺癌的鉴别

Table 3. Identification of ADM and well-differentiated ductal adenocarcinoma

项目	ADM	PDAC
带间质的腺体分布	只位于小叶内	腺癌浸润，小叶结构消失或者腺癌浸润至小叶间质
腺体形状	管腔和腺体轮廓不清	低倍即可见明显的腺体
促结缔组织反应	无	有
游离细胞簇
数量	少量	多量
形状	疏松连接的细胞簇	明显的腺体和细胞栅栏排列
核形状	小、圆、规则	增大、染色质深，大小和形状不规则

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表 4 1型AIP和2型AIP的组织学改变对比

Table 4. Comparison of histological changes between type 1 AIP and type 2 AIP

项目	1型AIP	2型AIP
炎症模式	纤维炎症累及胰管、小叶、静脉、胆总管	纤维炎症主要累及胰管以及胰腺内胆总管，小叶和静脉不明显
浸润	明显淋巴浆细胞浸润，常伴随嗜酸性粒细胞，少量中性粒细胞	多量淋巴浆细胞浸润，明显的中性粒细胞浸润中等及小导管、腺泡
胰管	致密性导管周围炎症，导管上皮保存完整	致密性导管周围炎症、中性粒细胞浸润毁损导管上皮（粒细胞上皮病变）
胰管内蛋白栓和结石	无	无
小叶	淋巴浆细胞浸润，累及替换腺泡组织	淋巴浆细胞斑片状浸润，常混合中性粒细胞
静脉	闭塞性静脉炎	闭塞性静脉炎罕见
动脉	明显的动脉炎症累及罕见	无动脉炎症累及
胰腺周围脂肪组织	纤维炎症过程可能延伸至胰腺周围区域	炎症通常局限于胰腺内
IgG4免疫组化	丰富（>10个/HPF）IgG4⁺浆细胞	少量或无IgG4⁺浆细胞

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