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中华胃肠内镜电子杂志 ›› 2023, Vol. 10 ›› Issue (01) : 46 -51. doi: 10.3877/cma.j.issn.2095-7157.2023.01.013

论著

自身免疫性胰腺炎临床特征分析
陈升鑫1, 翟亚奇1, 卜保国1, 陈德鑫1, 吴浪1, 方开萱1, 刘文静1, 李明阳1,(), 令狐恩强1,()   
  1. 1. 100853 北京,解放军总医院第一医学中心消化内科
  • 收稿日期:2022-12-29 出版日期:2023-02-15
  • 通信作者: 李明阳, 令狐恩强

Analysis of clinical features of autoimmune pancreatitis

Shenxin Chen1, Yaqi Zhai1, Baoguo Bu1, Dexin Chen1, Lang Wu1, Kaixuan Fang1, Wenjing Liu1, Mingyang Li1,(), Enqiang Linghu1,()   

  1. 1. Department of Gastroenterology, The First Medical Center, Chinese PLA General Hospital, 100853 Beijing, China
  • Received:2022-12-29 Published:2023-02-15
  • Corresponding author: Mingyang Li, Enqiang Linghu
引用本文:

陈升鑫, 翟亚奇, 卜保国, 陈德鑫, 吴浪, 方开萱, 刘文静, 李明阳, 令狐恩强. 自身免疫性胰腺炎临床特征分析[J/OL]. 中华胃肠内镜电子杂志, 2023, 10(01): 46-51.

Shenxin Chen, Yaqi Zhai, Baoguo Bu, Dexin Chen, Lang Wu, Kaixuan Fang, Wenjing Liu, Mingyang Li, Enqiang Linghu. Analysis of clinical features of autoimmune pancreatitis[J/OL]. Chinese Journal of Gastrointestinal Endoscopy(Electronic Edition), 2023, 10(01): 46-51.

目的

分析探讨国内单中心大样本量的自身免疫性胰腺炎(AIP)的临床诊疗特点,旨在提高诊疗认知。

方法

回顾性分析2011年1月至2020年12月经解放军总医院诊断为自身免疫性胰腺炎的103例患者的临床资料。

结果

103例AIP患者中男女比例约3.3∶1,平均年龄(61.4±10.5)岁,临床表现主要为梗阻性黄疸和轻度腹痛或腹部不适;64例(62.1%)影像学呈胰腺呈弥漫性肿大,其中34例(33.0%)呈特征性的"腊肠样"肿胀;42例(40.8%)伴有胰管扩张;58例(78.4%)的核磁增强扫描呈特征性延迟强化。胰腺外病变以多发淋巴结病、胆管受累常见。血清IgG4阳性率为96.1%。胰腺穿刺病理符合率为24.4%。激素及联合激素方案整体有效率达98.8%。11例接受手术切除或剖腹探查及吻合术。治疗后的复发率为48.5%,其主要复发表现为黄疸和胰腺肿大。

结论

我院AIP病例以1型为主,临床表现无特殊性,胰腺外病变、影像学、血清学、组织病理学具有独特特征,激素治疗非常敏感,但容易复发,局灶性表现的AIP与胰腺癌鉴别仍充满挑战,综合分析可以提高临床诊断率。

Objective

To explore the clinical characteristics of Chinese AIP patients with the aim to raise the clinical awareness of diagnosis and treatment.

Methods

All clinical data about 103 patients with AIP in the PLA General Hospital from December 2011 to December 2020 were analyzed.

Results

The clinical manifestations were mainly obstructive jaundice and mild abdominal pain or discomfort. 64 cases (62.1%) showed diffuse enlargement of the pancreas on imaging, including 34 cases (33.0%) showed characteristic "sausage-like" swelling, 42 cases (40.8%) had pancreatic duct dilatation, and 58 cases (78.4%) showed characteristic delayed enhancement on MRI-enhanced scans. The pancreatic ducts were dilated in 42 cases (40.8%). Extra-pancreatic lesions with multiple lymphadenopathy and bile duct involvement were common. The serum IgG4 positivity rate was 96.1%. The pancreatic puncture pathology compliance rate was 24.4%.The overall effectiveness of glucocorticoid and combined glucocorticoid regimens was 98.8%. 11 cases underwent surgical resection or dissection and anastomosis. The recurrence rate after treatment was 48.5%, and their main recurrent manifestations were jaundice and pancreatic enlargement.

Conclusion

Type 1 AIP is the main subtype of AIP in our hospital without special clinical symptoms, however, it has unique features of extra-pancreatic lesions, imaging, serology and histopathology. Glucocorticoid therapy is very sensitive, but prone to relapse. Differentiation of focal AIP from pancreatic cancer remains challenging, and comprehensive analysis can effectively increase the correct diagnosis rate of AIP.

表1 103例AIP患者的人口学、既往史、症状及胰腺外病灶分析[例(%)]
图1 AIP增强MRI的特点 注:A:胰腺呈"腊肠样"肿胀,T1WI呈弥漫性长T1信号;B:T2WI稍长T2信号,胰腺周围见"包壳样"短T2信号;C:增强扫描动脉期胰腺肿大呈低信号,逐渐强化;D:延迟期胰腺延迟增强,周围边缘见"包壳样"强化。
表2 103例AIP患者影像学特征[例(%)]
图2 AIP导管影像学特征 注:A:主胰管多处不规则狭窄(见箭头处),狭窄上游轻度扩张;B:头颈部胰管迂曲,主胰管长狭窄(见箭头处),上游轻度扩张,胆总管下端狭窄伴上游扩张
表3 AIP患者血清学特征
图3 1型AIP组织病理学特征 注:A:IgG4染色可见富含淋巴细胞中散在IgG4阳性浆细胞浸润;B、C:丰富的淋巴浆细胞浸润伴纤维化,见散在IgG4阳性浆细胞浸润;D:Storiform纤维变性:炎细胞与成纤维细胞和螺旋状胶原纤维混杂,形成一种"席纹状"图案
表4 103例AIP患者治疗方案[例(%)]
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