摘要
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。
Abstract
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.
万方数据