自身免疫性胰腺炎及其合并IgG4相关硬化性胆管炎的临床特征和预后比较

Clinical features and prognosis of autoimmune pancreatitis alone or with IgG4-related sclerosing cholangitis

目的比较分析单纯自身免疫性胰腺炎(AIP)与AIP合并IgG4相关硬化性胆管炎(IgG4-SC)患者的临床特征差异、诊治和预后。方法回顾性分析2015年6月—2020年1月郑州大学第一附属医院收治的40例1型AIP患者资料,其中包括29例单纯AIP患者及11例AIP合并IgG4-SC患者,对比两组患者的临床表现、实验室检查、影像学表现、治疗及预后。正态分布的计量资料两组间比较采用t检验,非正态分布的计量资料两组间比较采用Mann-Whitney U检验。计数资料两组间比较采用Fisher确切检验。采用Kaplan-Meier法计算患者复发率并绘制复发曲线,采用log-rank检验进行比较。结果与单纯AIP组相比,AIP合并IgG4-SC组受累器官数目更多[3.0(3.0~4.0)个vs 3.0(1.5~3.5)个,Z=-2.172,P=0.035],治疗前反应指数更高[12.0(12.0~15.0)vs 12.0(9.0~13.5),Z=-2.157,P=0.032]。AIP合并IgG4-SC组血清IgG水平为21.0(15.8~23.7)g/L,高于单纯AIP组的14.8(13.3~15.7)g/L,差异有统计学意义(Z=-2.711,P=0.004)。在中位随访时间15.8(6.5~31.3)个月内,AIP合并IgG4-SC组复发率明显高于单纯AIP组,差异有统计学意义(χ^(2)=8.155,P=0.004)。结论AIP合并IgG4-SC组血清IgG4水平更高、受累器官更多,且更易出现复发。早期识别、诊断和治疗可降低AIP的复发率。

Objective To investigate the clinical features,diagnosis,treatment,and prognosis of autoimmune pancreatitis(AIP)alone versus AIP with IgG4 sclerosing cholangitis(IgG4-SC).Methods A retrospective analysis was performed for the clinical data of 40 patients with type 1 AIP who were admitted to The First Affiliated Hospital of Zhengzhou University from June 2015 to January 2020,among whom 29 patients had AIP alone and 11 had AIP with IgG4-SC.The two groups were compared in terms of clinical manifestations,laboratory examination,imaging findings,treatment,and prognosis.The t-test was used for comparison of normally distributed continuous data between two groups,and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups;the Fisher’s exact test was used for comparison of categorical data between two groups.The Kaplan-Meier method was used to calculate recurrence rate and plot recurrence curve,and the log-rank test was used for univariate analysis.Results Compared with the AIP group,the AIP+IgG4-SC group had significantly higher number of affected organs[3.0(3.0-4.0)vs 3.0(1.5-3.5),Z=-2.172,P=0.035]and response index before treatment[12.0(12.0-15.0)vs 12.0(9.0-13.5),Z=-2.157,P=0.032].The AIP+IgG4-SC group had a significantly higher median serum IgG level than the AIP group[21.0(15.8-23.7)g/L vs 14.8(13.3-15.7)g/L,Z=-2.711,P=0.004].During the median follow-up time of 15.8(6.5-31.3)months,the AIP+IgG4-SC group had a significantly higher recurrence rate than the AIP group(χ^(2)=8.155,P=0.004).Conclusion Patients with AIP and IgG4-SC tend to have higher serum IgG4 level,number of affected organs,and recurrence rate than those with AIP alone.Early identification,diagnosis,and treatment can reduce the recurrence rate of AIP.