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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
DOI: 10.3969/j.issn.1001-5256.2021.04.032
利益冲突声明：本研究不存在研究者、伦理委员会成员、受试者监护人以及与公开研究成果有关的利益冲突。
作者贡献声明：丁航负责论文撰写；郑琳琳、刘源负责数据整理，统计学分析；张连峰提供指导性支持；周琳负责课题设计，指导撰写及论文修改。
Clinical features and prognosis of autoimmune pancreatitis alone or with IgG4-related sclerosing cholangitis
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摘要:
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(χ2=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。 Abstract: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 (χ2=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. -
Key words:
- Autoimmune Pancreatitis /
- Cholangitis /
- Diagnosis /
- Therapeutics /
- Prognosis
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表 1 两组AIP患者的临床资料比较
项目 全部患者(n=40) 单纯AIP组(n=29) AIP合并IgG4-SC组(n=11) 统计值 P值 年龄(岁) 56.28±11.17 55.59±10.68 57.82±12.78 t=-0.533 0.597 男/女(例) 36/4 26/3 10/1 >0.05 病程(月) 1.0(0.5~2.8) 1.0(0.5~2.5) 2.0(1.0~3.0) Z=-1.095 0.280 过敏史[例(%)] 6(15.0) 5(17.2) 1(9.1) >0.05 糖尿病[例(%)] 16(40.0) 12(41.3) 4(36.4) >0.05 器官受累数目(个) 3.0(2.0~4.0) 3.0(1.5~3.5) 3.0(3.0~4.0) Z=-2.172 0.035 RI 12.0(9.0~15.0) 12.0(9.0~13.5) 12.0(12.0~15.0) Z=-2.157 0.032 接受激素治疗患者[例(%)] 34(85.0) 23(79.3) 11(100.0) 0.162 
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表 2 两组患者实验室指标比较
项目 全部患者(n=40)> 单纯AIP组(n=29) AIP合并IgG4-SC组(n=11) 统计值 P值 Hb(g/L) 125.3±13.5 127.8±13.4 118.7±11.7 t=1.985 0.054 WBC(×109/L) 6.0±1.7 6.2±1.8 5.4±1.5 t=1.157 0.254 PLT(×109/L) 195.7±64.7 201.0±72.0 181.8±38.8 t=1.077 0.289 嗜酸性粒细胞计数(×109/L) 0.25(0.13~0.45) 0.24(0.13~0.47) 0.14(0.10~0.40) Z=-0.879 0.385 ALT(U/L) 137.0(42.3~265.0) 168.0(39.5~334.5) 121.0(72.0~187.0) Z=-0.591 0.570 AST(U/L) 86.5(39.5~173.6) 87.0(27.5~205.5) 86.0(57.0~146.0) Z=-0.454 0.660 GGT(U/L) 342.0(85.5~787.5) 341.0(42.0~892.0) 527.0(146.0~642.0) Z=-0.227 0.830 ALP(U/L) 258.0(125.8~475.5) 251.0(111.0~495.0) 391.0(178.0~469.0) Z=-0.984 0.334 TBil(μmol/L) 54.1(17.4~109.3) 53.4(10.6~118.0) 54.7(32.9~107.0) Z=-0.197 0.850 球蛋白(g/L) 32.0(27.4~38.0) 30.2(26.6~38.2) 34.0(30.0~36.5) Z=-0.924 0.369 CA19-9(g/L) 32.1(17.6~79.4) 28.0(9.3~56.6) 64.2(21.2~81.5) Z=-1.459 0.151 IgG(g/L) 15.5(14.4~20.7) 14.8(13.3~15.7) 21.0(15.8~23.7) Z=-2.711 0.004 IgG4≥2×ULN[例(%)] 34(85.0) 23(79.3) 11(100.0) 0.162 淀粉酶(U/L) 45.0(18.3~71.0) 49.0(22.0~74.0) 31.0(16.0~61.0) Z=-1.496 0.368 脂肪酶(U/L) 33.5(13.9~94.4) 46.0(14.0~101.4) 15.5(9.1~30.2) Z=-0.915 0.144
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