Characteristics of patients with primary biliary cholangitis-autoimmune hepatitis overlap syndrome and diverse anti-mitochondrial antibody in Qinghai region

Abstract

Abstract: Objective To delineate the clinical and pathological characteristics of primary biliary cholangitis-autoimmune hepatitis overlap syndrome(PBC-AIH OS) in Qinghai province, aiming to enhance clinical diagnosis and treatment protocols. Methods In a retrospective study spanning December 2017 to December 2022, 85 patients diagnosed with PBC-AIH OS were analyzed. The cohort was divided into two groups based on Anti-mitochondrial antibody status: 58 in Group A(positive) and 27 in Group B(negative). The study evaluated general clinical features, comorbid conditions, serum biochemical markers, immunological indices, and liver tissue pathology. Date adhering to a normal distribution were presented as c±s and analyzed using the independent sample t-test. Non-normally distributed measurements were expressed as medians(P₂₅, P₇₅) and compared using the Wilcoxon rank-sum test. Categorical data were analyzed using chi-square tests to compare proportions between groups; with a P-value of <0.05 denoting statistical significant. Results In the comparison between the two groups, there were no statistically significant differences observed interms of sex, age, TBil, γ-GGT, ALP, IgG, Ro52 presence, the detection rate of SSA, the prevalence of Sjogren′s syndrome, and the grades of liver inflammation and fibrosis (P>0.05). However, significant differences were noted in the levels of ALT (115.41±76.63 vs. 255.90±244.90) and IgM [4.05 (2.75, 5.43) vs. 1.99 (1.41, 3.51)], as well as in the detection rate of ANA-G(χ²=9.67) and the morbidity rate of autoimmune thyroid disease (AITD) (χ²=5.74), with P＜0.05 indicating statistical significance. Conclusion Patients in Group A exhibited levels of ALT, IgM and ANA-Gr detection rates, whereas the prevalence of AITD was greater in the Group B. The pathological histology for both groups predominantly revealed moderate to severe,inflammation and fibrosis rades, suggesting an insidious onset, prolonged disease course, and advanced stage at diagnosis for these patients. Given these findings, it is advisable for patients diagnosed with PBC-AIH OS to undergo thorough screening for,thyroid disorders.

Key words: Qinghai Region, Anti-mitochondrial antibody, Primary biliary cholangitis, Autoimmune hepatitis, Autoimmune liver disease, Overlap syndrome

WANG Chun-qiu, CHEN Ting, ZHANG Zhen-ying, ZHANG Xiao-fei. Characteristics of patients with primary biliary cholangitis-autoimmune hepatitis overlap syndrome and diverse anti-mitochondrial antibody in Qinghai region[J]. Chinese Hepatolgy, 2024, 29(4): 448-452.

References

[1] Joshita S, Umemura T, Anaka E, et al. Genetics and epigenetics in the pathogenesis of primary biliary cholangitis[J]. Clin J Gastroenterol,2018,11(1):11-18.
[2] Van Buuren R, Van Hoogstraten H, Terkivatan T, et al. High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis[J]. J Hepatol,2000,33(4):543-548.
[3] Muratori P, Fabbri A, Lalanne C, et al. Autoimmune liver disease and concomitant extrahepatic autoimmune disease[J]. Eur J Gastroenterol Hepatol,2015,27:1175-1179.
[4] 陈莎, 段维佳, 尤红, 等. 亚太肝病学会《原发性胆汁性胆管炎临床诊疗指南》推荐意见[J]. 中华肝脏病杂志,2022,30(2):196-198.
[5] 高冀蓉, 乔玲, 李侗曾, 等. 原发性胆汁性肝硬化和自身免疫性肝炎重叠综合征患者的临床特征分析[J]. 中国综合临床,2006,22(09):797-799.
[6] Fan X, Zhu Y, Menr, et al. Efficacy and safety of immunosuppressive therapy for PBC-AIH overlap syndrome accompanied by decompensated cirrhosis: A real-world study[J]. Can J Gastroenterol Hepatol,2022,20(18):965-971.
[7] Lohse AW, Zum Buschenfelde KH, Franz B, et al. Characterization of the overlap syndrome of primary bilary cirrhosis (PBC) and autoimmune hepatitis:evidence for it being a hepatitic form of PBC in genetically suscceptible individuals[J]. Hepatology,1999,29(4):1078-1084.
[8] 谭晓燕, 苗琪, 陈晓宇. 线粒体抗体阴性原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征的临床病理分析[J]. 中华肝脏病杂志,2019,27(5):376-380.
[9] Xiao Q, Tan SZ, Zhao TH, et a1. Value of antinuclear antibody and liver pathology in diagnosis of male patients with autoimmune liver diseases[J]. J Clin Hepatol,2019,35(5):1070-1073.
[10] 肖倩, 谭善忠, 赵天慧, 等. 抗核抗体及肝组织病理对男性自身免疫性肝病患者的诊断意义分析[J]. 临床肝胆病杂志,2019,35(5):1070-1073.
[11] Juliusson G, Imam M, Bjornsson ES, et al. Long-term outcomes in antimitochondrial antibody negative primary biliary cirrhosis[J]. Scand J Gastroenterol,2016,51(6):745-752.
[12] Saito H, Takahashi A, Abe K, et al. Autouamtibodies by line immunoassay in patients with primary biliary cirrhosis[J]. Fukushima J Med Sci,2012,58(2):107-116.
[13] 徐艳, 于秉楠, 武剑. 自身免疫性肝炎和原发性胆汁性肝硬化患者自身免疫性甲状腺疾病流行率调查[J]. 实用肝脏杂志,2022,1(25):42-45.
[14] 张梅芹, 沈兰超, 陈国峰, 等. 自身免疫性肝炎与原发性胆汁性胆管炎重叠综合征患者的临床病理学特征分析[J]. 肝脏,2020,25(9):965-967.

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