自身免疫性肝炎与原发性胆汁性肝硬化合并干燥综合征患者临床特征比较*

doi:10.3969/j.issn.1672-5069.2024.02.012

摘要/Abstract

摘要： 目的分析总结自身免疫性肝炎(AIH)和原发性胆汁性肝硬化(PBC)合并干燥综合征(SS)患者的临床特征。方法 2015年6月～2023年6月我院收治的自身免疫性肝炎(AIH)患者 50例、AIH合并SS 患者20例, PBC 患者85例和PBC合并SS 患者45例, 均接受肝活检, 采用免疫印迹法检测血清抗核抗体(ANA)、抗肝细胞溶质抗原1型抗体(LC-1)、抗可溶性肝抗原/肝胰抗原抗体(SLA/LP)、抗线粒体M2型抗体(AMA-M2)、抗可溶性酸性磷酸化核蛋白抗体(Sp100)、抗核膜糖蛋白210抗体(gp210)和抗Ro-52抗体(Ro-52)。结果 AIH合并SS患者口干/眼干发生率为95.0%, 显著高于AIH组的10.0%(P<0.05), PBC合并SS患者口干/眼干发生率为97.8%, 显著高于PBC组的12.9%(P<0.05), 而瘙痒和黄疸发生率分别为37.8%和40.0%, 显著低于PBC组的67.1%和60.6%(P<0.05);AIH合并SS患者血清总胆红素(TBIL)水平为(14.4±3.1)μmol/L, 显著低于AIH组【(23.8±5.3)μmol/L, P<0.05】;PBC合并SS患者血清TBIL和碱性磷酸酶(ALP)水平分别为(26.7±6.6)μmol/L和(159.1±14.3)U/L, 显著低于PBC组【分别为(61.4±13.8)μmol/L和(223.5±25.4)U/L, P<0.05】, 血清免疫球蛋白(Ig)G水平为(20.6±3.6)g/L, 显著高于PBC组【(15.6±2.5)g/L, P<0.05】;AIH合并SS患者血清抗Ro-52阳性率为55.0%, 显著高于AIH组的16.0%(P<0.05), PBC合并SS患者血清抗Ro-52阳性率为53.3%, 显著高于PBC组的25.9%(P<0.05);与AIH/PBC组比, AIH合并SS/PBC合并SS患者肝组织病理学特征差异无统计学意义(P＞0.05)。结论合并SS的AIH和PBC患者在临床表现和实验室检查结果方面存在一定的差异, 可能使病情具有复杂性, 临床在诊治过程中, 应注意识别和处理。

关键词: 自身免疫性肝炎, 原发性胆汁性肝硬化, 干燥综合征, 临床特征

Abstract: Objective The aim of this study was to compare the clinical characteristics of patients with autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) with concomitant Sjogren's syndrome (SS). Methods A retrospective study was conducted to summarize 200 patients with autoimmune liver diseases (AILD) admitted to our hospital between June 2015 and June 2023, including 50 cases of AIH, 20 cases of AIH with SS, 85 cases of PBC, and 45 cases of PBC concomitant with SS. The clinical manifestations, laboratory tests, positive rates of serum autoantibodies, and histopathology characteristics of liver were compared. Results The incidence of dry mouth/dry eye in patients with AIH /SS was 95.0%, significantly higher than 10.0% in patients with AIH (P<0.05), the incidence of dry mouth/dry eye in patients with PBC/SS was 97.8%, much higher than 12.9%(P<0.05), while the incidences of itching and jaundice were 37.8% and 40.0%, much lower than 67.1% and 60.6%(P<0.05)in patients with PBC; total serum bilirubin level (TSB) in patients with AIH/SS was (14.4±3.1)μmol/L, much lower than in patients with AIH; serum bilirubin and ALP levels in patients with PBC/SS were(26.7±6.6)μmol/L and (159.1±14.3)U/L, significantly lower than , while serum IgG level was (20.6±3.6)g/L, much higher than in patients with PBC; the positive rate of serum anti- Ro-52 in patients with AIH/SS was 55.0%, much higher than 16.0%(P<0.05) in patients with AIH, and that was 53.3% in patients with PBC/SS, much higher than 25.9%(P<0.05) in patients with PBC; there were no significant differences respect to liver histopathological features among patients with AIH/SS or with PBC/SS, as compared to in patients with AIH or with PBC(P＞0.05). Conclusion There are some differences in clinical manifestations and laboratory results between patients with AIH and with PBC with or without concomitant SS, the internists should take care of them and deal with appropriately in clinical practice.

Key words: Autoimmune hepatitis, Primary biliary cirrhosis, Sjogren&apos, s syndrome, Clinical features

卜玲玲, 殷燕, 潘丹烨, 陈晨. 自身免疫性肝炎与原发性胆汁性肝硬化合并干燥综合征患者临床特征比较^*[J]. 实用肝脏病杂志, 2024, 27(2): 206-209.

Bu Lingling, Yin Yan, Pan Danye, et al.. Comparison of clinical characteristics between patients with autoimmune hepatitis and primary biliary cirrhosis with Sjogren's syndrome[J]. Journal of Practical Hepatology, 2024, 27(2): 206-209.

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自身免疫性肝炎与原发性胆汁性肝硬化合并干燥综合征患者临床特征比较^*

Comparison of clinical characteristics between patients with autoimmune hepatitis and primary biliary cirrhosis with Sjogren's syndrome

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