原发性胆汁性肝硬化合并干燥综合征的临床特点分析

发布时间：2018-07-10 11:05

本文选题：原发性胆汁性肝硬化 + 干燥综合征　；参考：《山西医科大学》2017年硕士论文

【摘要】：目的:比较原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)合并干燥综合征(Sj?gren syndrome,SS)和单纯PBC、原发性干燥综合征(Primary Sj?gren syndrome,pSS)之间的异同,进一步分析PBC和SS之间的关系,提高对疾病的认识。方法:收集山西医科大学第二医院2010年11月1日~2016年6月1日确诊为PBC和pSS的病例,分别比较PBC合并SS与单纯PBC、pSS患者在临床特征、治疗反应及疾病进展方面的异同,分析PBC和SS之间的关系。结果:1.PBC合并SS患者确诊时基线资料中肝损害相关症状及生化指标均较单纯PBC患者轻;2.初诊诊断为PBC而后期合并SS的患者,初诊诊断时肝硬化及肝功能失代偿发生率较单纯PBC患者低,而口干症状较其明显;3.抗SSB抗体阳性率及发热症状在pSS患者较合并PBC患者多见;4.初诊诊断为SS而后期合并PBC的患者与pSS患者基线资料中干燥综合征相关症状及化验检查客观指标无差异;5.应用熊去氧胆酸+甲泼尼龙+吗替麦考酚酯治疗的PBC合并SS患者,其ALT、AST、ALP、GGT及ESR水平均较治疗前下降,但TBIL水平治疗前后变化不明显,且ALT、AST、ALP总体平均水平均在治疗3个月内达到正常范围;6.PBC合并SS患者应用熊去氧胆酸+甲泼尼龙+吗替麦考酚酯治疗1年,生化未缓解组较生化缓解组TBIL、ALP、GGT基线水平高;7.PBC合并SS患者和单纯PBC患者肝硬化及肝功能失代偿进展方面无差别。结论:1.PBC合并SS患者同时具有PBC和pSS的临床特点,但其确诊时基线资料中肝损害相关表现比单纯PBC患者轻,干燥综合征相关症状及免疫指标与pSS患者相似;2.伴口干症状的PBC患者易合并SS,需及早行干燥综合征相关检查进行筛查;3.应用熊去氧胆酸+甲泼尼龙+吗替麦考酚酯治疗PBC合并SS,可有效改善ALT、AST、ALP、GGT及ESR水平,但对TBIL改善不明显;基线ALP、GGT、TBIL水平可能与此治疗方案的生化缓解有关;4.PBC合并SS未加速PBC的疾病进展,甚至可能因两种自身免疫性疾病同时存在影响了PBC的疾病特点及预后,使肝损害较单纯PBC时轻。
[Abstract]:Objective: to compare the differences and similarities between primary biliary cirrhosis (primary biliary cirrhosis) and primary Sjgren syndrome with Sjgren syndrome (SS) and primary Sjgren syndrome (PSS), and to analyze the relationship between PBC and SS in order to improve the understanding of the disease. Methods: the cases of PBC and PSS diagnosed in the second Hospital of Shanxi Medical University from November 1, 2010 to June 1, 2016 were collected. The relationship between PBC and SS was analyzed. Results 1. The liver damage related symptoms and biochemical indexes in baseline data of PBC patients with SS were less than those of PBC patients. The incidence of liver cirrhosis and decompensation of liver function in the patients with PBC and SS in the first diagnosis was lower than that in the patients with PBC alone, but the symptoms of xerostomia were significantly lower than that of the patients with SS. The positive rate of anti SSB antibody and symptoms of fever were more common in PSS patients than in PBC patients. There was no significant difference in Sjogren's syndrome related symptoms and objective indexes of laboratory examination between the first diagnosis of SS and PBC in the baseline data of PSS patients. The levels of GGT and ESR in patients with PBC combined with SS treated with methylprednisolone ursodeoxycholate were lower than those before and after treatment, but the level of TBIL did not change significantly before and after treatment. The average level of ALP was within normal range within 3 months. Patients with PBC and SS were treated with methylprednisolone ursodeoxycholate for 1 year. The baseline level of TBILL ALP GGT in the non-remission group was higher than that in the biochemical remission group 7.There was no difference in liver cirrhosis and liver function decompensation between PBC patients with SS and PBC patients with simple PBC. Conclusion: 1. PBC patients with SS have the clinical characteristics of PBC and PSS simultaneously, but the liver damage related manifestations in baseline data of PBC patients are lighter than those in PBC patients alone. The symptoms and immune indexes of Sjogren's syndrome are similar to those of PSS patients. PBC patients with dry mouth symptoms are easy to be associated with SSS, and should be screened for Sjogren's syndrome (Sjogren syndrome) as early as possible. The application of methylprednisolone ursodeoxycholate in the treatment of PBC with SScan can effectively improve the levels of GGT and ESR in ALTAST, but the improvement of TBIL is not obvious. The level of TBIL may be related to the biochemical remission of the treatment regimen. 4. PBC combined with SS does not accelerate the progression of PBC, and may even affect the disease characteristics and prognosis of PBC because of the presence of two autoimmune diseases, which makes liver damage less severe than that of PBC alone.
【学位授予单位】：山西医科大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R575.22;R593.2

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