自身免疫性肝病患者的临床特点分析及其血清IgG4检测的临床意义
发布时间：2018-08-20 13:36
【摘要】：目的:总结分析自身免疫性肝病(AILD)患者的临床特征,为其早期诊断及治疗提供数据参考。探讨自身免疫性肝病患者血清Ig G4检测对自身免疫性肝病患者的诊断、鉴别诊断、病情评估等方面的临床意义,以期为临床工作中关于血清Ig G4的检测在自身免疫性肝病患者的价值提供一定数据参考。方法:回顾性分析我科近年来收治的522例自身免疫性肝病患者的一般资料、临床表现、自身抗体、血清生化学、肝活检病理等临床指标的差异,总结其各自的临床特点。这其中包括211名自身免疫性肝炎(AIH)患者,148例原发性胆汁性肝硬化(PBC)患者,155例重叠综合征(OS)例患者,8例原发性硬化性胆管炎(PSC)患者。继之,从上述病例中选取在我院标准治疗1年以上,且在实验室标本库中有治疗前后两次血清标本的自身免疫性肝病患者65例,其中AIH 30例,PBC 25例,OS10例,收集这65例自身免疫性肝病患者的血清标本,采用ELISA方法测定其治疗前后血清Ig G、IgG4的值,分析比较不同类型自身免疫性肝病患者血清Ig G4、Ig G4/Ig G水平的差异,分析血清IgG4水平与各临床指标的相关性,比较治疗前后Ig G4、Ig G4/Ig G的变化情况。结果:1.522例自身免疫性肝病患者中41~60岁年龄段人群所占比例最多,男女性别之比约为1:6。AIH患者临床表现中乏力、纳差、恶心症状较其余各组更为常见;PBC患者临床表现中黄疸较为常见,PSC患者临床表现中皮肤瘙痒较其余各组多见。部分患者在初诊时已有肝硬化、脾大或腹水表现,PBC患者在初诊时已有肝硬化者较其余各组多见。2.血清生化指标中反映肝细胞损伤的酶学指标ALT、AST升高在AIH组更为明显,而反映胆管损伤的酶学指标ALP、GGT指标升高在PBC及PSC组明显,PBC组中ALB下降较其余各组明显。免疫球蛋白IgG在AIH组升高明显,Ig M在PBC组升高明显,PSC组Ig G及Ig M升高无特异性。3.ANA阳性在AIH患者血清自身抗体检测中最多见,其次依次为p ANCA、ssa、ssb;而ama-m2在pbc患者血清自身抗体阳性中最常见,部分患者仅有ama-m2阳性,而无ama阳性。上述几种抗体阳性可以同时出现在os患者的血清检测中。4.aih组患者肝组织活检病理极少累及胆管,主要表现为肝组织中炎细胞浸润、肝细胞变性坏死及界面性肝炎。pbc组患者主要表现为明显淤胆及小胆管破坏和增生。psc患者组织病理主要表现为胆管周围水肿、胆管周围纤维化、汇管区胆管缺失及胆汁淤积。os组患者可兼有aih及pbc或psc患者的病理学特征。5.本地区自身免疫性肝病多合并其他自身免疫性系统疾病,主要包括风湿关节炎、甲状腺疾病、1型糖尿病、系统性红斑狼疮(sle)、干燥综合征、溃疡性结肠炎,而自身免疫性肝炎患者合并类风湿关节炎最为常见,8例psc患者中2例合并溃疡性结肠炎。6.65例自身免疫性肝病患者中有5例(7.7%)患者血清igg4水平≥1350mg/l,其中1例为pbc患者,其余4例均为aih患者,这4例aih患者中3例合并糖尿病,1例合并其他自身免疫性疾病。7.三种自身免疫性肝病患者血清igg4水平与所观察血清指标中alp及igg4/igg明显相关,且呈正相关,而与其他血清学指标没有显著相关性。8.血清igg4水平与aih、pbc、os三种自身免疫性肝病患者的不同诊断之间虽无显著相关性,但aih组患者血清igg4及igg4/igg的中位数水平略高于pbc及os组患者。9.治疗后血清酶学指标明显改善,而治疗前后血清igg4、igg4/igg的值差异无统计学意义。结论:1.在我国西南地区自身免疫性肝病患者的高发人群为50岁左右的围绝经期女性,aih、pbc及os较为常见,psc少见。西南地区自身免疫性肝炎以i型为主。psc患者男性所占比例小于国内其他报道。该地区相当部分自身免疫性肝病患者在初诊时病情多已进展。2.我国西南地区自身免疫性肝炎主要合并类风湿关节炎,而psc患者合并溃疡性结肠炎比例小于国内其他报道。ama阴性的pbc患者行ama-m2检测尤为重要。多数psc患者有自身抗体panca阳性,但其无特异性血清生化学及免疫球蛋白改变,特异性影像学检查及病理组织活检对其诊断十分重要。综合临床表现、自身抗体、免疫球蛋白水平、血清生化学指标、合并其他自身免疫系统疾病、影像学检查、病理活检对于自身免疫性肝病的诊断有重要意义。3.AIH患者血清Ig G4水平较PBC、OS患者稍高,合并其他自身免疫性疾病及代谢疾病的自身免疫性肝病患者血清Ig G4水平升高较为明显。血清Ig G4水平与AIH、PBC、OS三种自身免疫性肝病患者的不同诊断之间相关性不显著,这提示可能暂不能将Ig G4及Ig G4/Ig G作为三种自身免疫性肝病的鉴别诊断指标。三种自身免疫性肝病患者血清Ig G4水平与所观察血清指标中ALP明显相关,提示Ig G4可能参与了胆管损伤。血清IgG4与自身免疫性肝病患者病情改善无明显相关性。
[Abstract]:Objective: To summarize and analyze the clinical features of autoimmune liver disease (AILD) and provide data for its early diagnosis and treatment. Methods: Retrospective analysis of 522 patients with autoimmune liver disease treated in our department in recent years, the general data, clinical manifestations, autoantibodies, serum biochemistry, liver biopsy pathology and other clinical indicators of the differences, summed up their respective clinical characteristics. Patients with autoimmune hepatitis (AIH), 148 patients with primary biliary cirrhosis (PBC), 155 patients with overlapping syndrome (OS) and 8 patients with primary sclerosing cholangitis (PSC) were selected from the above-mentioned cases for more than one year, and the laboratory specimen bank had autoimmunity of serum samples before and after treatment for two times. Serum IgG and IgG4 were measured by ELISA in 65 patients with autoimmune liver disease, including 30 patients with AIH, 25 patients with PBC and 10 patients with OS. Results: Among the 1.522 patients with autoimmune liver disease, 41-60 years old accounted for the largest proportion, the ratio of male to female was about 1:6. The clinical manifestations of AIH patients were fatigue, poor appetite, nausea symptoms more common than the other groups; PBC patients with clinical manifestations of jaundice more common. Some of the patients had cirrhosis, splenomegaly or ascites at the time of initial diagnosis, and the patients with liver cirrhosis at the time of initial diagnosis were more common than the other groups. 2. Serum biochemical indicators of hepatocyte injury in ALT, AST increased more significantly in AIH group, but reflected bile duct injury. ALP and GGT increased significantly in PBC and PSC groups, and ALB decreased significantly in PBC group. IgG increased significantly in AIH group, Ig M increased significantly in PBC group, and IgG and Ig M increased nonspecific in PSC group. Serum autoantibodies were the most common in patients with chronic hepatitis C. only AMA-M2 was positive in some patients, but no AMA was positive. these antibodies could be detected simultaneously in serum of patients with chronic hepatitis C. 4. liver biopsy pathology in AIH group rarely involved bile ducts, mainly manifested as infiltration of inflammatory cells in liver tissue, degeneration and necrosis of hepatocytes and hepatitis interface. The main histopathological features of PSC patients were peribiliary edema, peribiliary fibrosis, absence of bile ducts in the portal area and cholestasis. Epidemic systemic diseases, including rheumatoid arthritis, thyroid disease, type 1 diabetes mellitus, systemic lupus erythematosus (sle), Sjogren's syndrome, and ulcerative colitis, were most common in autoimmune hepatitis patients with rheumatoid arthritis, 2 of 8 PSC patients with ulcerative colitis, 5 of 6.65 patients with autoimmune liver disease (7.7) Serum IgG4 levels were higher than 1 350 mg / L in 3 of the 4 AIH patients with diabetes mellitus and 1 with other autoimmune diseases. There was no significant correlation between serum IgG4 levels and the diagnosis of aih, pbc, OS three kinds of autoimmune liver disease, but the median serum IgG4 and IgG4 / IgG levels in AIH group were slightly higher than those in PBC and OS group. Conclusion: 1. In perimenopausal women aged 50 or so, aih, PBC and OS are more common and PSC is less common. In the southwest of China, autoimmune hepatitis is predominant in type I. The proportion of male patients with PSC is less than other reports in China. Most of the patients with autoimmune hepatitis in southwest China were accompanied by rheumatoid arthritis, while the proportion of PSC patients with ulcerative colitis was lower than other reports in China. Am-m2 test was especially important in patients with PBC negative for ama. Biochemical and immunoglobulin changes in heterosexual serum, specific imaging and pathological biopsy are important for the diagnosis of autoimmune liver disease. Significance. 3. The levels of serum Ig G4 in AIH patients were slightly higher than those in PBC and OS patients. The levels of serum Ig G4 in patients with autoimmune liver diseases complicated with other autoimmune diseases and metabolic diseases were significantly higher than those in patients with AIH, PBC and OS. Ig G4 and Ig G4/Ig G can be used as the differential diagnostic indexes of three kinds of autoimmune liver diseases.The levels of serum Ig G4 in patients with three kinds of autoimmune liver diseases are significantly correlated with ALP in the serum indexes observed, suggesting that Ig G4 may be involved in bile duct injury.There is no significant correlation between serum IgG4 and the improvement of the condition of patients with autoimmune liver diseases.
【学位授予单位】：第三军医大学
【学位级别】：硕士
【学位授予年份】：2015
【分类号】：R575
本文编号：2193805
[Abstract]:Objective: To summarize and analyze the clinical features of autoimmune liver disease (AILD) and provide data for its early diagnosis and treatment. Methods: Retrospective analysis of 522 patients with autoimmune liver disease treated in our department in recent years, the general data, clinical manifestations, autoantibodies, serum biochemistry, liver biopsy pathology and other clinical indicators of the differences, summed up their respective clinical characteristics. Patients with autoimmune hepatitis (AIH), 148 patients with primary biliary cirrhosis (PBC), 155 patients with overlapping syndrome (OS) and 8 patients with primary sclerosing cholangitis (PSC) were selected from the above-mentioned cases for more than one year, and the laboratory specimen bank had autoimmunity of serum samples before and after treatment for two times. Serum IgG and IgG4 were measured by ELISA in 65 patients with autoimmune liver disease, including 30 patients with AIH, 25 patients with PBC and 10 patients with OS. Results: Among the 1.522 patients with autoimmune liver disease, 41-60 years old accounted for the largest proportion, the ratio of male to female was about 1:6. The clinical manifestations of AIH patients were fatigue, poor appetite, nausea symptoms more common than the other groups; PBC patients with clinical manifestations of jaundice more common. Some of the patients had cirrhosis, splenomegaly or ascites at the time of initial diagnosis, and the patients with liver cirrhosis at the time of initial diagnosis were more common than the other groups. 2. Serum biochemical indicators of hepatocyte injury in ALT, AST increased more significantly in AIH group, but reflected bile duct injury. ALP and GGT increased significantly in PBC and PSC groups, and ALB decreased significantly in PBC group. IgG increased significantly in AIH group, Ig M increased significantly in PBC group, and IgG and Ig M increased nonspecific in PSC group. Serum autoantibodies were the most common in patients with chronic hepatitis C. only AMA-M2 was positive in some patients, but no AMA was positive. these antibodies could be detected simultaneously in serum of patients with chronic hepatitis C. 4. liver biopsy pathology in AIH group rarely involved bile ducts, mainly manifested as infiltration of inflammatory cells in liver tissue, degeneration and necrosis of hepatocytes and hepatitis interface. The main histopathological features of PSC patients were peribiliary edema, peribiliary fibrosis, absence of bile ducts in the portal area and cholestasis. Epidemic systemic diseases, including rheumatoid arthritis, thyroid disease, type 1 diabetes mellitus, systemic lupus erythematosus (sle), Sjogren's syndrome, and ulcerative colitis, were most common in autoimmune hepatitis patients with rheumatoid arthritis, 2 of 8 PSC patients with ulcerative colitis, 5 of 6.65 patients with autoimmune liver disease (7.7) Serum IgG4 levels were higher than 1 350 mg / L in 3 of the 4 AIH patients with diabetes mellitus and 1 with other autoimmune diseases. There was no significant correlation between serum IgG4 levels and the diagnosis of aih, pbc, OS three kinds of autoimmune liver disease, but the median serum IgG4 and IgG4 / IgG levels in AIH group were slightly higher than those in PBC and OS group. Conclusion: 1. In perimenopausal women aged 50 or so, aih, PBC and OS are more common and PSC is less common. In the southwest of China, autoimmune hepatitis is predominant in type I. The proportion of male patients with PSC is less than other reports in China. Most of the patients with autoimmune hepatitis in southwest China were accompanied by rheumatoid arthritis, while the proportion of PSC patients with ulcerative colitis was lower than other reports in China. Am-m2 test was especially important in patients with PBC negative for ama. Biochemical and immunoglobulin changes in heterosexual serum, specific imaging and pathological biopsy are important for the diagnosis of autoimmune liver disease. Significance. 3. The levels of serum Ig G4 in AIH patients were slightly higher than those in PBC and OS patients. The levels of serum Ig G4 in patients with autoimmune liver diseases complicated with other autoimmune diseases and metabolic diseases were significantly higher than those in patients with AIH, PBC and OS. Ig G4 and Ig G4/Ig G can be used as the differential diagnostic indexes of three kinds of autoimmune liver diseases.The levels of serum Ig G4 in patients with three kinds of autoimmune liver diseases are significantly correlated with ALP in the serum indexes observed, suggesting that Ig G4 may be involved in bile duct injury.There is no significant correlation between serum IgG4 and the improvement of the condition of patients with autoimmune liver diseases.
【学位授予单位】：第三军医大学
【学位级别】：硕士
【学位授予年份】：2015
【分类号】：R575
【参考文献】
相关期刊论文 前2条
1 张敏;韩涛;聂彩云;王伟;杜卫杰;周淑芬;王棕覃;孙扬;丁贤;朱争艳;;自身免疫性肝病患者血清IgG4水平分析[J];实用肝脏病杂志;2014年04期
2 施健,谢渭芬;自身免疫性肝病的发病机制[J];中华肝脏病杂志;2005年01期
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