141例原发性胆汁性胆管炎临床特征分析
发布时间：2018-07-23 14:10
【摘要】：目的:观察分析本院诊断为原发性胆汁性胆管炎(primary biliary cholangitis,PBC)的141例患者的临床资料(一般资料、临床表现、血液生化、自身抗体、影像学检查等)及其特点,探索抗Ro-52抗体与PBC之间的联系,对PBC患者食管/胃底静脉曲张相关指标做诊断价值评估,以进一步提高对该病的认识。方法:对我院2011年06月份~2016年12月份收治的具有完整病例资料的141例诊断为原发性胆汁性胆管炎患者的一般资料、临床症状及体征、临床并发症及合并症、血清生化指标、自身抗体、内镜检查、影像学检查及Fibro Scan检查资料进行回顾性分析。同一病人多次住院仅取第一次住院资料。结果:1.本研究男性26例(18.4%),女性115例(81.6%),男女比例为:1:4.42,所有病例平均确诊年龄(57.8±10.8)岁,病程240(60-700)天,住院天数11(8-15)天。2.本研究资料PBC患者常见的临床表现:黄疸、肝脾肿大、乏力、食欲减退、上腹部不适、腹水、皮肤瘙痒、眼干口干、下肢水肿、肝掌蜘蛛痣等。3.主要并发症有:电解质紊乱(37例)、消化道出血(20例)、感染(14例)、肝性脑病(2例);主要的合并症有:干燥综合征(Sjogren's syndrome 11例)、糖尿病(6例)、桥本氏甲状腺炎(5例)、类风湿关节炎(1例)、系统性红斑狼疮(1例)、成人STILL病(1例)、系统性硬化(1例)。4.PBC患者血清生化检查以ALP及GGT升高为主[261.6(155.5-489.1)U/L,179.1(81.0-430.5)U/L],并且于疾病早期阶段升高显著,到晚期升高不明显;AST、ALT轻度升高[69.0(43.0-114.0)U/L,54.0(32.0-93.5)U/L],血清总胆红素为30.5(19.2-64.6)umol/L,为正常上限,总胆汁酸TBA 20.1(8.5-45.8)umol/L,高于正常上限2倍,免疫球蛋白Ig M升高,为3.1(2.0-4.2)G/L。5.自身抗体检查,AMA阳性有91例(64.5%),AMA-M2阳性120例(85.1%),125例(88.7%)患者ANA阳性,31例(22.0%)抗着丝点B抗体阳性,66例(46.8%)抗Ro-52抗体阳性,22例(15.6%)SSA抗体阳性,15例(10.6%)SSB抗体阳性。6.研究对象中行内镜检查者总共65例,有35例(53.9%)有食管静脉曲张,9例(13.9%)有胃底静脉曲张,8例(12.3%)提示有溃疡和(或)糜烂,8例(12.3%)有门脉高压性胃病,29例(44.6%)提示慢性胃炎。8例(12.3%)同时有食管静脉曲张和胃底静脉曲张。7.共有118例患者行腹部超声检查,超声提示98例(83.1%)肝脏弥漫性病变,38例(32.2%)腹水,81例(68.6%)存在肝脾肿大,35例(29.7%)提示门脉/脾静脉增宽或流速减低,5例提示(4.2%)胆汁淤积,4例(3.4%)提示门脉血栓形成;6例(5.1%)超声检查正常。有41例患者行上腹部CT增强扫描或平扫,结果提示有25例(61.0%)肝硬化,有14例(34.2%)提示腹水,有29例(70.7%)存在肝脾大,有7例(17.1%)提示门脉高压,有19例(46.3%)提示食管静脉曲张,有16例(39.0%)提示胃底静脉曲张。有8例(19.5%)同时有食管胃镜静脉曲张及胃底静脉曲张。8.本研究共有26例患者行Fibro Scan检查,结果提示9例(34.6%)患者处于肝硬化阶段。对141例患者进行Mayo评分,评分均值为5.7±1.5。Mayo危险评分与Fibro Scan分期之间存在显著的正相关关系(相关系数0.948,P值0.000)。9.男性病程150(30-361)较女性组病程300(60-1000)短,有统计学差异(P=0.023)。AMA在女性组(68.7%)检出率高于男性组(46.2%),差异具有统计学差异。余临床表现、生化检查及影像学检查两组间无明显差异。10.Ro-52阳性组较阴性组更容易出现眼干口干、腹水,有统计学差异(P分别为0.014和0.012);阳性组合并干燥综合征的较阴性组多,存在统计学上存在差异(P=0.002);阳性组血清Ig G较阴性组高(P值0.023),但CHE较阴性组低(P=0.008)。11.对食管静脉曲张的诊断价值评价指标中,当Mayo评分等于4.8、血小板计数为130×109/L、血清白蛋白38.6g/L时所对应约登指数最大,三者对食管静脉曲张有一定的诊断价值。总胆红素和凝血酶原时间的静脉曲张的诊断价值有限。结论:原发性胆汁性胆管炎主要累及中年女性,女性患者病程更长,主要表现为黄疸、肝脾大、乏力、食欲减退、上腹不适、腹水、皮肤瘙痒、眼干口干,症状缺乏特异性,早期部分患者无明显症状,到疾病后期并发肝硬化并发症,也伴随自身免疫疾病,如干燥综合征。非侵入性检查中最具有诊断价值的是血清AMA/AMA-M2抗体,Ro-52阳性组更容易合并干燥综合征,患者血清中检出此抗体则更容易出现腹水,Ro-52阴性的患者可能肝脏合成功能较低。当Mayo评分大于4.8、血小板计数小于130×109/L、血清白蛋白小于38.6g/L的患者需密切定期随访胃镜检查。目前治疗以UCDA为主,需要找到更加有效安全的方法早期诊断早期治疗。
[Abstract]:Objective: To observe and analyze the clinical data of 141 patients diagnosed as primary biliary cholangitis (PBC) in our hospital (general data, clinical manifestation, blood biochemistry, autoantibody, imaging examination, etc.) and their characteristics, to explore the relationship between anti Ro-52 antibody and PBC, and to refer to the related indications of esophageal / gastric varices in PBC patients. Evaluation of the diagnostic value to further improve the understanding of the disease. Methods: 141 patients with complete cases of cholangitis diagnosed in our hospital in 2011, ~2016, ~2016 December, were diagnosed as general data, clinical symptoms and signs, clinical complications and complications, serum biochemical indexes, autoantibodies, and endoscopy. Review, imaging examination and Fibro Scan examination data. The same patient was hospitalized for the first time in hospitalization. Results: 1. men were 26 (18.4%) and 115 women (81.6%). The male and female ratio was 1:4.42, the average age of all cases was (57.8 + 10.8) years, the course of disease was 240 (60-700) days, and the hospital days were 11 (8-15) days.2. Ben. The common clinical manifestations of PBC patients: jaundice, hepatomegaly, asthenia, anorexia, upper abdominal discomfort, ascites, skin itching, dry mouth, edema of the lower extremities, and spider nevus of the liver, including electrolyte disorder (37 cases), gastrointestinal bleeding (20 cases), infection (14 cases), hepatic encephalopathy (2 cases); main complication: drying synthesis Syndrome (Sjogren's syndrome 11), diabetes (6 cases), Hashimoto's thyroiditis (5 cases), rheumatoid arthritis (1 cases), systemic lupus erythematosus (1 cases), adult STILL disease (1 cases), systemic sclerosis (1 cases) of.4.PBC patients with ALP and GGT elevation as the main [261.6 (155.5-489.1) U/L, 179.1 (81.0-430.5) U/L], and in the early stage of disease. Gao Xian was not significantly higher at the late stage; AST, ALT slightly elevated [69.0 (43.0-114.0) U/L, 54 (32.0-93.5) U/L], serum total bilirubin 30.5 (19.2-64.6) umol/L, the normal upper limit, the total bile acid TBA 20.1 (8.5-45.8) umol/L, 2 times higher than the normal upper limit, 3.1 (6) autoantibody examination, 91 cases (6) positive. 4.5%) AMA-M2 positive 120 cases (85.1%), 125 (88.7%) patients with ANA positive, 31 (22%) anti filal B antibody positive, 66 (46.8%) anti Ro-52 antibody positive, 22 cases (15.6%) SSA antibody positive, 15 (10.6%) SSB positive.6. research subjects in total 65 cases, there are esophageal varices in patients with esophageal varices and gastric fundus veins 8 cases (12.3%) showed ulcers and (or) erosion, 8 cases (12.3%) had portal hypertensive gastropathy, 29 cases (44.6%) showed chronic gastritis.8 (12.3%) with esophageal varices and fundus varicose.7. in 118 patients with abdominal ultrasound, 98 (83.1%) liver diffuse lesions, 38 (32.2%) ascites, 81 cases (68.6%)). In hepatomegaly, 35 cases (29.7%) showed portal / splenic vein widening or decreased flow rate, 5 cases (4.2%) cholestasis, 4 cases (3.4%) indicating portal vein thrombosis; 6 cases (5.1%) ultrasound examination normal. There were 41 patients with upper abdominal CT scan or plain scan. The results suggested that 25 cases (61%) cirrhosis, 14 cases (34.2%) suggest ascites, and there are 29 cases. In the liver and spleen, 7 cases (17.1%) showed portal hypertension, 19 cases (46.3%) were suggestive of esophageal varices, 16 cases (39%) were suggestive of varicose gastric fundus vein. There were 8 cases (19.5%) with esophageal gastroscope varicose and fundus varicose.8. in 26 patients with Fibro Scan examination. The results suggested that 9 patients (34.6%) were in the stage of cirrhosis. 14 (14) patients were in the period of cirrhosis. 1 patients with Mayo score, the mean value of 5.7 + 1.5.Mayo risk score and Fibro Scan staging have significant positive correlation (correlation coefficient 0.948, P value 0).9. male course 150 (30-361) compared with female group course 300 (60-1000) short, statistical difference (P= 0.023).AMA in female group (68.7%) detection rate is higher than male group (46.2%), difference There were statistical differences. There was no significant difference between the two groups in the clinical and biochemical examination. The.10.Ro-52 positive group was more prone to dry mouth dry mouth, ascites, with statistical difference (P respectively 0.014 and 0.012); positive combination and Sjogren syndrome were more than negative groups, there were statistical differences (P=0.002); positive group; positive group The serum Ig G was higher than that of the negative group (P value 0.023), but CHE was lower (P=0.008).11. for the diagnostic value of esophageal varices, when the Mayo score was equal to 4.8, the platelet count was 130 x 109/L, the serum albumin 38.6g/L was the largest, and the three had a certain diagnostic value for the esophageal varices. Total bilirubin and coagulation were of certain value. The diagnostic value of venous varicose in the time of blood enzyme is limited. Conclusion: primary biliary cholangitis mainly involves middle-aged women, and female patients have longer course of disease. The main manifestations are jaundice, liver and spleen, asthenia, anorexia, abdominal discomfort, ascites, skin itching, dry mouth and dry mouth, symptoms lack specificity, early part of the patients have no obvious symptoms, to disease in early part of the patients to disease. Later complications of liver cirrhosis accompanied by autoimmune diseases such as Sjogren's syndrome. The most diagnostic value of the non invasive examination was the serum AMA/AMA-M2 antibody. The Ro-52 positive group was more likely to merge with Sjogren syndrome. The antibody in the patient's sera was more prone to abdominal water, and the Ro-52 negative patients may have lower liver function. When the Mayo score is greater than 4.8, the platelet count is less than 130 x 109/L, the patients with serum albumin less than 38.6g/L need to follow up the gastroscopy regularly. At present, the treatment is based on UCDA, and it is necessary to find a more effective and safe method for early diagnosis of early treatment.
【学位授予单位】：兰州大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R575.2
[Abstract]:Objective: To observe and analyze the clinical data of 141 patients diagnosed as primary biliary cholangitis (PBC) in our hospital (general data, clinical manifestation, blood biochemistry, autoantibody, imaging examination, etc.) and their characteristics, to explore the relationship between anti Ro-52 antibody and PBC, and to refer to the related indications of esophageal / gastric varices in PBC patients. Evaluation of the diagnostic value to further improve the understanding of the disease. Methods: 141 patients with complete cases of cholangitis diagnosed in our hospital in 2011, ~2016, ~2016 December, were diagnosed as general data, clinical symptoms and signs, clinical complications and complications, serum biochemical indexes, autoantibodies, and endoscopy. Review, imaging examination and Fibro Scan examination data. The same patient was hospitalized for the first time in hospitalization. Results: 1. men were 26 (18.4%) and 115 women (81.6%). The male and female ratio was 1:4.42, the average age of all cases was (57.8 + 10.8) years, the course of disease was 240 (60-700) days, and the hospital days were 11 (8-15) days.2. Ben. The common clinical manifestations of PBC patients: jaundice, hepatomegaly, asthenia, anorexia, upper abdominal discomfort, ascites, skin itching, dry mouth, edema of the lower extremities, and spider nevus of the liver, including electrolyte disorder (37 cases), gastrointestinal bleeding (20 cases), infection (14 cases), hepatic encephalopathy (2 cases); main complication: drying synthesis Syndrome (Sjogren's syndrome 11), diabetes (6 cases), Hashimoto's thyroiditis (5 cases), rheumatoid arthritis (1 cases), systemic lupus erythematosus (1 cases), adult STILL disease (1 cases), systemic sclerosis (1 cases) of.4.PBC patients with ALP and GGT elevation as the main [261.6 (155.5-489.1) U/L, 179.1 (81.0-430.5) U/L], and in the early stage of disease. Gao Xian was not significantly higher at the late stage; AST, ALT slightly elevated [69.0 (43.0-114.0) U/L, 54 (32.0-93.5) U/L], serum total bilirubin 30.5 (19.2-64.6) umol/L, the normal upper limit, the total bile acid TBA 20.1 (8.5-45.8) umol/L, 2 times higher than the normal upper limit, 3.1 (6) autoantibody examination, 91 cases (6) positive. 4.5%) AMA-M2 positive 120 cases (85.1%), 125 (88.7%) patients with ANA positive, 31 (22%) anti filal B antibody positive, 66 (46.8%) anti Ro-52 antibody positive, 22 cases (15.6%) SSA antibody positive, 15 (10.6%) SSB positive.6. research subjects in total 65 cases, there are esophageal varices in patients with esophageal varices and gastric fundus veins 8 cases (12.3%) showed ulcers and (or) erosion, 8 cases (12.3%) had portal hypertensive gastropathy, 29 cases (44.6%) showed chronic gastritis.8 (12.3%) with esophageal varices and fundus varicose.7. in 118 patients with abdominal ultrasound, 98 (83.1%) liver diffuse lesions, 38 (32.2%) ascites, 81 cases (68.6%)). In hepatomegaly, 35 cases (29.7%) showed portal / splenic vein widening or decreased flow rate, 5 cases (4.2%) cholestasis, 4 cases (3.4%) indicating portal vein thrombosis; 6 cases (5.1%) ultrasound examination normal. There were 41 patients with upper abdominal CT scan or plain scan. The results suggested that 25 cases (61%) cirrhosis, 14 cases (34.2%) suggest ascites, and there are 29 cases. In the liver and spleen, 7 cases (17.1%) showed portal hypertension, 19 cases (46.3%) were suggestive of esophageal varices, 16 cases (39%) were suggestive of varicose gastric fundus vein. There were 8 cases (19.5%) with esophageal gastroscope varicose and fundus varicose.8. in 26 patients with Fibro Scan examination. The results suggested that 9 patients (34.6%) were in the stage of cirrhosis. 14 (14) patients were in the period of cirrhosis. 1 patients with Mayo score, the mean value of 5.7 + 1.5.Mayo risk score and Fibro Scan staging have significant positive correlation (correlation coefficient 0.948, P value 0).9. male course 150 (30-361) compared with female group course 300 (60-1000) short, statistical difference (P= 0.023).AMA in female group (68.7%) detection rate is higher than male group (46.2%), difference There were statistical differences. There was no significant difference between the two groups in the clinical and biochemical examination. The.10.Ro-52 positive group was more prone to dry mouth dry mouth, ascites, with statistical difference (P respectively 0.014 and 0.012); positive combination and Sjogren syndrome were more than negative groups, there were statistical differences (P=0.002); positive group; positive group The serum Ig G was higher than that of the negative group (P value 0.023), but CHE was lower (P=0.008).11. for the diagnostic value of esophageal varices, when the Mayo score was equal to 4.8, the platelet count was 130 x 109/L, the serum albumin 38.6g/L was the largest, and the three had a certain diagnostic value for the esophageal varices. Total bilirubin and coagulation were of certain value. The diagnostic value of venous varicose in the time of blood enzyme is limited. Conclusion: primary biliary cholangitis mainly involves middle-aged women, and female patients have longer course of disease. The main manifestations are jaundice, liver and spleen, asthenia, anorexia, abdominal discomfort, ascites, skin itching, dry mouth and dry mouth, symptoms lack specificity, early part of the patients have no obvious symptoms, to disease in early part of the patients to disease. Later complications of liver cirrhosis accompanied by autoimmune diseases such as Sjogren's syndrome. The most diagnostic value of the non invasive examination was the serum AMA/AMA-M2 antibody. The Ro-52 positive group was more likely to merge with Sjogren syndrome. The antibody in the patient's sera was more prone to abdominal water, and the Ro-52 negative patients may have lower liver function. When the Mayo score is greater than 4.8, the platelet count is less than 130 x 109/L, the patients with serum albumin less than 38.6g/L need to follow up the gastroscopy regularly. At present, the treatment is based on UCDA, and it is necessary to find a more effective and safe method for early diagnosis of early treatment.
【学位授予单位】：兰州大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R575.2
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