IgG4相关硬化性胆管炎34例临床分析  

Immunoglobulin G4-related sclerosing cholangitis in 34 patients

作　　者：王天龙[1] 吴刚[2] WANG Tianlong;WU Gang(Department of Geriatric Surgery,the First Affiliated Hospital of China Medical University,Shenyang110001,China;Department of Hepatobiliary Surgery,the First Affiliated Hospital of China Medical University,Shenyang110001,China)

机构地区：[1]中国医科大学附属第一医院老年外科,沈阳110001 [2]中国医科大学附属第一医院肝胆外科,沈阳110001

出　　处：《中华实用诊断与治疗杂志》2019年第9期887-890,共4页Journal of Chinese Practical Diagnosis and Therapy

基　　金：辽宁省科技厅科学技术计划项目(2011225020);沈阳市科技项目(F11-262-9-22)

摘　　要：目的总结IgG4相关硬化性胆管炎(immunoglobulin G4-related sclerosing cholangitis,ISC)的临床特征。方法回顾性分析34例ISC患者的临床资料。结果34例患者临床表现为黄疸25例,上腹部疼痛不适17例,乏力11例,纳差及体质量减轻8例,皮肤瘙痒7例,发热4例,腹泻2例;影像学检查显示病灶位于肝门部胆管6例,远端胆管24例,肝内外胆管4例,伴胰腺弥漫性或局限性肿大23例,肾脏低密度病灶9例,肺结节及斑片影6例,腹腔内肿大淋巴结8例,腹膜后纤维化4例;血清学检查显示IgG4、碱性磷酸酶、γ-谷氨酰转肽酶水平均有不同程度增高,血清总胆红素水平增高28例,抗核抗体、抗线粒体抗体低滴度阳性5例,糖链抗原19-9增高8例;12例行胆管毛刷刷检,均为阴性;3例拟诊肝门部胆管癌者行肝门部胆管切除+胆肠吻合术,1例拟诊胰头癌者行胰十二指肠切除术,术后组织病理证实检查为ISC,给予醋酸泼尼松治疗,余30例明确ISC诊断者给予醋酸泼尼松治疗(其中4例醋酸泼尼松治疗初期出现重度黄疸行胆管内支架置入术),症状均明显好转;随访6~48个月,4例停用醋酸泼尼松后复发,再次应用醋酸泼尼松或联合免疫抑制剂治疗,症状好转。结论ISC临床表现缺乏特异性,与胆管癌、胰腺癌鉴别困难,综合分析临床表现、影像学表现、血清学指标、组织病理学及糖皮质激素治疗反应可明确诊断。Objective To summarize the clinical characteristics of immunoglobulin G4(IgG4)-related sclerosing cholangitis(ISC).Methods The clinical data of 34patients with ISC were retrospectively analyzed.Results The clinical manifestations of 34patients were jaundice in 25patients,upper abdominal pain and discomfort in 17,fatigue in 11,anorexia and body mass loss in 8,pruritus in 7,fever in 4and diarrhea in 2.Imaging examination showed that the lesions were located in the hilar bile duct in 6patients,distal bile duct in 24,intrahepatic and extrahepatic bile ducts in 4,diffuse or localized pancreatic enlargement in 23,renal low density focus in 9,pulmonary nodules and patchy shadows in 6,intraperitoneal enlarged lymph nodes in 8and retroperitoneal fibrosis in 4.The serological examination showed that IgG4,alkaline phosphatase and gamma-glutamyl transferase increased in various degrees in all patients,serum total bilirubin level increased in 28patients,anti-nuclear antibody and anti-mitochondrial antibody low titers were positive in 5and carbohydrate antigen 19-9increased in 8.Twelve patients underwent biliary tract brush examination,showing negative.Three patients with suspected hilar cholangiocarcinoma underwent hilar cholangiotomy plus choledochojejunostomy,and 1 patient with suspected pancreatic head cancer underwent pancreaticoduodenectomy,all of which were pathologically confirmed ISC after operation and were given prednisone treatment;the other 30patients with definite diagnosis of ISC were treated with prednisone,in which 4patients with severe jaundice occuring in the early stage of prednisone treatment underwent biliary stent implantation and the symptoms were markedly improved.In 6-to 48-month follow-up,relapse occurred in 4patients after withdrawal of prednisone and the symptoms were improved after retreatment with prednisone or combined immunosuppressive agents.Conclusion The clinical manifestations of ISC are lack of specificity,therefore it is difficult to differentiate from cholangiocarcinoma and pancreatic ca

关 键 词：IgG4相关硬化性胆管炎 黄疸 胆管内支架置入术 

分 类 号：R73[医药卫生—肿瘤]