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12例IgG4相关硬化性胆管炎临床特征分析
Clinical features of IgG4-associated sclerosing cholangitis: An analysis of 12 cases
文章发布日期:2018年05月07日  来源:  作者:王艳,潘静,霍娜,等  点击次数:318次  下载次数:44次

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【摘要】:目的探讨分析IgG4相关硬化性胆管炎(IgG4-SC)的临床特征以及激素应答与复发情况,帮助缩短临床诊断时间并降低复发率。方法收集2011年12月-2017年3月北京大学第一医院收治的IgG4-SC患者12例,均符合2012年IgG4-SC临床诊断标准。回顾性分析临床资料、临床特点以及随访资料。结果12例确诊患者,男女比例11∶1。平均发病年龄为(61.7±8.6)岁,平均确诊时间24.2个月。其中11例(92%)患者出现黄疸,血清TBil平均水平为(198.7±57.7)μmol/L。血清ALT水平均有不同程度升高(55~453 U/L);5例(41.7%)患者急性腹痛,CA19-9平均水平(60.8±12.5)U/ml。血清IgG4升高1.5~18.9倍,平均(7.7±2.0)g/L。12例患者磁共振胰胆管造影均显示典型的胆管壁增厚、管腔狭窄,肝内外胆管扩张等表现,合并自身免疫性胰腺炎病变的有11例(92%),同时累及3个以上脏器的2例(16.7%)。2例治疗前接受胆管内支架置入术的患者均在激素使用4~6周后取出胆管内支架。平均激素应答时间5.4个月。其中4例(50%,4/8)在激素减量过程中出现复发,2例发生在激素停药后8~12个月。结论IgG4-SC诊断需要时间比较长,常易误诊、漏诊。老年男性急起黄疸,磁共振胰胆管造影结果提示胆管狭窄并管壁增厚伴胰腺受累,需要筛查血清IgG4水平。一旦确诊IgG4-SC,尽早激素治疗,激素应答至少需要5~6个月,需缓慢减量并长期密切监测复发及肿瘤发生。
【Abstract】:ObjectiveTo investigate the clinical features, steroid response, and recurrence of IgG4-associated sclerosing cholangitis (IgG4-SC), and to shorten the time for clinical diagnosis and reduce recurrence rate. MethodsA total of 12 patients with IgG4-SC who were admitted to Peking University First Hospital from December 2011 to March 2017 were enrolled, and they all met the clinical diagnostic criteria for IgG4-SC in 2012. A retrospective analysis was performed for their clinical data, clinical features, and follow-up data. Results Of all 12 patients, the male/female ratio was 11∶1. The mean age of onset was 61.7±8.6 years, and the mean time to a confirmed diagnosis was 24.2 months. Of all 12 patients, 11 (92%) had jaundice, with a mean serum total bilirubin level of 198.7±57.7 μmol/L. There were varying degrees of increase in serum alanine aminotransferase level (range 55-453 U/L). Of all patients, 5 (41.7%) had acute abdominal pain, with a mean CA19-9 level of 60.8±12.5 U/ml. Serum IgG4 was increased by 1.5-18.9 times, with a mean level of 7.7±20 g/L. Magnetic resonance cholangiopancreatography (MRCP) performed for all patients showed typical manifestations including bile duct wall thickening, lumen stenosis, and intrahepatic and extrahepatic bile duct dilatation; 11 patients (92%) were found to have autoimmune pancreatitis, and 2 (16.7%) had involvement of more than 3 visceral organs. In two patients who underwent biliary stent implantation before treatment, the biliary stent was removed after 4-6 weeks of steroid treatment. The mean duration of steroid response was 5.4 months. Among the 8 patients who underwent steroid treatment, 4 (50%,4/8) experienced recurrence during the reduction of the dose of steroid, among whom 2 experienced recurrence within 8-12 weeks after drug withdrawal. ConclusionIgG4-SC needs a long time for prognosis, with frequent misdiagnosis and missed diagnosis. Serum IgG4 level should be measured for elderly male patients with an acute onset of jaundice and bile duct stenosis, bile duct wall thickening, and involvement of the pancreas on MRCP. Steroid treatment should be given as early as possible after diagnosis. Patients may need at least 5-6 months to achieve steroid response. The dose of steroid should be reduced slowly and recurrence and tumorigenesis should be closely monitored.
【关键字】:胆管炎, 硬化性; 免疫球蛋白G; 疾病特征
【Key words】:cholangitis, sclerosing; immunoglobulin G; disease attributes
【引证本文】:
WANG Y, PAN J, HUO N, et al. Clinical features of IgG4-associated sclerosing cholangitis: An analysis of 12 cases[J]. J Clin Hepatol, 2018, 34(6): 1253-1258. (in Chinese) 王艳, 潘静, 霍娜, 等. 12例IgG4相关硬化性胆管炎临床病例特征分析[J]. 临床肝胆病杂志, 2018, 34(6): 1253-1258.

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