临床肝胆病杂志2018，Vol.34Issue(6)：1253-1258,6.DOI:10.3969/j.issn.1001-5256.2018.06.024
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
王艳 1潘静 1霍娜 1张真真 1程浩 1王贵强1
作者信息
- 1. 北京大学第一医院感染疾病科,北京100034
- 折叠
摘要
Abstract
Objective To 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 .Methods A 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.Conclusion IgG4 -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 jaun - dice 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 .关键词
胆管炎,硬化性/免疫球蛋白G/疾病特征Key words
cholangitis/sclerosing/immunoglobulin G/disease attributes分类
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王艳,潘静,霍娜,张真真,程浩,王贵强..12例IgG4相关硬化性胆管炎临床特征分析[J].临床肝胆病杂志,2018,34(6):1253-1258,6.
