12例IgG4相关硬化性胆管炎临床特征分析

王艳; 潘静; 霍娜; 张真真; 程浩; 王贵强

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

DOI: 10.3969/j.issn.1001-5256.2018.06.024

北京大学第一医院感染疾病科

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中图分类号: R575.7
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出版历程
- 收稿日期: 2017-11-20
- 出版日期: 2018-06-20

Clinical features of IgG4-associated sclerosing cholangitis: An analysis of 12 cases

Department of Infectious Diseases, Peking University First Hospital

摘要

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（55453 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1.518.9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46周后取出胆管内支...
- 胆管炎,硬化性 /
- 免疫球蛋白G /
- 疾病特征
Abstract:
Objective To investigate the clinical features, steroid response, and recurrence of Ig G4-associated sclerosing cholangitis (Ig G4-SC) , and to shorten the time for clinical diagnosis and reduce recurrence rate. Methods A total of 12 patients with Ig G4-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 Ig G4-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 Ig G4 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 Ig G4-SC needs a long time for prognosis, with frequent misdiagnosis and missed diagnosis. Serum Ig G4 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.
- cholangitis, sclerosing /
- immunoglobulin G /
- disease attributes

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