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目的 探讨IgG4相关硬化性胆管炎(IgG4-related sclerosing cholangitis, IgG4-SC)的临床特点 目的 2004年1月至2012年12月在北京协和医院住院治疗的IgG4-SC患者共36例, 均符合2012年日本学者提出的IgG4-SC临床诊断标准的确诊要求。回顾性分析其临床资料, 包括症状、实验室检查、影像学检查、病理结果和随访情况 结果 36例患者性别比(女/男)为0.24:1, 平均发病年龄为(62.8±9.2)岁。最常见的临床症状为黄疸(77.8%, 28/36)和腹痛(50.0%, 18/36)。8例(22.2%)患者的胆红素水平在正常范围, 其中3例谷氨酰转肽酶水平在正常范围。超声内镜发现34例(94.4%)患者胆管增厚, 其检出率明显高于腹部超声(8.3%, 3/36)和腹部CT(33.3%, 12/36)(P < 0.05)。7例患者行胆管刷检或胆管活检, 病理结果均为阳性。随访超过2年的23例患者, 复发率为39.1%(9/23), 胆管外受累器官越多或受累胆管节段越多者, 越容易复发 结论 IgG4-SC多见于中老年男性, 符合慢性胆管炎的临床特点。少数患者临床没有任何胆管梗阻的征象。诊断IgG4-SC时, 胆管病理的阳性率并不理想, 影像学上非狭窄段的胆管壁均匀增厚对诊断十分有意义, 超声内镜应作为疑诊IgG4-SC患者的常规检查项目。对IgG4-SC患者应密切随访, 警惕复发及并存的恶性肿瘤。 -
关键词:
- IgG4相关性疾病 /
- IgG4相关硬化性胆管炎 /
- 超声内镜 /
- 诊断
Abstract:Objective To summarize the clinical features of IgG4-related sclerosing cholangitis (IgG4-SC). Methods Clinical data of 36 cases with IgG4-SC hospitalized in Peking Union Medical College Hospital in the period of January 2004 to December 2012 were retrospectively analyzed, including symptoms, laboratory tests, imaging results, pathological results and follow-up records. All the cases were diagnosed according to 2012 Japanese clinical diagnostic criteria of IgG4-SC. Results The sex ratio of the 36 IgG4-SC patients was 0.24:1. The mean age of onset was (62.8±9.2) years. The most common symptoms were jaundice (77.8%, 28/36) and abdominal pain (50.0%, 18/36). Serum bilirubin level was normal in 8 cases (22.2%), among whom serum gamma-glutamyl transpeptidase (GGT) level was also normal in 3 cases. Endoscopic ultrasound found bile duct wall thickening in 34 cases (94.4%), significantly more sensitive than abdominal ultrasound (8.3%, 3/36) and CT scan (33.3%, 12/36) (P < 0.05). Seven patients underwent brush cytology or biopsy of bile duct, and the pathological results were all negative. Relapses occurred in 39.1%(9/23) of the 23 cases who were followed up for over 2 years. The patients with more extrabiliary organs or more biliary segments involved were more prone to relapse. Conclusions IgG4-SC is primarily found in middle-aged and elderly men, with clinical manifestations of chronic cholangitis. However, some patients might have no signs of biliary obstruction. The sensitivity of biliary biopsy is poor. The radiological uniform bile duct wall thickening in non-stricture segments is highly suggestive of IgG4-SC. Endoscopic ultrasound should be used as a routine test in suspected cases. Close follow-up is necessary for IgG4-SC patients to detect relapse or concurrent malignancy. -
表 1 IgG4相关硬化性胆管炎复发相关危险因素分析
危险因素 例数 复发[例(%)] 未复发[例(%)] 其他受累器官数目 1 15 4(26.7) 11(73.3) ≥2 8 5(62.5) 3(37.5) 胆管受累节段数目 1 10 3(30.0) 7(70.0) ≥2 3 2(66.7) 1(33.3) 
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