Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.
Department of General Medicine, Hiroshima University, Graduate School of Medical Science, Hiroshima, Japan.
Clin Gastroenterol Hepatol. 2017 Jun;15(6):920-926.e3. doi: 10.1016/j.cgh.2016.12.038. Epub 2017 Jan 20.
BACKGROUND & AIMS: Immunoglobulin G4 sclerosing cholangitis (IgG4-SC) is a biliary tract manifestation of IgG4-related systemic disease. We investigated the demographics, clinical features at presentation, treatment response, and outcomes of IgG4-SC using data from a large-scale survey in Japan.
We performed a retrospective cohort study of 527 patients with IgG4-SC (436 female; median age, 66.2 years; range, 23-89 years) in Japan from 2000 to 2015. Data on patient demographics, presentation, treatment response, and outcomes were collected from questionnaires given to patients at 211 referral centers in Japan in 2015. Patients were diagnosed with IgG4-SC based on the clinical diagnostic criteria established by the Japanese Biliary Association in 2012. Patients were followed for a median of 4.1 ± 3.1 years. Survival was assessed using the Kaplan-Meier curve and log-rank test.
Symptoms at presentation included jaundice (35% of patients) and pruritus (13% of patients); 28% of patients were asymptomatic. It was extremely rare for patients with IgG4-SC to present with symptoms of decompensated cirrhosis. At presentation, serum levels of IgG4 were increased (>135 mg/dL) in 84% of patients. Response to prednisolone was excellent in 90% of patients. No patients received liver transplants, and cholangiocarcinoma was found in only 4 patients (0.7%). Restenosis of bile ducts was observed in 19% of patients but did not affect overall survival.
In a retrospective cohort study of 527 patients with IgG4-SC in Japan, we found the disease to be benign. Most patients (90%) respond to treatment with prednisolone and few develop decompensated cirrhosis or cholangiocarcinoma.
免疫球蛋白 G4 硬化性胆管炎（IgG4-SC）是 IgG4 相关系统性疾病的胆道表现。我们利用日本一项大规模调查的数据，研究了 IgG4-SC 的人口统计学、起病时的临床特征、治疗反应和结局。
我们对 2000 年至 2015 年期间日本的 527 例 IgG4-SC 患者（436 例女性；中位年龄 66.2 岁；范围 23-89 岁）进行了回顾性队列研究。从 2015 年日本 211 家转诊中心的患者问卷调查中收集了患者的人口统计学、表现、治疗反应和结局数据。根据日本胆道协会 2012 年建立的临床诊断标准，诊断 IgG4-SC。对患者进行了中位 4.1±3.1 年的随访。采用 Kaplan-Meier 曲线和对数秩检验评估生存情况。
起病时的症状包括黄疸（35%的患者）和瘙痒（13%的患者）；28%的患者无症状。IgG4-SC 患者极罕见出现失代偿性肝硬化的症状。起病时，84%的患者血清 IgG4 水平升高（>135mg/dL）。90%的患者对泼尼松龙治疗反应良好。没有患者接受肝移植，仅 4 例（0.7%）患者发现胆管癌。19%的患者出现胆管再狭窄，但不影响总体生存。
在日本一项对 527 例 IgG4-SC 患者的回顾性队列研究中，我们发现该疾病为良性。大多数患者（90%）对泼尼松龙治疗有反应，很少发展为失代偿性肝硬化或胆管癌。
