Cho Sung Hyun, Song Tae Jun, Park Jin-Seok, Yoon Jai Hoon, Yang Min Jae, Yoon Seung Bae, Lee Jae Min, Lee Yun Nah, Kim Seong-Hun, Choi Eun Kwang, Park Se Woo, Oh Dongwook, Park Do Hyun, Lee Sang Soo, Seo Dong-Wan, Lee Sung Koo, Kim Myung-Hwan
Department of Gastroenterology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea.
Division of Gastroenterology, Department of Internal Medicine, Inha University School of Medicine, Incheon, South Korea.
J Gastroenterol Hepatol. 2023 Apr;38(4):648-655. doi: 10.1111/jgh.16136. Epub 2023 Feb 9.
Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is considered a biliary manifestation of IgG4-related diseases. However, there has been a controversy on the clinical outcomes according to the location of the involved bile duct. We therefore compared the clinical outcomes and long-term prognosis of IgG4-SC with proximal bile duct involvement (proximal IgG4-SC) and IgG4-SC with distal bile duct involvement (distal IgG4-SC).
We reviewed the data of patients with IgG4-SC that were prospectively collected at 10 tertiary centers between March 2002 and October 2020. Clinical manifestations, outcomes, association with autoimmune pancreatitis (AIP), steroid-responsiveness, and relapse of IgG4-SC were evaluated.
A total of 148 patients (proximal IgG4-SC, n = 59; distal IgG4-SC, n = 89) were analyzed. The median age was 65 years (IQR, 56.25-71), and 86% were male. The two groups were similar in terms of jaundice at initial presentation (51% vs 65%; P = 0.082) and presence of elevated serum IgG4 (66% vs 70%; P = 0.649). The two groups showed significant differences in terms of steroid-responsiveness (91% vs 100%; P = 0.008), association with AIP (75% vs 99%; P = 0.001), and occurrence of liver cirrhosis (9% vs 1%; P = 0.034). During a median follow-up of 64 months (IQR, 21.9-84.7), the cumulative relapse-free survival was significantly different between the two groups (67% vs 79% at 5 years; P = 0.035).
Relapse of IgG4-SC frequently occurred during follow-up. Proximal IgG4-SC and distal IgG4-SC had different long-term outcomes in terms of steroid-responsiveness, occurrence of liver cirrhosis, and recurrence. It may be advantageous to determine the therapeutic and follow-up strategies according to the location of bile duct involvement.
免疫球蛋白G4相关性硬化性胆管炎（IgG4-SC）被认为是IgG4相关性疾病的一种胆道表现。然而，根据受累胆管的位置，临床结局存在争议。因此，我们比较了近端胆管受累的IgG4-SC（近端IgG4-SC）和远端胆管受累的IgG4-SC（远端IgG4-SC）的临床结局和长期预后。
我们回顾了2002年3月至2020年10月期间在10个三级中心前瞻性收集的IgG4-SC患者的数据。评估了临床表现、结局、与自身免疫性胰腺炎（AIP）的关联、类固醇反应性以及IgG4-SC的复发情况。
共分析了148例患者（近端IgG4-SC，n = 59；远端IgG4-SC，n = 89）。中位年龄为65岁（四分位间距，56.25 - 71），86%为男性。两组在初次就诊时的黄疸情况（51%对65%；P = 0.082）和血清IgG4升高情况（66%对70%；P = 0.649）方面相似。两组在类固醇反应性（91%对100%；P = 0.008）、与AIP的关联（75%对99%；P = 0.001）以及肝硬化的发生率（9%对1%；P = 0.034）方面存在显著差异。在中位随访64个月（四分位间距，21.9 - 84.7）期间，两组的累积无复发生存率有显著差异（5年时分别为67%对79%；P = 0.035）。
IgG4-SC在随访期间频繁复发。近端IgG4-SC和远端IgG4-SC在类固醇反应性、肝硬化的发生率和复发方面有不同的长期结局。根据胆管受累位置确定治疗和随访策略可能是有益的。
