Zhu Menghua, Li Hongyu, Zhou Wei, Wang Wei, Yin Yue, Xu Shixue, Yu Kai, Qi Xingshun
Department of Gastroenterology, General Hospital of Northern Theater Command, Shenyang, China.
Postgraduate College, Jinzhou Medical University, Jinzhou, China.
Transl Gastroenterol Hepatol. 2022 Mar 17;8:22. doi: 10.21037/tgh-21-111. eCollection 2023.
Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) often has a good response to initial steroid therapy, but a high relapse rate during follow-up. Knowledge about the predictors and treatment strategy of relapsing IgG4-SC is of great significance.
In this paper, we reported that a 57-year-old male was diagnosed with IgG4-SC accompanied by type 1 autoimmune pancreatitis (AIP) at the first onset of his diseases and had a good response to steroid therapy. However, during low-dose steroids maintenance therapy, IgG4-SC relapsed with clinical presentations related to severe bile duct stricture, but improved rapidly after re-administration of full-dose steroids, accompanied by resolution of jaundice, improvement of intrahepatic and extrahepatic bile duct stricture, and gradual recovery of liver function. At the last follow-up in December 2021, he was still stable with methylprednisolone tablets at 4 mg/day.
IgG4-SC is likely to relapse in patients who have high serum IgG4 level at initial onset and receive low-dose steroids maintenance treatment. The predictors of disease relapse also include steroids interruption, more severe bile duct stricture, long duration from diagnosis to treatment, history of allergy, and high serum tumor necrosis factor-alpha (TNF-alpha) and soluble interleukin-2 receptor (sIL-2R) levels. Re-administration or up-dose of steroids, immunosuppressors, and rituximab are effective for treating relapsing disease.
免疫球蛋白G4相关性硬化性胆管炎（IgG4-SC）通常对初始类固醇治疗反应良好，但随访期间复发率较高。了解复发性IgG4-SC的预测因素和治疗策略具有重要意义。
在本文中，我们报告了一名57岁男性，在疾病首次发作时被诊断为IgG4-SC并伴有1型自身免疫性胰腺炎（AIP），对类固醇治疗反应良好。然而，在低剂量类固醇维持治疗期间，IgG4-SC复发，出现与严重胆管狭窄相关的临床表现，但在重新给予全剂量类固醇后迅速改善，伴有黄疸消退、肝内外胆管狭窄改善以及肝功能逐渐恢复。在2021年12月的最后一次随访中，他服用4毫克/天的甲泼尼龙片仍病情稳定。
初始发病时血清IgG4水平高且接受低剂量类固醇维持治疗的患者，IgG4-SC可能复发。疾病复发的预测因素还包括类固醇中断、更严重的胆管狭窄、从诊断到治疗的时间长、过敏史以及血清肿瘤坏死因子-α（TNF-α）和可溶性白细胞介素-2受体（sIL-2R）水平高。重新给予或增加剂量的类固醇、免疫抑制剂和利妥昔单抗对治疗复发性疾病有效。
