低剂量类固醇维持治疗期间复发的免疫球蛋白g4相关的硬化性胆管炎1例报告

IF 3 4区 医学 Q1 Medicine
Menghua Zhu, Hongyu Li, Wei Zhou, Wei Wang, Yue Yin, Shixue Xu, Kai Yu, Xingshun Qi
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引用次数: 0

摘要

背景:免疫球蛋白g4相关的硬化性胆管炎(IgG4-SC)通常对初始类固醇治疗有良好的反应,但在随访期间复发率高。了解IgG4-SC复发的预测因素和治疗策略具有重要意义。病例描述:在本文中,我们报道了一名57岁男性在发病时被诊断为IgG4-SC并伴有1型自身免疫性胰腺炎(AIP),并对类固醇治疗有良好反应。然而,在低剂量类固醇维持治疗期间,IgG4-SC复发,临床表现与严重胆管狭窄有关,但在再次给予全剂量类固醇后迅速改善,黄疸消退,肝内和肝外胆管狭窄改善,肝功能逐渐恢复。在2021年12月的最后一次随访中,他仍然稳定服用甲基强的松龙片剂,剂量为4 mg/天。结论:在初始发病时血清IgG4水平较高并接受低剂量类固醇维持治疗的患者中,IgG4- sc有复发的可能。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,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。再给药或增加剂量类固醇、免疫抑制剂和利妥昔单抗对治疗复发性疾病有效。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Relapsing immunoglobulin G4-related sclerosing cholangitis during maintenance treatment with low-dose steroids: a case report.

Relapsing immunoglobulin G4-related sclerosing cholangitis during maintenance treatment with low-dose steroids: a case report.

Relapsing immunoglobulin G4-related sclerosing cholangitis during maintenance treatment with low-dose steroids: a case report.

Relapsing immunoglobulin G4-related sclerosing cholangitis during maintenance treatment with low-dose steroids: a case report.

Background: 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.

Case description: 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.

Conclusions: 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.

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来源期刊
CiteScore
8.20
自引率
0.00%
发文量
1
期刊介绍: Translational Gastroenterology and Hepatology (Transl Gastroenterol Hepatol; TGH; Online ISSN 2415-1289) is an open-access, peer-reviewed online journal that focuses on cutting-edge findings in the field of translational research in gastroenterology and hepatology and provides current and practical information on diagnosis, prevention and clinical investigations of gastrointestinal, pancreas, gallbladder and hepatic diseases. Specific areas of interest include, but not limited to, multimodality therapy, biomarkers, imaging, biology, pathology, and technical advances related to gastrointestinal and hepatic diseases. Contributions pertinent to gastroenterology and hepatology are also included from related fields such as nutrition, surgery, public health, human genetics, basic sciences, education, sociology, and nursing.
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