{"title":"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","authors":"Menghua Zhu, Hongyu Li, Wei Zhou, Wei Wang, Yue Yin, Shixue Xu, Kai Yu, Xingshun Qi","doi":"10.21037/tgh-21-111","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Case description: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":23267,"journal":{"name":"Translational gastroenterology and hepatology","volume":null,"pages":null},"PeriodicalIF":3.0000,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/c6/9d/tgh-08-21-111.PMC10184037.pdf","citationCount":"0","resultStr":"{\"title\":\"Relapsing immunoglobulin G4-related sclerosing cholangitis during maintenance treatment with low-dose steroids: a case report.\",\"authors\":\"Menghua Zhu, Hongyu Li, Wei Zhou, Wei Wang, Yue Yin, Shixue Xu, Kai Yu, Xingshun Qi\",\"doi\":\"10.21037/tgh-21-111\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>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.</p><p><strong>Case description: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>\",\"PeriodicalId\":23267,\"journal\":{\"name\":\"Translational gastroenterology and hepatology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":3.0000,\"publicationDate\":\"2023-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/c6/9d/tgh-08-21-111.PMC10184037.pdf\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Translational gastroenterology and hepatology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.21037/tgh-21-111\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Translational gastroenterology and hepatology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.21037/tgh-21-111","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"Medicine","Score":null,"Total":0}
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.
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.