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A case report of immunoglobulin G4-related sclerosing cholangitis with multiple relapse
RATIONALE: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is classified as a biliary tract manifestation of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoid is the first-line therapy for most patients, but the optimal starting dose, adequate maintaining dose and withdrawal tim...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer Health
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5959437/ https://www.ncbi.nlm.nih.gov/pubmed/29742724 http://dx.doi.org/10.1097/MD.0000000000010700 |
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author | Dong, Xiaoqin Huo, Na Wu, Zhao Wang, Guiqiang Wang, He Zhao, Hong |
author_facet | Dong, Xiaoqin Huo, Na Wu, Zhao Wang, Guiqiang Wang, He Zhao, Hong |
author_sort | Dong, Xiaoqin |
collection | PubMed |
description | RATIONALE: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is classified as a biliary tract manifestation of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoid is the first-line therapy for most patients, but the optimal starting dose, adequate maintaining dose and withdrawal time remain disputable. PATIENT CONCERNS: An elderly male patient presented to our hospital with neoplasms of the bile duct and pancreas at first visit in December 2011. Further examination revealed bile duct stenosis and obstruction, and elevated serum IgG4 level. DIAGNOSES: A diagnosis of IgG4-SC was established by examination results and effectiveness of steroid therapy, although IgG4-positive plasma cells were seldom seen in the liver sample. INTERVENTIONS: Prednisolone was started from 40 mg daily, tapered gradually, and totally withdrawn after 22 months of treatment. OUTCOMES: A new-onset cholangitis was detected 2 months later. Prednisolone 10 mg daily was administered again. Prednisolone was reduced to 5 mg every other day without consultation with his doctor 1 year ago in May 2017, then he presented to our hospital again with recurrent abdominal pain and jaundice. LESSONS: IgG4-SC is a protean condition and can be distinguished from primary sclerosing cholangitis, malignancy, and other inflammatory disorders based on 4 clinical criteria. Serum IgG4/IgG1 ratio is a practicable diagnostic algorithm to distinguish PSC from IgG4-SC. The dose and duration of glucocorticoid for treatment should be adjusted according to clinical situations, and proper maintaining dose is essential for a better prognosis. |
format | Online Article Text |
id | pubmed-5959437 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Wolters Kluwer Health |
record_format | MEDLINE/PubMed |
spelling | pubmed-59594372018-05-24 A case report of immunoglobulin G4-related sclerosing cholangitis with multiple relapse Dong, Xiaoqin Huo, Na Wu, Zhao Wang, Guiqiang Wang, He Zhao, Hong Medicine (Baltimore) Research Article RATIONALE: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is classified as a biliary tract manifestation of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoid is the first-line therapy for most patients, but the optimal starting dose, adequate maintaining dose and withdrawal time remain disputable. PATIENT CONCERNS: An elderly male patient presented to our hospital with neoplasms of the bile duct and pancreas at first visit in December 2011. Further examination revealed bile duct stenosis and obstruction, and elevated serum IgG4 level. DIAGNOSES: A diagnosis of IgG4-SC was established by examination results and effectiveness of steroid therapy, although IgG4-positive plasma cells were seldom seen in the liver sample. INTERVENTIONS: Prednisolone was started from 40 mg daily, tapered gradually, and totally withdrawn after 22 months of treatment. OUTCOMES: A new-onset cholangitis was detected 2 months later. Prednisolone 10 mg daily was administered again. Prednisolone was reduced to 5 mg every other day without consultation with his doctor 1 year ago in May 2017, then he presented to our hospital again with recurrent abdominal pain and jaundice. LESSONS: IgG4-SC is a protean condition and can be distinguished from primary sclerosing cholangitis, malignancy, and other inflammatory disorders based on 4 clinical criteria. Serum IgG4/IgG1 ratio is a practicable diagnostic algorithm to distinguish PSC from IgG4-SC. The dose and duration of glucocorticoid for treatment should be adjusted according to clinical situations, and proper maintaining dose is essential for a better prognosis. Wolters Kluwer Health 2018-05-11 /pmc/articles/PMC5959437/ /pubmed/29742724 http://dx.doi.org/10.1097/MD.0000000000010700 Text en Copyright © 2018 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by-nc/4.0 This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc/4.0 |
spellingShingle | Research Article Dong, Xiaoqin Huo, Na Wu, Zhao Wang, Guiqiang Wang, He Zhao, Hong A case report of immunoglobulin G4-related sclerosing cholangitis with multiple relapse |
title | A case report of immunoglobulin G4-related sclerosing cholangitis with multiple relapse |
title_full | A case report of immunoglobulin G4-related sclerosing cholangitis with multiple relapse |
title_fullStr | A case report of immunoglobulin G4-related sclerosing cholangitis with multiple relapse |
title_full_unstemmed | A case report of immunoglobulin G4-related sclerosing cholangitis with multiple relapse |
title_short | A case report of immunoglobulin G4-related sclerosing cholangitis with multiple relapse |
title_sort | case report of immunoglobulin g4-related sclerosing cholangitis with multiple relapse |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5959437/ https://www.ncbi.nlm.nih.gov/pubmed/29742724 http://dx.doi.org/10.1097/MD.0000000000010700 |
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