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Co-occurrence of Wilsons disease and systemic lupus erythematosus: a case report and literature review

BACKGROUND: Wilsons disease (WD) is a rare autosomal recessive disease associated with defective biliary excretion of copper. The simultaneous occurrence of WD and systemic lupus erythematosus (SLE) has seldom been reported. Therefore, this study aimed to report the co-occurrence of SLE and WD with...

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Autores principales: Xu, Lishan, Liu, Bin, Liu, Zhaoyang, Tang, Ning, She, Chunhui, Wang, Jing, Zang, Bo, Yang, Yifei
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8139024/
https://www.ncbi.nlm.nih.gov/pubmed/34020599
http://dx.doi.org/10.1186/s12876-021-01814-5
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author Xu, Lishan
Liu, Bin
Liu, Zhaoyang
Tang, Ning
She, Chunhui
Wang, Jing
Zang, Bo
Yang, Yifei
author_facet Xu, Lishan
Liu, Bin
Liu, Zhaoyang
Tang, Ning
She, Chunhui
Wang, Jing
Zang, Bo
Yang, Yifei
author_sort Xu, Lishan
collection PubMed
description BACKGROUND: Wilsons disease (WD) is a rare autosomal recessive disease associated with defective biliary excretion of copper. The simultaneous occurrence of WD and systemic lupus erythematosus (SLE) has seldom been reported. Therefore, this study aimed to report the co-occurrence of SLE and WD with hepatic involvement in a patient so as to improve the understanding of the coexistence of these two conditions. CASE PRESENTATION: A 35-year-old woman with SLE was found to have liver fibrosis during a routinely abdominal ultrasound examination. Her laboratory evaluation showed low serum ceruloplasmin and high 24h urine copper levels. The slit-lamp examination revealed the presence of KayseriFleischer ring in her cornea. Liver biopsy demonstrated the enlargement of the portal area with hyperplasia of the fibrous tissue, infiltration of lymphoid plasma cells, swelling of hepatocytes, and steatosis, demonstrating liver fibrosis. Ensuing genetic testing confirmed the diagnosis of WD. CONCLUSIONS: Clinicians should bear in mind that unexplained liver fibrosis in patients with SLE may be related to WD, so as to avoid a missed or delayed diagnosis.
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spelling pubmed-81390242021-05-21 Co-occurrence of Wilsons disease and systemic lupus erythematosus: a case report and literature review Xu, Lishan Liu, Bin Liu, Zhaoyang Tang, Ning She, Chunhui Wang, Jing Zang, Bo Yang, Yifei BMC Gastroenterol Case Report BACKGROUND: Wilsons disease (WD) is a rare autosomal recessive disease associated with defective biliary excretion of copper. The simultaneous occurrence of WD and systemic lupus erythematosus (SLE) has seldom been reported. Therefore, this study aimed to report the co-occurrence of SLE and WD with hepatic involvement in a patient so as to improve the understanding of the coexistence of these two conditions. CASE PRESENTATION: A 35-year-old woman with SLE was found to have liver fibrosis during a routinely abdominal ultrasound examination. Her laboratory evaluation showed low serum ceruloplasmin and high 24h urine copper levels. The slit-lamp examination revealed the presence of KayseriFleischer ring in her cornea. Liver biopsy demonstrated the enlargement of the portal area with hyperplasia of the fibrous tissue, infiltration of lymphoid plasma cells, swelling of hepatocytes, and steatosis, demonstrating liver fibrosis. Ensuing genetic testing confirmed the diagnosis of WD. CONCLUSIONS: Clinicians should bear in mind that unexplained liver fibrosis in patients with SLE may be related to WD, so as to avoid a missed or delayed diagnosis. BioMed Central 2021-05-21 /pmc/articles/PMC8139024/ /pubmed/34020599 http://dx.doi.org/10.1186/s12876-021-01814-5 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Xu, Lishan
Liu, Bin
Liu, Zhaoyang
Tang, Ning
She, Chunhui
Wang, Jing
Zang, Bo
Yang, Yifei
Co-occurrence of Wilsons disease and systemic lupus erythematosus: a case report and literature review
title Co-occurrence of Wilsons disease and systemic lupus erythematosus: a case report and literature review
title_full Co-occurrence of Wilsons disease and systemic lupus erythematosus: a case report and literature review
title_fullStr Co-occurrence of Wilsons disease and systemic lupus erythematosus: a case report and literature review
title_full_unstemmed Co-occurrence of Wilsons disease and systemic lupus erythematosus: a case report and literature review
title_short Co-occurrence of Wilsons disease and systemic lupus erythematosus: a case report and literature review
title_sort co-occurrence of wilsons disease and systemic lupus erythematosus: a case report and literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8139024/
https://www.ncbi.nlm.nih.gov/pubmed/34020599
http://dx.doi.org/10.1186/s12876-021-01814-5
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