Cargando…
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...
Autores principales: | , , , , , , , |
---|---|
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 |
_version_ | 1783695921444290560 |
---|---|
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. |
format | Online Article Text |
id | pubmed-8139024 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT xulishan cooccurrenceofwilsonsdiseaseandsystemiclupuserythematosusacasereportandliteraturereview AT liubin cooccurrenceofwilsonsdiseaseandsystemiclupuserythematosusacasereportandliteraturereview AT liuzhaoyang cooccurrenceofwilsonsdiseaseandsystemiclupuserythematosusacasereportandliteraturereview AT tangning cooccurrenceofwilsonsdiseaseandsystemiclupuserythematosusacasereportandliteraturereview AT shechunhui cooccurrenceofwilsonsdiseaseandsystemiclupuserythematosusacasereportandliteraturereview AT wangjing cooccurrenceofwilsonsdiseaseandsystemiclupuserythematosusacasereportandliteraturereview AT zangbo cooccurrenceofwilsonsdiseaseandsystemiclupuserythematosusacasereportandliteraturereview AT yangyifei cooccurrenceofwilsonsdiseaseandsystemiclupuserythematosusacasereportandliteraturereview |