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Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis

Sarcoidosis is a multisystem granulomatous disease of unknown origin. All organs may be affected. Liver involvement is common but it is rarely symptomatic. Only a few cases of Budd-Chiari syndrome (BCS) secondary to a hepatic sarcoidosis have been described so far. We describe a case of multisystemi...

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Autores principales: Ennaifer, Rym, Bacha, Dhouha, Romdhane, Hayfa, Cheikh, Myriam, Nejma, Houda Ben, BelHadj, Najet
Formato: Online Artículo Texto
Lenguaje:English
Publicado: PAGEPress Publications, Pavia, Italy 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4736049/
https://www.ncbi.nlm.nih.gov/pubmed/26900444
http://dx.doi.org/10.4081/cp.2015.768
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author Ennaifer, Rym
Bacha, Dhouha
Romdhane, Hayfa
Cheikh, Myriam
Nejma, Houda Ben
BelHadj, Najet
author_facet Ennaifer, Rym
Bacha, Dhouha
Romdhane, Hayfa
Cheikh, Myriam
Nejma, Houda Ben
BelHadj, Najet
author_sort Ennaifer, Rym
collection PubMed
description Sarcoidosis is a multisystem granulomatous disease of unknown origin. All organs may be affected. Liver involvement is common but it is rarely symptomatic. Only a few cases of Budd-Chiari syndrome (BCS) secondary to a hepatic sarcoidosis have been described so far. We describe a case of multisystemic sarcoidosis presenting with BCS. A 42-year old female was referred to our department for chronic and anicteric cholestasis. Laboratory and imaging investigations disclosed features of chronic BCS associated with multisystemic sarcoidosis. The positive diagnosis was based on microscopic features, which showed hepatic, gastric and cutaneous non-caseating granulomas. Screening for an underlying thrombophilic disorder was negative. The diagnosis of BCS complicating hepatic sarcoidosis was the most likely. She was put on corticosteroids and anticoagulation therapy. To our knowledge, few cases of sarcoidosis-related BCS have been reported in the literature. In addition to being an uncommon presentation of sarcoidosis, this case illustrates the importance of recognizing an unusual cause of BCS and its therapeutic difficulties.
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spelling pubmed-47360492016-02-19 Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis Ennaifer, Rym Bacha, Dhouha Romdhane, Hayfa Cheikh, Myriam Nejma, Houda Ben BelHadj, Najet Clin Pract Case Report Sarcoidosis is a multisystem granulomatous disease of unknown origin. All organs may be affected. Liver involvement is common but it is rarely symptomatic. Only a few cases of Budd-Chiari syndrome (BCS) secondary to a hepatic sarcoidosis have been described so far. We describe a case of multisystemic sarcoidosis presenting with BCS. A 42-year old female was referred to our department for chronic and anicteric cholestasis. Laboratory and imaging investigations disclosed features of chronic BCS associated with multisystemic sarcoidosis. The positive diagnosis was based on microscopic features, which showed hepatic, gastric and cutaneous non-caseating granulomas. Screening for an underlying thrombophilic disorder was negative. The diagnosis of BCS complicating hepatic sarcoidosis was the most likely. She was put on corticosteroids and anticoagulation therapy. To our knowledge, few cases of sarcoidosis-related BCS have been reported in the literature. In addition to being an uncommon presentation of sarcoidosis, this case illustrates the importance of recognizing an unusual cause of BCS and its therapeutic difficulties. PAGEPress Publications, Pavia, Italy 2015-12-21 /pmc/articles/PMC4736049/ /pubmed/26900444 http://dx.doi.org/10.4081/cp.2015.768 Text en ©Copyright R. Ennaifer et al. http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Ennaifer, Rym
Bacha, Dhouha
Romdhane, Hayfa
Cheikh, Myriam
Nejma, Houda Ben
BelHadj, Najet
Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis
title Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis
title_full Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis
title_fullStr Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis
title_full_unstemmed Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis
title_short Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis
title_sort budd-chiari syndrome: an unusual presentation of multisystemic sarcoidosis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4736049/
https://www.ncbi.nlm.nih.gov/pubmed/26900444
http://dx.doi.org/10.4081/cp.2015.768
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