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Budd–Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco

Budd–Chiari syndrome (BCS) is considered a rare but serious complication of Behçet’s disease (BD). This study was performed to define the prevalence, clinical and biological features, treatment, and clinical course of BSC associated with BD in a Moroccan population. We retrospectively analyzed the m...

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Autores principales: Allaoui, Abire, Echchilali, Khadija, Fares, Manal, Belabbes, Fatim-Zahra, Jabbouri, Rajaa, Naitlho, Abdelhamid, Moudatir, Mina, Alaoui, Fatim Zohra, Elkabli, Hassan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9646571/
https://www.ncbi.nlm.nih.gov/pubmed/36343052
http://dx.doi.org/10.1097/MD.0000000000031308
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author Allaoui, Abire
Echchilali, Khadija
Fares, Manal
Belabbes, Fatim-Zahra
Jabbouri, Rajaa
Naitlho, Abdelhamid
Moudatir, Mina
Alaoui, Fatim Zohra
Elkabli, Hassan
author_facet Allaoui, Abire
Echchilali, Khadija
Fares, Manal
Belabbes, Fatim-Zahra
Jabbouri, Rajaa
Naitlho, Abdelhamid
Moudatir, Mina
Alaoui, Fatim Zohra
Elkabli, Hassan
author_sort Allaoui, Abire
collection PubMed
description Budd–Chiari syndrome (BCS) is considered a rare but serious complication of Behçet’s disease (BD). This study was performed to define the prevalence, clinical and biological features, treatment, and clinical course of BSC associated with BD in a Moroccan population. We retrospectively analyzed the medical records of 1578 patients fulfilling the international diagnostic criteria for BD, including those with BSC. Eighteen male and 3 female patients, with a mean age of 36 ± 8.6 years. The inferior vena cava was involved in 81% (n = 17) of cases. All forms of BCS were noted: the chronic form in 52.4% (n = 11), the subacute form in 38% (n = 8), and the fulminant form (2 cases). Ascites was the main clinical sign and was present in 62% of patients (n = 13). Other venous thromboses (superior vena cava and lower limbs) were associated with BSC in 52.4% of patients (n = 11). Arterial involvement was noted in 28.6% (n = 6). Cardiac manifestations were present in 19% (n = 4) of the patients. All the patients received anticoagulants associated with corticosteroids. Immunosuppressants were used in 95% (n = 20). One patient received infliximab. Severe complications were noted in 38% (n = 8) of patients (digestive bleeding, confusion, infections and liver failure). Four patients have died during the study period. BCS in patients with BD is not uncommon and can be life threatening. It is frequently associated with other vascular manifestations that can be difficult to treat, particularly in the presence of pulmonary artery aneurysms. Prognosis improved with the use of immunosuppressants. Biologics can be promising in the early stages.
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spelling pubmed-96465712022-11-14 Budd–Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco Allaoui, Abire Echchilali, Khadija Fares, Manal Belabbes, Fatim-Zahra Jabbouri, Rajaa Naitlho, Abdelhamid Moudatir, Mina Alaoui, Fatim Zohra Elkabli, Hassan Medicine (Baltimore) 3600 Budd–Chiari syndrome (BCS) is considered a rare but serious complication of Behçet’s disease (BD). This study was performed to define the prevalence, clinical and biological features, treatment, and clinical course of BSC associated with BD in a Moroccan population. We retrospectively analyzed the medical records of 1578 patients fulfilling the international diagnostic criteria for BD, including those with BSC. Eighteen male and 3 female patients, with a mean age of 36 ± 8.6 years. The inferior vena cava was involved in 81% (n = 17) of cases. All forms of BCS were noted: the chronic form in 52.4% (n = 11), the subacute form in 38% (n = 8), and the fulminant form (2 cases). Ascites was the main clinical sign and was present in 62% of patients (n = 13). Other venous thromboses (superior vena cava and lower limbs) were associated with BSC in 52.4% of patients (n = 11). Arterial involvement was noted in 28.6% (n = 6). Cardiac manifestations were present in 19% (n = 4) of the patients. All the patients received anticoagulants associated with corticosteroids. Immunosuppressants were used in 95% (n = 20). One patient received infliximab. Severe complications were noted in 38% (n = 8) of patients (digestive bleeding, confusion, infections and liver failure). Four patients have died during the study period. BCS in patients with BD is not uncommon and can be life threatening. It is frequently associated with other vascular manifestations that can be difficult to treat, particularly in the presence of pulmonary artery aneurysms. Prognosis improved with the use of immunosuppressants. Biologics can be promising in the early stages. Lippincott Williams & Wilkins 2022-11-04 /pmc/articles/PMC9646571/ /pubmed/36343052 http://dx.doi.org/10.1097/MD.0000000000031308 Text en Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc. https://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) (https://creativecommons.org/licenses/by-nc/4.0/) , 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.
spellingShingle 3600
Allaoui, Abire
Echchilali, Khadija
Fares, Manal
Belabbes, Fatim-Zahra
Jabbouri, Rajaa
Naitlho, Abdelhamid
Moudatir, Mina
Alaoui, Fatim Zohra
Elkabli, Hassan
Budd–Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco
title Budd–Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco
title_full Budd–Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco
title_fullStr Budd–Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco
title_full_unstemmed Budd–Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco
title_short Budd–Chiari syndrome associated to Behcet disease: An observational retrospective multicenter study in Morocco
title_sort budd–chiari syndrome associated to behcet disease: an observational retrospective multicenter study in morocco
topic 3600
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9646571/
https://www.ncbi.nlm.nih.gov/pubmed/36343052
http://dx.doi.org/10.1097/MD.0000000000031308
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