Hepatic venous outflow block in a young patient with Systemic Lupus Erythematosus

Introduction: Hepatic venous outflow block or Budd-Chiari syndrome is a severe liver disease with a 3 years survival rate of 50%. Several conditions have been implicated as a cause of Budd-Chiari syndrome, including myeloproliferative disorders, paroxysmal nocturnal hemoglobinuria, the presence of l...

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Main Author: Ali Ghavidel
Format: Article
Language:English
Published: Tabriz University of Medical Sciences 2015-08-01
Series:Journal of Analytical Research in Clinical Medicine
Subjects:
Online Access:http://journals.tbzmed.ac.ir/JARCM/Manuscript/JARCM-3-190.pdf
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author Ali Ghavidel
author_facet Ali Ghavidel
author_sort Ali Ghavidel
collection DOAJ
description Introduction: Hepatic venous outflow block or Budd-Chiari syndrome is a severe liver disease with a 3 years survival rate of 50%. Several conditions have been implicated as a cause of Budd-Chiari syndrome, including myeloproliferative disorders, paroxysmal nocturnal hemoglobinuria, the presence of lupus anti-coagulant, oral contraceptives, pregnancy, and others. In a small number of cases, Budd-Chiari syndrome is associated with the presence of lupus anticoagulant. Anticardiolipin antibodies (ACA) are similar to lupus anti-coagulant antiphospholipid antibodies (APLAs), which have been described in patients with recurrent arterial and venous thrombosis, thrombocytopenia, fetal loss, or miscarriage. Case Report: A 23-year-old woman is reported with Budd-Chiari syndrome in whom lupus anticoagulant and anticardiolipin antibodies were shown; 9 months after diagnosis of systemic lupus erythematosus (SLE) treatment with steroids admitted with gastrointestinal problems, abdominal pain and ascites and treated oral anticoagulants induced a considerable improvement. This treatment was continued after 1 year, but interruption was followed by redevelopment of ascites. Further treatment with anticoagulants was continued for 5 years with noticeable improvement. Conclusion: Patients with Budd-Chiari syndrome should be tested for lupus anticoagulants and anticardiolipin antibodies, Budd-Chiari syndrome resulting from this cause may have a good response to treatment with oral anticoagulants; this treatment should be maintained permanently, and pregnancy in such patients may initiate serious difficulties. The condition of the patient at follow-up was good.
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spelling doaj.art-6290b0ffdf6c498aadbba18c1f84efa92022-12-22T02:21:12ZengTabriz University of Medical SciencesJournal of Analytical Research in Clinical Medicine2345-49702345-49702015-08-013319019310.15171/jarcm.2015.030JARCM_1156_20140615114306Hepatic venous outflow block in a young patient with Systemic Lupus ErythematosusAli Ghavidel0Assistant Professor, Liver and Gastrointestinal Diseases Research Center, Imam Reza Hospital, Tabriz University of Medical Sciences, Tabriz, IranIntroduction: Hepatic venous outflow block or Budd-Chiari syndrome is a severe liver disease with a 3 years survival rate of 50%. Several conditions have been implicated as a cause of Budd-Chiari syndrome, including myeloproliferative disorders, paroxysmal nocturnal hemoglobinuria, the presence of lupus anti-coagulant, oral contraceptives, pregnancy, and others. In a small number of cases, Budd-Chiari syndrome is associated with the presence of lupus anticoagulant. Anticardiolipin antibodies (ACA) are similar to lupus anti-coagulant antiphospholipid antibodies (APLAs), which have been described in patients with recurrent arterial and venous thrombosis, thrombocytopenia, fetal loss, or miscarriage. Case Report: A 23-year-old woman is reported with Budd-Chiari syndrome in whom lupus anticoagulant and anticardiolipin antibodies were shown; 9 months after diagnosis of systemic lupus erythematosus (SLE) treatment with steroids admitted with gastrointestinal problems, abdominal pain and ascites and treated oral anticoagulants induced a considerable improvement. This treatment was continued after 1 year, but interruption was followed by redevelopment of ascites. Further treatment with anticoagulants was continued for 5 years with noticeable improvement. Conclusion: Patients with Budd-Chiari syndrome should be tested for lupus anticoagulants and anticardiolipin antibodies, Budd-Chiari syndrome resulting from this cause may have a good response to treatment with oral anticoagulants; this treatment should be maintained permanently, and pregnancy in such patients may initiate serious difficulties. The condition of the patient at follow-up was good.http://journals.tbzmed.ac.ir/JARCM/Manuscript/JARCM-3-190.pdfSystemic Lupus ErythematosusBudd-Chiari SyndromeNephrotic Syndrome
spellingShingle Ali Ghavidel
Hepatic venous outflow block in a young patient with Systemic Lupus Erythematosus
Journal of Analytical Research in Clinical Medicine
Systemic Lupus Erythematosus
Budd-Chiari Syndrome
Nephrotic Syndrome
title Hepatic venous outflow block in a young patient with Systemic Lupus Erythematosus
title_full Hepatic venous outflow block in a young patient with Systemic Lupus Erythematosus
title_fullStr Hepatic venous outflow block in a young patient with Systemic Lupus Erythematosus
title_full_unstemmed Hepatic venous outflow block in a young patient with Systemic Lupus Erythematosus
title_short Hepatic venous outflow block in a young patient with Systemic Lupus Erythematosus
title_sort hepatic venous outflow block in a young patient with systemic lupus erythematosus
topic Systemic Lupus Erythematosus
Budd-Chiari Syndrome
Nephrotic Syndrome
url http://journals.tbzmed.ac.ir/JARCM/Manuscript/JARCM-3-190.pdf
work_keys_str_mv AT alighavidel hepaticvenousoutflowblockinayoungpatientwithsystemiclupuserythematosus