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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Format: | Article |
Language: | English |
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Tabriz University of Medical Sciences
2015-08-01
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Series: | Journal of Analytical Research in Clinical Medicine |
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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. |
first_indexed | 2024-04-14T01:07:09Z |
format | Article |
id | doaj.art-6290b0ffdf6c498aadbba18c1f84efa9 |
institution | Directory Open Access Journal |
issn | 2345-4970 2345-4970 |
language | English |
last_indexed | 2024-04-14T01:07:09Z |
publishDate | 2015-08-01 |
publisher | Tabriz University of Medical Sciences |
record_format | Article |
series | Journal of Analytical Research in Clinical Medicine |
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 |