Budd-Chiari syndrome in association with Behçet's disease: review of the literature

Carvalho, Daniela; Oikawa, Fernando Teiichi Costa; Matsuda, Nilce Mitiko; Yamada, André Fukunishi

doi:10.1590/s1516-31802011000200009

Cited by 8 publications

(4 citation statements)

References 29 publications

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“…Early diagnosis is therefore important for patients with BCS to receive prompt and specific treatment (12). Furthermore, misdiagnosis may occur if the patient is not diagnosed with BD prior to BCS (8,9). Case 2 initially presented with symptoms of liver cirrhosis and was given non-specific treatment, but was diagnosed with BD 6 months later at The First Affiliated Hospital of Sun Yat-sen University.…”

Section: Discussionmentioning

confidence: 99%

“…It has been reported that ~5% of patients with BCS in western countries, 9% in Turkey and 13% in Egypt may be attributed to BD (6–9). However, misdiagnosis is a common occurrence in cases of BD associated BCS (BD-BCS) (8,9). Certain patients exhibit no overt symptoms and others experience abdominal pain, jaundice, nausea, enlarged liver/spleen and ascites (8,9).…”

Section: Introductionmentioning

confidence: 99%

“…However, misdiagnosis is a common occurrence in cases of BD associated BCS (BD-BCS) (8,9). Certain patients exhibit no overt symptoms and others experience abdominal pain, jaundice, nausea, enlarged liver/spleen and ascites (8,9). Inappropriate or delayed treatment may therefore occur if other symptoms of BD are unnoticed.…”

Section: Introductionmentioning

confidence: 99%

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Budd‑Chiari syndrome in Behcet's disease: A report of two cases

et al. 2018

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Budd-Chiari syndrome (BCS) is a rare but severe venous form of Behcet's disease (BD) that is caused by the obstruction of the venous outflow tract that transports blood from hepatic veins into the inferior vena cava. In countries where BD is prevalent, including the Middle East and Far East, BCS awareness is important. In the present study, two cases of BCS are presented in two male Chinese patients with BD. The clinical characteristics, treatment and outcomes were recorded and compared with previous studies, and the features of BD-BCS were summarized. The clinical characteristics of the two patients documented were similar. Each patient presented with insidious onset, abdominal symptoms and recurrent aphthous ulcers. Accurate diagnosis was delayed as other symptoms of BD were overlooked. Each patient responded well to TNF-α inhibitor treatment in combination with cyclophosphamide (CYC). One patient with good compliance was removed from CYC and corticosteroid therapy. Unfortunately, the other patient with poor compliance faced a poor outcome. It was concluded that multiple vessel lesions in ≥2 sites are common in vasculo-BD and that misdiagnosis may occur if other symptoms of BD are not noticed. BD-BCS is associated with a high mortality rate, but appropriate treatment may result in a favorable outcome.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Budd‑Chiari syndrome in Behcet's disease: A report of two cases

et al. 2018

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“…(Pasmant et al, 2010). In addition, thrombophlebitis, erythrocytosis, multiple myeloma, and positive antiphospholipid antibody due to long-term oral administration of contraceptives are other causes of these medical conditions (Carvalho et al, 2011;Yadav et al, 2012). Although the aforementioned studies have identified BCS manifested by hepatic venous obstruction due to thrombosis, they cannot provide an explanation for that manifested by MOVC.…”

Section: Discussionmentioning

confidence: 99%