The risk that patients with Behçet's disease may develop various thrombotic complications has been previously described. Although vascular complications from Budd-Chiari syndrome associated with Behçet's disease have been described, the pathogenic mechanisms are still unknown. Severe vascular complications present in Budd-Chiari syndrome associated with Behçet's disease are very common among young male adults. The objective of this study was to review the literature and present the association of Budd-Chiari syndrome with Behçet's disease.O risco de pacientes com doença de Behçet desenvolverem várias complicações trombóticas já foi descrito. Apesar de complicações vasculares na síndrome de Budd-Chiari associada à doença de Behçet terem sido descritas, os mecanismos patogenéticos ainda são desconhecidos. Complicações vasculares graves presentes na síndrome de Budd-Chiari associada à doença de Behçet são muito mais comuns no adulto jovem do sexo masculino. O objetivo deste trabalho é o de revisar a literatura e apresentar a associação da síndrome de Budd-Chiari com a doença de Behçet

CARVALHO, Daniela

MATSUDA, Nilce Mitiko

OIKAWA, Fernando

YAMADA, Alice Tatsuko

RCAAP - Repositório Científico de Acesso Aberto de Portugal

Síndrome de Budd-Chiari em associação com a doença de Behçet: revisão de literatura

The risk that patients with Behçet's disease may develop various thrombotic complications has been previously described. Although vascular complications from Budd-Chiari syndrome associated with Behçet's disease have been described, the pathogenic mechanisms are still unknown. Severe vascular complications present in Budd-Chiari syndrome associated with Behçet's disease are very common among young male adults. The objective of this study was to review the literature and present the association of Budd-Chiari syndrome with Behçet's disease.</jats:p

Daniela Carvalho

Fernando Oikawa

Nilce Mitiko Matsuda

Alice Tatsuko Yamada

Crossref

Sao Paulo Medical Journal

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

Universidade de São Paulo

Biblioteca Digital da Produção Intelectual da Universidade de São Paulo (BDPI/USP)

Sao Paulo Med J. 2011;129(2):107-9 107
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Budd-Chiari syndrome in association with Behçet’s disease: 
review of the literature
Síndrome de Budd-Chiari em associação com a doença de Behçet: revisão de literatura
Daniela CarvalhoI, Fernando OikawaI, Nilce Mitiko MatsudaII, Alice Tatsuko YamadaIII
Department of Surgery and Anatomy, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo (FMRP-USP), Ribeirão Preto, São Paulo, Brazil
IMD. Resident Physician, Hospital Municipal de Campo Limpo “Dr. Fernando Mauro Pires da Rocha”, São Paulo, SP, Brazil. 
IIMD. Resident Physician, Hospital Municipal de Campo Limpo “Dr. Fernando Mauro Pires da Rocha”, São Paulo, SP, Brazil.
IIIMD, PhD. Research associate, Department of Surgery and Anatomy, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo (FMRP-USP), Ribeirão Preto, SP, Brazil. 
IVMD, PhD. Cardiologist, Instituto do Coração (InCor), Hospital das Clínicas (HC), School of Medicine, Universidade de São Paulo (USP), São Paulo, and Hospital Municipal de Campo Limpo “Dr. Fernando Mauro Pires da Rocha”, 
São Paulo, SP, Brazil.
ABSTRACT
The risk that patients with Behçet’s disease may develop various thrombotic complications has been previously described. Although vascular 
complications from Budd-Chiari syndrome associated with Behçet’s disease have been described, the pathogenic mechanisms are still unknown. 
Severe vascular complications present in Budd-Chiari syndrome associated with Behçet’s disease are very common among young male adults. The 
objective of this study was to review the literature and present the association of Budd-Chiari syndrome with Behçet’s disease.
RESUMO 
O risco de pacientes com doença de Behçet desenvolverem várias complicações trombóticas já foi descrito. Apesar de complicações vasculares na 
síndrome de Budd-Chiari associada à doença de Behçet terem sido descritas, os mecanismos patogenéticos ainda são desconhecidos. Complicações 
vasculares graves presentes na síndrome de Budd-Chiari associada à doença de Behçet são muito mais comuns no adulto jovem do sexo masculino. 
O objetivo deste trabalho é o de revisar a literatura e apresentar a associação da síndrome de Budd-Chiari com a doença de Behçet.
KEY WORDS: 
Budd-Chiari syndrome. 
Behçet’s syndrome. 
Liver. 
Male. 
Young adult.
PALAVRAS-CHAVE: 
Síndrome de Budd-Chiari. 
Síndrome de Behçet. 
Fígado. 
Masculino. 
Adulto jovem.
INTRODUCTION
Although the pathogenic mechanisms of vascular complications 
from Budd-Chiari syndrome in Behçet’s disease are unknown, se-
vere vascular complications from Budd-Chiari syndrome in patients 
with Behçet’s disease are much more common in young adult male 
patients.1-3 Budd-Chiari syndrome associated with Behçet’s disease has 
worldwide distribution, but it is more common in the Middle and Far 
East and rare in the Americas and Europe.1-3 Therefore, the purpose of 
this study was to review and summarize Budd-Chiari syndrome in as-
sociation with Behçet’s disease. 
Budd-Chiari syndrome
Budd-Chiari syndrome is caused by blood clots that completely or 
partially block the large veins that carry blood from the liver (hepatic 
veins) into the inferior vena cava.4,5 Some people have no overt symp-
toms, but others experience fatigue, abdominal pain, nausea, jaundice, 
enlarged liver and spleen, edema in the legs, ascites, and sometimes 
rupture and bleeding in the varicose veins of the esophagus. Usually, 
the symptoms develop gradually over weeks or months and Doppler 
ultrasonography can detect narrowed or blocked veins.4,5 Budd-Chiari 
syndrome is suspected when there are findings of an enlarged liver, as-
cites, liver failure or cirrhosis but there is no obvious cause, even af-
ter testing.4,5
Although the pathophysiology is unknown, the diagnosis of 
Budd-Chiari syndrome in patients with Behçet’s disease is responsible 
for 3% of the cases of Budd-Chiari syndrome and the risk that patients 
with Behçet’s disease will develop thrombotic complications is several 
times higher.1-3 
Behçet’s disease
Behçet’s disease is a multisystem disorder presenting with recur-
rent oral and/or genital ulcerations and chronic relapsing uveitis that 
may cause blindness and neurological impairments. The diagnosis is 
clinical since there is no specific evidence, pathognomonic symptoms 
or specific laboratory findings.6-8
According to the international criteria, the diagnosis of Behçet’s 
disease requires the presence of recurrent oral ulceration in the absence 
of other clinical explanations, and two of the following; recurrent gen-
ital ulceration, eye lesions, skin lesions and/or a pathergy test.6-8 Al-
though Behçet’s disease has worldwide distribution, it is rare in the 
Americas and Europe and is more prevalent in Turkey, the Middle East 
and the Far East. It mainly affects young adults, and men have more 
severe vascular complications with this disease.1,9-11
Budd-Chiari syndrome in association with Behçet’s disease
Budd-Chiari syndrome or hepatic venous outflow obstruction was 
diagnosed in 30 patients over a 10-year period in a university hospital 
Sao Paulo Med J. 2011;129(2):107-9
Carvalho D, Oikawa F, Matsuda NM, Yamada AT
108
in Turkey and Behçet’s disease constituted the largest group in the etio-
logical distribution.12 Because of occlusion of the major hepatic veins, 
the adjacent inferior vena cava, or both, Budd-Chiari syndrome is a rare 
and serious complication of Behçet’s disease. In the abovementioned 
cases series in Turkey, inferior vena cava involvement was more com-
mon in these patients.12 
Budd-Chiari syndrome as a complication of Behçet’s was seen in 
four young male patients in another clinical follow-up at the same Turk-
ish university hospital.13 Out of 220 Tunisian patients who fulfilled the 
international criteria for the diagnosis of Behçet’s disease, those with 
Budd-Chiari syndrome were selected. It was found that seven male pa-
tients with a mean age of 29 years who were already diagnosed with  Be-
hçet’s disease had Budd-Chiari syndrome.1 Furthermore, the case of a 
young male patient with Behçet’s syndrome and presenting with Budd-
Chiari syndrome who died during emergency thrombectomy surgery 
was reported from another teaching hospital in Turkey.10 
The evidence from countries in which Behçet’s disease is prevalent 
suggests that this disease should be included among the diagnostic pos-
sibilities in cases of Budd-Chiari syndrome, since the third most com-
mon cause among a total of 75 patients diagnosed with Budd-Chiari 
syndrome was Behçet’s disease.14 
Thus, although Budd-Chiari syndrome associated with Behçet’s dis-
ease has worldwide distribution, it is more common in the Middle and 
Far East and affects mainly young male adults.1,10-13,15 The association of 
Budd-Chiari syndrome and Behçet’s disease in women is related to oral 
contraceptive usage and pregnancy.16-18
We performed a search for Budd-Chiari syndrome and Behçet’s dis-
ease in relevant databases: Cochrane Database of Systematic Reviews, 
Embase Biomedical Answers, Literatura Latino-Americana e do Caribe 
em Ciências da Saúde (Lilacs) and the United States National Library 
of Medicine, National Institutes of Health (PubMed).1,2,10-48 The results 
are presented in Table 1.
Although the mortality rate due to Behçet’s disease is low, most pa-
tients with Behçet’s disease who develop Budd-Chiari syndrome may 
die as a consequence of hepatic venous outflow obstruction.9,10,13,15 The 
hepatic venous outflow obstruction in Behçet’s disease is often associ-
ated with other types of venous thrombosis and the prognosis may be 
favorable with medical interventions, including anticoagulation treat-
ment for vasculitis and the use of diuretics when required.11
CONCLUSION
Although the pathogenic mechanisms for Budd-Chiari syndrome 
are unknown, this vascular complication may be associated with Be-
hçet’s disease. In countries in which the prevalence of Behçet’s disease is 
high, such as Turkey and others in the Middle and Far East, the evidence 
suggests that this disease should be included among the diagnostic pos-
sibilities in cases of Budd-Chiari syndrome. Severe vascular complica-
tions from Budd-Chiari syndrome in patients with Behçet’s disease are 
much more common among young adult male patients.
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Table 1. Databases for Budd-Chiari syndrome and Behçet’s disease
Database
Search 
Strategy
Results
Articles found Related articles
Cochrane Budd-Chiari 
syndrome and 
Behcet disease
0 0 -
Embase Budd-Chiari 
syndrome and 
Behcet disease
71 38* 22 case reports
10 case series
2 prognostic studies
2 reviews
2 case-control studies
Lilacs Budd-Chiari 
syndrome and 
Behcet disease
0 0 -
PubMed Budd-Chiari 
syndrome and 
Behcet disease
45 37† 21 case reports
9 case series
3 prognostic studies
2 reviews
2 case-control studies
*References = 1,2,10-45; †References = 1,2,10-20,22-25,27,28,30-41,43-48
Cochrane = Cochrane Database of Systematic Reviews; Embase = Embase Biomedical Answers; Lilacs = 
Literatura Latino-Americana e do Caribe em Ciências da Saúde; PubMed = United States National Library of 
Medicine, National Institutes of Health.
Budd-Chiari syndrome in association with Behçet’s disease: review of the literature
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Sources of funding: Fundação de Amparo à Pesquisas do Estado de São Paulo (Fapesp 
2006/50084-2) and Conselho Nacional de Desenvolvimento Científico e Tecnológico 
(CNPq 474531/2008-2)
Conflict of interest: None
Date of first submission: June 14, 2010
Last received: September 28, 2010
Accepted: November 22, 2010
Address for correspondence: 
Nilce Mitiko Matsuda  
Departamento de Cirurgia e Anatomia  
Faculdade de Medicina de Ribeirão Preto  
Universidade de São Paulo  
Av. Bandeirantes, 3.900 — anexo B — bloco C 
Monte Alegre — Ribeirão Preto (SP) — Brasil 
CEP 14049-900 
Tel. (+55 16) 3602-3298 
E-mail: nmmatsuda@uol.com.br


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Síndrome de Budd-Chiari em associação com a doença de Behçet: revisão de literatura

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