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Sao Paulo Medical Journal

Print version ISSN 1516-3180

Sao Paulo Med. J. vol.129 no.2 São Paulo Mar. 2011

https://doi.org/10.1590/S1516-31802011000200009 

REVIEW ARTICLE

 

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

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

Address for correspondence

 

 


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.

Key-words: Budd-Chiari syndrome. Behçet's syndrome. Liver. Male. Young adult


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.

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, severe 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 association 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 symptoms, 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, ascites, liver failure or cirrhosis but there is no obvious cause, even after 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 recurrent 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 genital ulceration, eye lesions, skin lesions and/or a pathergy test.6-8 Although 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 in Turkey and Behçet's disease constituted the largest group in the etiological 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 common 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 Turkish 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 patients with a mean age of 29 years who were already diagnosed with  Behçet'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 possibilities in cases of Budd-Chiari syndrome, since the third most common 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 disease 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 disease 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 patients 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 associated with other types of venous thrombosis and the prognosis may be favorable with medical interventions, including anticoagulation treatment 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 Behçet'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 possibilities in cases of Budd-Chiari syndrome. Severe vascular complications from Budd-Chiari syndrome in patients with Behçet's disease are much more common among young adult male patients.

 

REFERENCES

1. Ben Ghorbel I, Ennaifer R, Lamloum M, et al. Budd-Chiari syndrome associated with Behçet's disease. Gastroenterol Clin Biol. 2008;32(3):316-20.         [ Links ]

2. Houman H, Lamloum M, Ben Ghorbel I, Khiari-Ben Salah I, Miled M. Thromboses caves dans la maladie de Behçet. Analyse d'une série de 10 observations. [Vena cava thrombosis in Behçet's disease. Analysis of a series of 10 cases]. Ann Med Interne (Paris). 1999;150(8):587-90.         [ Links ]

3. Jayachandran NV, Rajasekhar L, Chandrasekhara PK, Kanchinadham S, Narsimulu G. Multiple peripheral arterial and aortic aneurysms in Behcet's syndrome: a case report. Clin Rheumatol. 2008;27(2):265-7.         [ Links ]

4. Ludwig J, Hashimoto E, McGill DB, van Heerden JA. Classification of hepatic venous outflow obstruction: ambiguous terminology of the Budd-Chiari syndrome. Mayo Clin Proc. 1990;65(1):51-5.         [ Links ]

5. Okuda K, Kage M, Shrestha SM. Proposal of a new nomenclature for Budd-Chiari syndrome: hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion. Hepatology. 1998;28(5):1191-8.         [ Links ]

6. Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. Lancet. 1990;335(8697):1078-80.         [ Links ]

7. Jorizzo JL, Abernethy JL, White WL, et al. Mucocutaneous criteria for the diagnosis of Behçet's disease: an analysis of clinicopathologic data from multiple international centers. J Am Acad Dermatol. 1995;32(6):968-76.         [ Links ]

8. Mignogna MD, Fedele S, Lo Russo L. International diagnostic criteria and delay of diagnosis in Behçet's disease. J Rheumatol. 2000;27(11):2725.         [ Links ]

9. B'Chir Hamzaoui S, Harmel A, Bouslama K, et al. La maladie de Behçet en Tunisie. Etude clinique de 519 cas [Behçet's disease in Tunisia. Clinical study of 519 cases]. Rev Med Interne. 2006;27(10):742-50.         [ Links ]

10. Danaci M, Gül S, Yazgan Y, Hülagü S, Uskent N. Budd-Chiari syndrome as a complication of Behçet's syndrome. A case report. Angiology. 1996;47(1):93-5.         [ Links ]

11. Korkmaz C, Kasifoglu T, Kebapçi M. Budd-Chiari syndrome in the course of Behcet's disease: clinical and laboratory analysis of four cases. Joint Bone Spine. 2007;74(3):245-8.         [ Links ]

12. Saatci I, Ozmen M, Balkanci F, Akhan O, Senaati S. Behçet's disease in the etiology of Budd-Chiari disease. Angiology. 1993;44(5):392-8.         [ Links ]

13. Bayraktar Y, Balkanci F, Bayraktar M, Calguneri M. Budd-Chiari syndrome: a common complication of Behçet's disease. Am J Gastroenterol. 1997;92(5):858-62.         [ Links ]

14. Uskudar O, Akdogan M, Sasmaz N, et al. Etiology and portal vein thrombosis in Budd-Chiari syndrome. World J Gastroenterol. 2008;14(18):2858-62.         [ Links ]

15. Orloff LA, Orloff MJ. Budd-Chiari syndrome caused by Behçet's disease: treatment by side-to-side portacaval shunt. J Am Coll Surg. 1999;188(4):396-407.         [ Links ]

16. Akbaş T, Imeryüz N, Bayalan F, et al. A case of Budd-Chiari syndrome with Behcet's disease and oral contraceptive usage. Rheumatol Int. 2007;28(1):83-6.         [ Links ]

17. Marsal S, Falgá C, Simeon CP, Vilardell M, Bosch JA. Behçet's disease and pregnancy relationship study. Br J Rheumatol. 1997;36(2):234-8.         [ Links ]

18. Bosch Gil JA, Falga Tirado C, Simeón Aznar CP, Marsal S. Síndrome de Budd-Chiari durante el puerperio en una paciente con enfermedad de Bechçet [Budd-Chiari syndrome during puerperium in a patient with Behcet's disease]. Med Clin (Barc). 1995;105(6):236-7.         [ Links ]

19. Linnemann B, Greinacher A, Lindhoff-Last E. Alteration of pharmacokinetics of lepirudin caused by anti-lepirudin antibodies occurring after long-term subcutaneous treatment in a patient with recurrent VTE due to Behcets disease. Vasa. 2010;39(1):103-7.         [ Links ]

20. Seyahi E, Hamuryudan V, Hatemi G, et al. Infliximab in the treatment of hepatic vein thrombosis (Budd-Chiari syndrome) in three patients with Behcet's syndrome. Rheumatology (Oxford). 2007;46(7):1213-4.         [ Links ]

21. Khuroo MS, Al-Suhabani H, Al-Sebayel M, et al. Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepatic portosystemic shunt. J Gastroenterol Hepatol. 2005;20(10):1494-502.         [ Links ]

22. Han SW, Kim GW, Lee J, Kim YJ, Kang YM. Successful treatment with stent angioplasty for Budd-Chiari syndrome in Behçet's disease. Rheumatol Int. 2005;25(3):234-7.         [ Links ]

23. Kuniyoshi Y, Koja K, Miyagi K, et al. Surgical treatment of Budd-Chiari syndrome induced by Behcet's disease. Ann Thorac Cardiovasc Surg. 2002;8(6):374-80.         [ Links ]

24. Jiménez-Sáenza M, Mendoza J, Marcob F, Herrerías-Gutiérrez JM. Síndrome de Budd-Chiari tratado mediante angioplastia transluminal en un paciente con enfermedad de Behçet: seguimiento a largo plazo [Budd-Chiari syndrome treated with transluminal angioplasty in a patient with Behçet's disease: long-term follow-up. Med Clin (Barc). 2000;114(17):678-9.         [ Links ]

25. Bayraktar Y, Ozaslan E, Van Thiel DH. Gastrointestinal manifestations of Behcet's disease. J Clin Gastroenterol. 2000;30(2):144-54.         [ Links ]

26. Al-Damegh S. Budd-Chiari syndrome: a short radiological review. J Gastroenterol Hepatol. 1999;14(11):1057-61.         [ Links ]

27. Oge N, Alli N. Budd-Chiari syndrome as a presenting syndrome for Behçet's disease. Int J Dermatol. 1997;36(7):556-7.         [ Links ]

28. Bayraktar Y, Balkanci F, Kansu E, et al. Cavernous transformation of the portal vein: a common manifestation of Behçet's disease. Am J Gastroenterol. 1995;90(9):1476-9.         [ Links ]

29. Bayraktar Y, Balkanci F, Kansu E, et al. Budd-Chiari syndrome: analysis of 30 cases. Angiology. 1993;44(7):541-51.         [ Links ]

30. Corbella X, Casanovas T, Benasco C, Casais L. Budd-Chiari syndrome complicating Behçet's disease. Am J Gastroenterol. 1991;86(4):526-7.         [ Links ]

31. al-Dalaan A, al-Balaa S, Ali MA, et al. Budd-Chiari syndrome in association with Behçet's disease. J Rheumatol. 1991;18(4):622-6.         [ Links ]

32. Benamour S, Zeroual B, Bennis R, Amraoui A, Bettal S. Maladie de Behçet. 316 cas [Behçet's disease. 316 cases]. Presse Med. 1990;19(32):1485-9.         [ Links ]

33. Orloff MJ, Girard B. Long term results of treatment of Budd-Chiari syndrome by side to side portacaval shunt. Surg Gynecol Obstet. 1989;168(1):33-41.         [ Links ]

34. Le Treut YP, Comiti Y, Bremondy A, et al. Syndrome de Budd-Chiari et maladie de Behçet. Une observation traitée par prothèse mésentérico-atriale [Budd-Chiari syndrome and Behçet's disease. A case treated by mesenterico-atrial prosthesis]. Gastroenterol Clin Biol. 1988;12(3):265-9.         [ Links ]

35. Carvalho A, Duarte R, Neves M, et al. Sindroma de Budd-Chiari na doença de Behçet [Budd-Chiari syndrome in Behçet's disease]. Acta Med Port. 1988;1(3):217-21.         [ Links ]

36. Urano Y, Ohmori H, Sugimura H, Fukushima T. Maladie de Behçet avec syndrome de Budd-Chiari. A propos d'un cas. Revue des cas autopsiés au Japon et de la littérature [Behçet's disease with Budd-Chiari syndrome. Apropos of a case. Review of cases autopsied in Japan and of the literature]. Ann Pathol. 1986;6(3):192-6.         [ Links ]

37. Nakayama S, Sakata J, Kusumoto S, et al. Ultrasonic appearance of the liver in hepatic venous outflow obstruction (Budd-Chiari syndrome): a case of pseudohepatic infarct associated with Behçet's disease. J Clin Ultrasound. 1986;14(4):300-3.         [ Links ]

38. Montagnac R, Schillinger F, Bressieux JM. Le syndrome de Budd-Chiari, complication inhabituelle de la maladie de Behçet [Budd-Chiari syndrome, an uncommon complication of Behçet's disease]. Presse Med. 1986;15(30):1427.         [ Links ]

39. Schattner A. Budd-Chiari syndrome and renal failure in Behcet's disease. Am J Med. 1984;77(2):A86, A91.         [ Links ]

40. Wilkey D, Yocum DE, Oberley TD, Sundstrom WR, Karl L. Budd-Chiari syndrome and renal failure in Behcet disease. Report of a case and review of the literature. Am J Med. 1983;75(3):541-50.         [ Links ]

41. Varon D, Schattner A, Geltner D. [Effect of colchicine and streptokinase therapy on Budd-Chiary syndrome in Behçet's disease]. Harefuah. 1983;104(3):91-2.         [ Links ]

42. Thevenet A, Joyeux A. Syndrome de Budd-Chiari et obstruction membraneuse de la veine cave inférieure. A propos de 3 observations [Budd-Chiari syndrome and membranous obstruction of the inferior vena cava. Study of 3 cases]. Chirurgie. 1982;108(3):267-72.         [ Links ]

43. McDonald G, Gad-Al-Rab J. Behçet's disease with endocarditis and the Budd-Chiari syndrome. J Clin Pathol. 1980;33(7):660-9.         [ Links ]

44. Valleteau de Moulliac M, Morin D, Graveleau J, et al. A propos d'un cas d'association de syndrome de Budd-Chiari et de syndrome de Behçet [On a case of association of the Budd- Chiari syndrome and Behçet's syndrome]. Sem Hop. 1974;50(43):2679-83.         [ Links ]

45. Maruyama R, Kumaki T, Miyashita M, Yoshida K, Takano Y. [Case of Behcet's syndrome accompanied by Budd-Chiari syndrome]. Naika. 1967;19(5):959-64.         [ Links ]

46. Saadoun D, Wechsler B, Desseaux K, et al. Mortality in Behçet's disease. Arthritis Rheum. 2010;62(9):2806-12.         [ Links ]

47. Goktekin O, Korkmaz C, Timuralp B, et al. Widespread thrombosis associated with recurrent intracardiac masses in a patient with Behçet's disease. Int J Cardiovasc Imaging. 2002;18(6):431-4.         [ Links ]

48. Ferraris R, Colzani G, Galatola G, Fiorentini MT. Ascites with suprahepatic portal hypertension in a case of Behçet's disease. Panminerva Med. 1985;27(1):43-4.         [ Links ]

 

 

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

Date of first submission: June 14, 2010
Last received: September 28, 2010
Accepted: November 22, 2010
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.

 

 

Department of Surgery and Anatomy, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo (FMRP-USP), Ribeirão Preto, São Paulo, Brazil

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