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Chronic Budd–Chiari syndrome in Behçet's disease successfully managed with transjugular intrahepatic portosystemic shunt: a case report and literature review

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Abstract

Budd–Chiari syndrome (BCS) is characterized by an obstruction of hepatic venous outflow from small hepatic veins to inferior vena cava, caused by acute thrombosis or its fibrous sequellae. An underlying myeloproliferative neoplasm is present in 50% of cases. Clinical manifestations are widely variable, from asymptomatic to fulminant episodes. Long-term complications range from cirrhosis to hepatocellular carcinoma. Behçet’s disease (BD) is a rare recurrent inflammatory multisystemic disorder characterized by recurrent skin-mucosa lesions and systemic involvement. Vascular involvement is observed in up to 40% of the patients with BD, and it is one of the major causes of mortality and morbidity. BCS is a rare complication of BD with a frequency of < 5% among patients with vascular involvement. Immunosuppressive treatment is the cornerstone for the management of vascular involvement in BD, while anticoagulant therapy has been an issue of debate. Transjugular intrahepatic portosystemic shunt (TIPS) in severe cases of BCS—of all causes— improves survival. However, there is scarce evidence about the role of TIPS in the setting of BCS in BD. We present a case of a vascular Behçet’s disease associated with chronic Budd–Chiari syndrome with progression of thrombosis despite adequate anticoagulant and immunosuppressive treatment, successfully managed with TIPS.

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References

  1. Seyahi E. Behçet’s disease: How to diagnose and treat vascular involvement. Best Pract Res Clin Rheumatol. 2016;30:279–95.

    Article  Google Scholar 

  2. Becatti M, Emmi G, Silvestri E, et al. Neutrophil activation promotes fibrinogen oxidation and thrombus formation in Behçet’s disease. Circulation. 2015;133:302–11.

    Article  Google Scholar 

  3. Emmi G, Becatti M, Bettiol A, et al. Behçet’s syndrome as a model of thrombo-inflammation: the role of neutrophils. Front Inmunol. 2019;10:1–8.

    Google Scholar 

  4. Becatti M, Emmi G, Bettiol A, et al. Behçet's syndrome as a tool to dissect mechanism of thrombo-inflamaation: clinical and pathogenetic aspects. Clin Exp Immunol. 2019;195:322–33.

    Article  CAS  Google Scholar 

  5. Desbois AC, Wechsler B, Resche-Rigon M, et al. Immunosuppressants reduce venous thrombosis relapse in Behçet’s disease. Arthritis Rheum. 2012;64:2753–60.

    Article  CAS  Google Scholar 

  6. Alibaz-Oner F, Karadeniz A, Yilmaz S, et al. Behçet disease with vascular involvement. Medicine (Baltimore). 2015;94:e494.

    Article  CAS  Google Scholar 

  7. Garcia-Pagán JC, Buscarini E, Janssen HLA, et al. EASL clinical practice guidelines: vascular diseases of the liver. J Hepatol. 2016;64:179–202.

    Article  Google Scholar 

  8. Valla DC. Primary Budd–Chiari syndrome. J Hepatol. 2009;50:195–203.

    Article  Google Scholar 

  9. Ahn JK, Lee YS, Jeon CH, et al. Treatment of venous thrombosis associated with Behcet’s disease: immunosuppressive therapy alone versus immunosuppressive therapy plus anticoagulation. Clin Rheumatol. 2008;27:201–5.

    Article  Google Scholar 

  10. Hatemi G, Christensen R, Bang D, et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis. 2018;77:808–18.

    PubMed  Google Scholar 

  11. García-Pagán JC, Heydtmann M, Raffa S, et al. TIPS for Budd–Chiari syndrome: long-term results and prognostics factors in 124 patients. Gastroenterology. 2008;135:808–15.

    Article  Google Scholar 

  12. Galeano-Valle F, Demelo-Rodriguez P, Álvarez-Sala-Walther L, et al. Intracardiac thrombosis in Behçet’s disease successfully treated with immunosuppressive agents: a case of vascular pathergy phenomenon. Intractable Rare Dis Res. 2018;7:54–7.

    Article  Google Scholar 

  13. Davatchi F, Abdollahi BS, Chams-davatchi C, et al. The saga of diagnostic/classification criteria in Behcet’s disease. JEADV. 2015;18:594–605.

    Google Scholar 

  14. Goel RM, Johnston EL, Patel KV, et al. Budd–Chiari syndrome: investigation, treatment and outcomes. Postgrad Med J. 2015;91:692–7.

    Article  Google Scholar 

  15. Emmi G, Bettiol A, Silvestri E, et al. Vascular Behçet’s syndrome: an update. Intern Emerg Med. 2019;14:645–52.

    Article  Google Scholar 

  16. Seyahi E, Caglar E, Ugurlu S, et al. An outcome survey of 43 patients with Budd–Chiari syndrome due to Behçet’s syndrome followed up at a single, dedicated center. Semin Arthritis Rheum. 2015;44:602–9.

    Article  Google Scholar 

  17. Wang H, Guo X, Tian Z, et al. Intracardiac thrombus in patients with Behcet’s disease: clinical correlates, imaging features, and outcome: a retrospective, single-center experience. Clin Rheumatol. 2016;35:2501–7.

    Article  Google Scholar 

  18. Demirelli S, Degirmenci H, Inci S, et al. Cardiac manifestations in Behcet’s disease. Intractable Rare Dis Res. 2015;4:70–5.

    Article  Google Scholar 

  19. Park MC, Hong BK, Kwon HM, et al. Surgical outcomes and risk factors for postoperative complications in patients with Behcet’s disease. Clin Rheumatol. 2007;26:1475–80.

    Article  Google Scholar 

  20. Saleh Z, Arayssi T. Update on the therapy of Behçet disease. Ther Adv Chronic Dis. 2014;5:112–34.

    Article  CAS  Google Scholar 

  21. Toledo-Samaniego N, Galeano-Valle F, Pinilla-Llorente B, et al. Clinical features and management of venous thromboembolism in patients with Behçet's syndrome: a single center case-control study. Intern Emerg Med. 2019. https://doi.org/10.1007/s11739-019-02237-7.

    Article  PubMed  Google Scholar 

  22. Oblitas CM, Galeano-Valle F, Toledo-Samaniego N, et al. Budd–Chiari syndrome in Behçet’s disease successfully managed with immunosuppressive and anticoagulant therapy: a case report and literature review. Intractable Rare Dis Res. 2019;8:60–6.

    Article  Google Scholar 

  23. Desbois AC, Rautou PE, Biard L, et al. Behcet’s disease in Budd–Chiari syndrome. Orphanet J Rare Dis. 2015;9:1–9.

    Google Scholar 

  24. Tekbas G, Oguzkurt L, Gur S, et al. Endovascular treatment of veno-occlusive Behcet’s disease. Cardiovasc Intervent Radiol. 2012;35:826–31.

    Article  Google Scholar 

  25. 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;28:83–6.

    Article  Google Scholar 

  26. Han SW, Kim GW, Lee J, et al. Successful treatment with stent angioplasty for Budd–Chiari syndrome in Behçet’s disease. Rheumatol Int. 2005;25:234–7.

    Article  Google Scholar 

  27. Jiménez-Sáenza M, Mendoza J, Marcob F, et al. Síndrome de Budd–Chiari tratado mediante angioplastia transluminal en un paciente con enfermedad de Behçet: seguimiento a largo plazo. Med Clin (Barc). 2000;114:678–9.

    Article  Google Scholar 

  28. Kuniyoshi Y, Koja K, Miyagi K. Surgical treatment of Budd–Chiari syndrome induced by Behcet's disease. Ann Thorac Cardiovasc Surg. 2002;8:374–80.

    PubMed  Google Scholar 

  29. Orloff LA, Orloff MJ. Budd–Chiari syndrome caused by Behcet’s disease: treatment by side-to- side portacaval shunt. J Am Coll Surg. 1999;188:396–407.

    Article  CAS  Google Scholar 

  30. Danaci M, Gül S, Yazgan Y, et al. Budd–Chiari syndrome as a complication of Behçet's syndrome. A case report Angiology. 1996;47:93–5.

    CAS  PubMed  Google Scholar 

  31. Al-Dalaan A, Al-Balaa S, Ali MA, et al. Budd–Chiari syndrome in association with Behçet’s disease. J Rheumatol. 1991;18:622–6.

    CAS  PubMed  Google Scholar 

  32. Bismuth E, Hadengue A, Hammel P, et al. Hepatic vein thrombosis in Behçet’s disease. Hepatology. 1990;11:969–74.

    Article  CAS  Google Scholar 

  33. Orloff MJ, Girard B. Long term results of treatment of Budd–Chiari syndrome by side to side portacaval shunt. Surg Gynecol Obstet. 1989;168:33–41.

    CAS  PubMed  Google Scholar 

  34. Wilkey D, Yocum DE, Oberley TD, et al. Budd-Chiari syndrome and renal failure in Behcet disease Report of a case and review of the literature. Am J Med. 1983;75:541–50.

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Venous Thromboembolism Unit, Department of Internal Medicine, Hospital General Universitario Gregorio Marañón, C/. Doctor Esquerdo, 46, 28007, Madrid, Spain

    Crhistian-Mario Oblitas, Neera Toledo-Samaniego, Francisco Galeano-Valle, Jorge Del-Toro-Cervera & Pablo Demelo-Rodriguez

  2. Department of Gastroenterology and Hepatology, Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Hospital General Universitario Gregorio Marañón, Madrid, Spain

    Ainhoa Fernández-Yunquera, Fernando Díaz-Fontenla & Rafael Bañares-Cañizares

  3. Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain

    Francisco Galeano-Valle, Jorge Del-Toro-Cervera, Rafael Bañares-Cañizares & Pablo Demelo-Rodriguez

  4. Universidad Complutense de Madrid, Madrid, Spain

    Crhistian-Mario Oblitas, Neera Toledo-Samaniego, Ainhoa Fernández-Yunquera, Fernando Díaz-Fontenla, Francisco Galeano-Valle, Jorge Del-Toro-Cervera, Rafael Bañares-Cañizares & Pablo Demelo-Rodriguez

  5. Universidad CEU San Pablo, Madrid, Spain

    Pablo Demelo-Rodriguez

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  1. Crhistian-Mario Oblitas
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  2. Neera Toledo-Samaniego
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Correspondence to Crhistian-Mario Oblitas.

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Oblitas, CM., Toledo-Samaniego, N., Fernández-Yunquera, A. et al. Chronic Budd–Chiari syndrome in Behçet's disease successfully managed with transjugular intrahepatic portosystemic shunt: a case report and literature review. Clin J Gastroenterol 13, 572–578 (2020). https://doi.org/10.1007/s12328-020-01106-7

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  • DOI: https://doi.org/10.1007/s12328-020-01106-7

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