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Timing of Treatment for Budd–Chiari Syndrome: Still an Open Issue

  • Andrea Mancuso
    Correspondence
    Address for correspondence: Prof Andrea Mancuso, Centro di Riferimento Regionale Malattie Rare, Sindrome di Budd-Chiari e Teleangectasia Emorragica Ereditaria, Medicina Interna 1, ARNAS Civico - Di Cristina - Benfratelli, Piazzale Leotta 4, 90100, Palermo, Italy.
    Affiliations
    Centro di Riferimento Regionale Malattie Rare, Sindrome di Budd-Chiari e Teleangectasia Emorragica Ereditaria, Medicina Interna 1, ARNAS Civico - Di Cristina - Benfratelli, Palermo, Italy
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      I read with interest the paper published in Journal of Clinical and Experimental Hepatology, reporting data on 138 patients with Budd–Chiari syndrome (BCS) who were treated with medical therapy alone, including anticoagulation, of whom 55% had complete response (CR), 18% partial response (PR) and 26% no response (NR). None with PR or NR had CR later. Moreover, at a median follow-up of 40 months, lost of response (LoR) was more common in the PR group than in the CR group and was significantly associated with the presence of ascites at presentation. Furthermore, mortality was higher in NR compared with CR and PR. Anyway, the all-group 10-year survival was only 33%.
      • Agrawal D.
      • Gupta D.
      • Nathani R.
      • et al.
      Presence of ascites at presentation is associated with absence of long-term response amongst patients with budd-chiari syndrome when treated with medical therapy alone: a single centre real-life experience.
      Overall, the results of this study forcefully enter the debate about timing of interventional treatment for BCS.
      • Agrawal D.
      • Gupta D.
      • Nathani R.
      • et al.
      Presence of ascites at presentation is associated with absence of long-term response amongst patients with budd-chiari syndrome when treated with medical therapy alone: a single centre real-life experience.
      ,
      • Mancuso A.
      Timing of TIPS in Budd chiari syndrome: an Italian hepatologist's perspective.
      Actually, there are two main trends for BCS management, both lacking formal validation, because of the rarity of BCS. The most popular indication for BCS management is the so-called step-by-step strategy, suggesting medical therapy as first line, revascularization or transjugular intrahepatic portosystemic shunt if no response to medical therapy, and liver transplant as rescue therapy.
      • Northup P.G.
      • Garcia-Pagan J.C.
      • Garcia-Tsao G.
      • et al.
      Vascular liver disorders, portal vein thrombosis, and procedural bleeding in patients with liver disease: 2020 practice guidance by the American association for the study of liver diseases.
      However, controversies exist about the management of BCS. In fact, the step-by-step strategy is based on arbitrary criteria requiring validation and is not evidence based. Moreover, the present study, together with a previous one, suggests that only medical therapy results in survival of only one-third of the cases after long-term follow-up.
      • Agrawal D.
      • Gupta D.
      • Nathani R.
      • et al.
      Presence of ascites at presentation is associated with absence of long-term response amongst patients with budd-chiari syndrome when treated with medical therapy alone: a single centre real-life experience.
      ,
      • Seijo S.
      • Plessier A.
      • Hoekstra J.
      • et al.
      Good longterm outcome of Budd-Chiari syndrome with a step-wise management.
      Consequently, a real alternative to the step-by-step strategy is the so-called Early Interventional Treatment strategy, proposing that intervention, in the presence of any sign of portal hypertension, could improve outcome.
      • Mancuso A.
      An update on management of Budd-Chiari syndrome: the issues of timing and choice of treatment.
      In fact, following the latter strategy, excellent survival was reported both in East and West.
      • He F.
      • Zhao H.
      • Dai S.
      • et al.
      Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome with diffuse occlusion of hepatic veins.
      ,
      • Rosenqvist K.
      • Sheikhi R.
      • Eriksson L.G.
      • et al.
      Endovascular treatment of symptomatic Budd-Chiari syndrome - in favour of early transjugular intrahepatic portosystemic shunt.
      Anyway, it is noteworthy that for both strategies, ascites not rapidly responding to medical therapy is a clear indication for further intervention.
      • Northup P.G.
      • Garcia-Pagan J.C.
      • Garcia-Tsao G.
      • et al.
      Vascular liver disorders, portal vein thrombosis, and procedural bleeding in patients with liver disease: 2020 practice guidance by the American association for the study of liver diseases.
      ,
      • Mancuso A.
      An update on management of Budd-Chiari syndrome: the issues of timing and choice of treatment.
      However, as the present study confirms, in real life, BCS management is not ruled by a sharp indication but follows different attitudes.
      Consequently, identifying which subgroup of patients could safely go on sole medical Therapy and which one could benefit early intervention is a crucial issue for the management of BCS. Recently, liver stiffness (LS), a noninvasive estimate of liver fibrosis, although previously considered inappropriate for BCS because of liver congestion, was reported as a promising tool to predict the outcome for BCS on sole medical therapy,
      • Mancuso A.
      • Amata M.
      • Politi F.
      • Mitra M.
      • Marsala M.G.L.
      • Maringhini A.
      Controversies in Budd-Chiari syndrome management: potential role of liver stiffness.
      but further data are needed for confirmation.
      Finally, the present considerations are furtherly complicated by geographical differences about BCS regarding both etiologies and management.
      • Qi X.
      • Han G.
      • Guo X.
      • et al.
      Review article: etiology of primary Budd-Chiari syndrome: difference between West and China.
      ,
      • Mancuso A.
      Budd-Chiari syndrome in the West and the East: same syndrome, different diseases.
      In conclusion, further data are needed to define the optimal timing of BCS treatment.
      Due to ongoing absence of critical clinical factors, a comparison between traditional management with early interventional treatment should be adviced.

      Credit authorship contribution statement

      Author declares that he is solely responsible for the paper.

      Conflicts of interest

      None.

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        Timing of TIPS in Budd chiari syndrome: an Italian hepatologist's perspective.
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        Vascular liver disorders, portal vein thrombosis, and procedural bleeding in patients with liver disease: 2020 practice guidance by the American association for the study of liver diseases.
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        Good longterm outcome of Budd-Chiari syndrome with a step-wise management.
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        An update on management of Budd-Chiari syndrome: the issues of timing and choice of treatment.
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        Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome with diffuse occlusion of hepatic veins.
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