The Budd–Chiari Syndrome
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《新英格兰医药杂志》
To the Editor: We would like to comment on the definitions and classification used in the comprehensive review of the Budd–Chiari syndrome by Menon et al. (Feb. 5 issue).1 Agreement on a uniform nomenclature is an essential requirement for clinical practice and future collaborative studies. Veno-occlusive disease, also referred to as the sinusoidal obstruction syndrome, is defined as nonthrombotic obstruction of sinusoids or central hepatic veins due to toxic injury of the sinusoidal wall.2 The epidemiology, pathophysiology, and treatment of veno-occlusive disease are so distinct from those of the Budd–Chiari syndrome that it is generally considered a separate disease entity.3 In contrast to what is suggested in the review, the Budd–Chiari syndrome also does not include venous obstruction at the level of the right atrium.4
Treatment recommendations according to classification of the Budd–Chiari syndrome as fulminant, acute, subacute, or chronic have not been shown to be clinically relevant. Many patients have an acute-on-chronic form of the Budd–Chiari syndrome, and to guide physicians' management, classification should be based on factors influencing the prognosis.5,6 These factors should derive from large, ongoing, prospective, multicenter cohort studies.3 We prefer to use a purely descriptive stratification until such a classification has been validated.
Harry L.A. Janssen, M.D., Ph.D.
Erasmus University Medical Center Rotterdam
3015 GD Rotterdam, the Netherlands
h.janssen@erasmusmc.nl
Juan-Carlos Garcia-Pagan, M.D., Ph.D.
Hospital Clinic Barcelona
08036 Barcelona, Spain
Dominique-Charles Valla, M.D., Ph.D.
H?pital Beaujon
92110 Clichy, France
References
Menon KVN, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J Med 2004;350:578-585.
DeLeve LD, Shulman HM, McDonald GB. Toxic injury to hepatic sinusoids: sinusoidal obstruction syndrome (veno-occlusive disease). Semin Liver Dis 2002;22:27-42.
Janssen HL, Garcia-Pagan JC, Elias E, Mentha G, Hadengue A, Valla DC. Budd-Chiari syndrome: a review by an expert panel. J Hepatol 2003;38:364-371.
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;65:51-55.
Langlet P, Escolano S, Valla D, et al. Clinicopathological forms and prognostic index in Budd-Chiari syndrome. J Hepatol 2003;39:496-501.
Murad SD, Valla DC, de Groen PC, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology 2004;39:500-508.
To the Editor: Menon et al. describe several clinical manifestations of the Budd–Chiari syndrome, but an additional important aspect is that of prognostic factors that help determine outcome and management. A recent multicenter study of 237 patients evaluated determinants of survival in the Budd–Chiari syndrome.1 Hepatic encephalopathy, ascites, the bilirubin level, and the prothrombin time were found to have prognostic significance, and a prognostic index grouped patients into three classes: class I, in which the prognosis was good (five-year survival rate, 89 percent); class II, in which the prognosis was intermediate (five-year survival rate, 74 percent); and class III, in which the prognosis was poor (five-year survival rate, 42 percent). This classification was useful for determining intervention strategies in relation to survival; patients in class II who received a portosystemic shunt had a better outcome than those in class II who underwent other types of intervention. Menon et al. make useful recommendations about decisions regarding portosystemic shunts, but an objective prognostic index may be more appropriate for deciding which patients with the Budd–Chiari syndrome will benefit from the use of a portosystemic shunt.
Andres Cardenas, M.D.
Harvard Medical School
Boston, MA 02115
acardena@bidmc.harvard.edu
References
Murad SD, Valla DC, de Groen PC, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology 2004;39:500-508.
The authors reply: As Dr. Janssen and colleagues and Dr. Cardenas note, prospectively validated classifications based on factors that influence the prognosis in the Budd–Chiari syndrome would indeed be useful for stratifying risk and guiding therapy in cases of this challenging condition. The authors of two of the cited studies,1,2 which were published after our review article had been accepted for publication, should be commended for their initial efforts in this direction. In contrast to prospectively validated classifications, retrospectively derived scoring systems have the drawback that currently available therapies may not have been available for all the patients in the database. In fact, the study on prognostic classification in patients with the Budd–Chiari syndrome,2 referred to by Dr. Janssen and colleagues and Dr. Cardenas, included patients from our institution at a time when transjugular intrahepatic portosystemic shunts were not available as a treatment approach. Therefore, the use of this scoring system to determine whether a patient will benefit from this type of shunt may not be completely valid.
Until such scoring systems are prospectively validated in centers in which all treatment options are available, we prefer a classification that is based on clinical presentation and that helps guide therapy. Indeed, owing to the heterogeneous features and manifestations of the Budd–Chiari syndrome, therapy often needs to be tailored to the individual patient, according to local expertise and the availability of therapeutic options.
K.V. Narayanan Menon, M.D.
Vijay Shah, M.D.
Patrick S. Kamath, M.D.
Mayo Clinic College of Medicine
Rochester, MN 55905
References
Langlet P, Escolano S, Valla D, et al. Clinicopathological forms and prognostic index in Budd-Chiari syndrome. J Hepatol 2003;39:496-501.
Murad SD, Valla DC, de Groen PC, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology 2004;39:500-508.
Treatment recommendations according to classification of the Budd–Chiari syndrome as fulminant, acute, subacute, or chronic have not been shown to be clinically relevant. Many patients have an acute-on-chronic form of the Budd–Chiari syndrome, and to guide physicians' management, classification should be based on factors influencing the prognosis.5,6 These factors should derive from large, ongoing, prospective, multicenter cohort studies.3 We prefer to use a purely descriptive stratification until such a classification has been validated.
Harry L.A. Janssen, M.D., Ph.D.
Erasmus University Medical Center Rotterdam
3015 GD Rotterdam, the Netherlands
h.janssen@erasmusmc.nl
Juan-Carlos Garcia-Pagan, M.D., Ph.D.
Hospital Clinic Barcelona
08036 Barcelona, Spain
Dominique-Charles Valla, M.D., Ph.D.
H?pital Beaujon
92110 Clichy, France
References
Menon KVN, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J Med 2004;350:578-585.
DeLeve LD, Shulman HM, McDonald GB. Toxic injury to hepatic sinusoids: sinusoidal obstruction syndrome (veno-occlusive disease). Semin Liver Dis 2002;22:27-42.
Janssen HL, Garcia-Pagan JC, Elias E, Mentha G, Hadengue A, Valla DC. Budd-Chiari syndrome: a review by an expert panel. J Hepatol 2003;38:364-371.
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;65:51-55.
Langlet P, Escolano S, Valla D, et al. Clinicopathological forms and prognostic index in Budd-Chiari syndrome. J Hepatol 2003;39:496-501.
Murad SD, Valla DC, de Groen PC, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology 2004;39:500-508.
To the Editor: Menon et al. describe several clinical manifestations of the Budd–Chiari syndrome, but an additional important aspect is that of prognostic factors that help determine outcome and management. A recent multicenter study of 237 patients evaluated determinants of survival in the Budd–Chiari syndrome.1 Hepatic encephalopathy, ascites, the bilirubin level, and the prothrombin time were found to have prognostic significance, and a prognostic index grouped patients into three classes: class I, in which the prognosis was good (five-year survival rate, 89 percent); class II, in which the prognosis was intermediate (five-year survival rate, 74 percent); and class III, in which the prognosis was poor (five-year survival rate, 42 percent). This classification was useful for determining intervention strategies in relation to survival; patients in class II who received a portosystemic shunt had a better outcome than those in class II who underwent other types of intervention. Menon et al. make useful recommendations about decisions regarding portosystemic shunts, but an objective prognostic index may be more appropriate for deciding which patients with the Budd–Chiari syndrome will benefit from the use of a portosystemic shunt.
Andres Cardenas, M.D.
Harvard Medical School
Boston, MA 02115
acardena@bidmc.harvard.edu
References
Murad SD, Valla DC, de Groen PC, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology 2004;39:500-508.
The authors reply: As Dr. Janssen and colleagues and Dr. Cardenas note, prospectively validated classifications based on factors that influence the prognosis in the Budd–Chiari syndrome would indeed be useful for stratifying risk and guiding therapy in cases of this challenging condition. The authors of two of the cited studies,1,2 which were published after our review article had been accepted for publication, should be commended for their initial efforts in this direction. In contrast to prospectively validated classifications, retrospectively derived scoring systems have the drawback that currently available therapies may not have been available for all the patients in the database. In fact, the study on prognostic classification in patients with the Budd–Chiari syndrome,2 referred to by Dr. Janssen and colleagues and Dr. Cardenas, included patients from our institution at a time when transjugular intrahepatic portosystemic shunts were not available as a treatment approach. Therefore, the use of this scoring system to determine whether a patient will benefit from this type of shunt may not be completely valid.
Until such scoring systems are prospectively validated in centers in which all treatment options are available, we prefer a classification that is based on clinical presentation and that helps guide therapy. Indeed, owing to the heterogeneous features and manifestations of the Budd–Chiari syndrome, therapy often needs to be tailored to the individual patient, according to local expertise and the availability of therapeutic options.
K.V. Narayanan Menon, M.D.
Vijay Shah, M.D.
Patrick S. Kamath, M.D.
Mayo Clinic College of Medicine
Rochester, MN 55905
References
Langlet P, Escolano S, Valla D, et al. Clinicopathological forms and prognostic index in Budd-Chiari syndrome. J Hepatol 2003;39:496-501.
Murad SD, Valla DC, de Groen PC, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology 2004;39:500-508.