Clinicopathological forms and prognostic index in Budd-Chiari syndrome
Introduction
Studying a cohort of adult patients with non-tumorous Budd-Chiari syndrome (BCS) and a patent portal vein, we recently brought out [1] that: (1) the outcome of the syndrome has improved in recent years (i.e. since 1985); (2) age, Pugh score, ascites score and serum creatinine at the time of diagnosis have independent prognostic values and can be combined into a clinically useful prognostic index; and (3) after adjustment on these prognostic factors, surgical portosystemic shunting (SPSS) had no detectable impact on survival. In this study, liver biopsy findings appeared of no prognostic value raising the issue of the absence of significance of pathological data [1]. Indeed, there are limitations to the use of pathological data for determining the presence of acute injury (grade) [2] and/or chronic lesions (stage) [3] mainly due to the heterogeneity in the distribution of lesions resulting in a high rate of sampling variability [4]. This prompted us to design a novel clinicopathological classification of BCS [5] taking into account all clinical, radiological, biochemical and histological information available at the time of diagnosis for grading and staging the disease. In this classification the three following clinicopathological forms are separated: type I form (acute injury only, corresponding to the onset of hepatic outflow obstruction); type II form (chronic lesions only, corresponding to the sequel of remote hepatic outflow obstruction); and type III form (acute injury superimposed on chronic lesions). The prognostic value of this novel classification has been demonstrated in children [5], but not yet in adults.
The aims of this work were: (1) in an independent sample of adult patients with BCS, to validate the prognostic value of the previously reported prognostic index; (2) to assess the prognostic significance of the novel clinicopathological classification in adult patients; and (3) to evaluate if adjustment for this classification would uncover a significant effect of SPSS on survival.
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Patients and methods
Six specialized medical and/or surgical units participated in this retrospective survey. Consecutive adult patients with BCS seen between January 1985 and December 1997 were enrolled. As our first survey ended in December 1992, patients seen between January 1985 and December 1992 (i.e. 54 patients) by the three departments which participated to the previous survey were not included in this series. Criteria of inclusion were similar to those defined in our previous study. Briefly, patients were
Characteristics of patients
Out of the 87 unselected adult patients with BCS admitted to the six units participating to this study, 73 patients fitted the criteria of inclusion. Fourteen patients were not included for the following reasons: three had cancer; three had portal vein thrombosis; one had both; three were treated with thrombectomy; and four others were treated with angioplasty plus hepatic vein stenting. No liver transplantation was performed as a first treatment during the survey period. Out of these 73
Discussion
This study in patients with BCS confirms the conclusions from our previous multivariate analysis regarding overall survival, the validity of the previously reported prognostic index, and the absence of significant impact of SPSS on overall survival [1]. In addition, the present study shows that, similar to children, in adult BCS patients, features of simultaneous acute injury and chronic lesions (type III clinicopathological forms) are independently associated with a poor outcome as compared to
Acknowledgements
We are thankful to The members of the Observatoire National du Syndrome de Budd-Chiari for their continuous support. We gratefully acknowledge the unrestricted financial support provided by the Société Nationale Française de Gastro-Entérologie and Laboratoires Choay.
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