The impact of liver transplantation on the phenotype of primary biliary cirrhosis patients in the UK-PBC cohort

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• 作者：Greta Pells¹ ; ^&dagger ; ; George F. Mells² ; ³ ; ^&dagger ; ; Marco Carbone² ; ³ ; ^&dagger ; ; Julia L. Newton⁴ ; Andrew J. Bathgate⁵ ; Andrew K. Burroughs⁶ ; Michael A. Heneghan⁷ ; James M. Neuberger⁸ ; Darren B. Day² ; Samantha J. Ducker¹ ; The UK-PBC Consortium
• 关键词：PBC ; primary biliary cirrhosis ; UDCA ; ursodeoxycholic acid ; AMA ; anti-mitochondrial antibody ; ESS ; Epworth Sleepiness Scale ; OGS ; Orthostatic Grading Scale ; HADS ; Hospital Anxiety and Depression Scale
• 刊名：Journal of Hepatology
• 出版年：2013
• 出版时间：July, 2013
• 年：2013
• 卷：59
• 期：1
• 页码：67-73
• 全文大小：1264 K

文摘

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Background & Aims

Liver transplantation improves survival in end-stage primary biliary cirrhosis (PBC), but the benefit for systemic symptoms including fatigue is less clear. The aim of this study was to utilise the comprehensive UK-PBC Research Cohort, including 380 post-transplant patients and 2300 non-transplanted patients, to answer key questions regarding transplantation for PBC.

Methods

Cross-sectional study of post-transplant PBC patients and case-matched non-transplanted patients. Detailed clinical information was collected, together with patient systemic symptom impact data using validated assessment tools.

Results

Over 25 % of patients in the transplant cohort were grafted within 2 years of PBC diagnosis suggesting advanced disease at presentation. Transplanted patients were significantly younger at presentation than non-transplanted (mean 7 years) and >35 % of all patients in the UK-PBC cohort who presented under 50 years had already undergone liver transplantation at the study censor point (>50 % were treatment failures (post-transplant or unresponsive to UDCA)). Systemic symptom severity (fatigue and cognitive symptoms) was identical in female post-transplant patients and matched non-transplanted controls and unrelated to disease recurrence or immunosuppression type. In males, symptoms were worse in transplanted than in non-transplanted patients.

Conclusions

Age at presentation is a major risk factor for progression to transplant (as well as UDCA non-response) in PBC. Although both confirmatory longitudinal studies, and studies utilising objective as well as subjective measures of function, are needed if we are to address the question definitively, we found no evidence of improved systemic symptoms after liver transplantation in PBC and patients should be advised accordingly. Consideration needs to be given to enhancing rehabilitation approaches to improve function and life quality after liver transplant for PBC.