Asymptomatic primary biliary cirrhosis: clinical features, prognosis, and symptom progression in a large population based cohort

Prince, M. I.; Chetwynd, A.; Craig, W. L.; Metcalf, J. V.; James, O. F. W.
June 2004
Gut;Jun2004, Vol. 53 Issue 6, p865
Academic Journal
Background: Many patients with primary biliary cirrhosis (PBC) are asymptomatic at the time of diagnosis. However, because most studies of asymptomatic PBC have been small and from tertiary centres, asymptomatic PBC remains poorly characterised. Aims: To describe the features and progression of initially asymptomatic PBC patients. Methods: Follow up by interview and note review of a large geographically and temporally defined cohort of patients with PBC, collected by multiple methods. Results: Of a total of 770 patients, 469 (61%) were asymptomatic at diagnosis. These patients had biochemically and histologically less advanced disease than initially symptomatic patients. Median survival was similar in both groups (9.6 v 8.0 years, respectively) possibly due to excess of non-liver related deaths in asymptomatic patients (31% v 57% of deaths related to liver disease). Survival in initially asymptomatic patients was not affected by subsequent symptom development. By the end of follow up, 20% of initially asymptomatic patients had died of liver disease or required liver transplantation. The majority of initially asymptomatic patients developed symptoms of liver disease if they were followed up for long enough (Kaplan-Meier estimate of proportion developing symptoms: 50% after five years, 95% after 20 years). However, 45% of patients remained asymptomatic at the time of death. Conclusions: Although asymptomatic PBC is less severe at diagnosis than symptomatic disease, it is not associated with a better prognosis, possibly due to an increase in non-hepatic deaths. The reasons for this are unclear but may reflect confounding by other risk factors or surveillance bias. These findings have important implications for future treatment strategies.


Related Articles

  • Organ tolerance following cadaveric liver transplantation for chronic graft-versus-host disease after allogeneic bone marrow transplantation. Urban, Ch.; Deutschmann, A.; Kerbl, R.; H., Lackner; Schwinger, W.; Konigsrainer, A.; Margreiter, R. // Bone Marrow Transplantation;10/15/2002, Vol. 30 Issue 8, p535 

    Presents a study that examined the effect of orthotopic liver transplantation after the development of cirrhosis of the liver. Details on the dysfunction of the liver; Causes of liver failure; Information on haematolymphopoietic chimerism.

  • Prognostic assessment in patients with hepatic encephalopathy. García-Martínez, Rita; Simón-Talero, Macarena; Córdoba, Juan // Disease Markers;2011, Vol. 31 Issue 3, p171 

    Hepatic encephalopathy (HE) is a common complication of liver failure that is associated with poor prognosis. However, the prognosis is not uniform and depends on the underlying liver disease. Acute liver failure is an uncommon cause of HE that carries bad prognosis but is potentially...

  • Retrasplante hepático. Primer reporte en México. Mendoza-Sánchez, Federico; Herrera-Rodríguez, Roberto; López-Bañuelos, Carlos; Romero-Beltrán, Omar; Navarro-López, Ricardo; Ramírez-Ramos, José // Cirugia y Cirujanos;ene/feb2009, Vol. 77 Issue 1, p65 

    Background: The only therapeutic option for patients with hepatic graft failure is retransplant. In Mexico, the number of hepatic transplants is scarce and is even less for retransplants. There are no reports of patients with good results after hepatic retransplant. Here we present the first...

  • Reversal of severe hepatopulmonary syndrome in chronic hepatic cirrhosis by living donor liver transplantation: Report of two cases. KEFEI CHEN; BO LI // Surgery Today;Mar2011, Vol. 41 Issue 3, p441 

    Severe hepatopulmonary syndrome (HPS) is a rare complication of decompensated liver cirrhosis and a relative contraindication for liver transplantation. This report describes the treatment of two cases with severe HPS resulting from chronic liver failure by living donor liver transplantation...

  • External validation of a prognostic model for predicting survival of cirrhotic patients with refractory ascites. Guardiola, Jordi; Baliellas, Carme; Xiol, Xavier; Fernandez Esparrach, Glòria; Ginès, Pere; Ventura, Pere; Vazquez, Santiago // American Journal of Gastroenterology;Sep2002, Vol. 97 Issue 9, p2374 

    OBJECTIVE:Cirrhotic patients with refractory ascites (RA) have a poor prognosis, although individual survival varies greatly. A model that could predict survival for patients with RA would be helpful in planning treatment. Moreover, in cases of potential liver transplantation, a model of these...

  • The Use of Screening and Preventive Therapies for Gastroesophageal Varices in Patients Referred for Evaluation of Orthotopic Liver Transplantation. Arguedas, Miguel R.; McGuire, Brendan M.; Fallon, Michael B.; Abrams, Gary A. // American Journal of Gastroenterology;Mar2001, Vol. 96 Issue 3, p833 

    OBJECTIVE: Screening for varices has been recommended in patients with cirrhosis to prevent variceal hemorrhage (primary prophylaxis). In addition, therapy is recommended after the initial episode of variceal bleeding to prevent recurrence (secondary prophylaxis). However, the degree of...

  • ACUTE LIVER FAILURE IN SCOTLAND: AN OBSERVATIONAL STUDY 1992-2003.  // Gut;Apr2004 Supplement 3, Vol. 53, pA92 

    The article presents an observational study of acute liver failure (ALF) in Scotland. Acute liver failure is an uncommon clinical problem. The causes and outcomes differ in different populations. The provision of a single liver transplantation unit in Scotland has allowed the study of acute...

  • Fasting and postprandial plasma ghrelin levels are decreased in patients with liver failure previous to liver transplantation. Maria Diz-Lois; Jesús Garcia-Buela; Francisco Suarez; Ovidio Vidal // Endocrine (1355008X);Jun2009, Vol. 35 Issue 3, p467 

    Abstract  Anorexia is a problem of paramount importance in patients with advanced liver failure. Ghrelin has important actions on feeding and weight homeostasis. Concentrations of ghrelin are controversial in liver cirrhosis. Our aim was to study fasting ghrelin and their response to an...

  • An Update on Hepatic Stem Cells: Bench to Bedside. Parveen, N.; Aleem, A. K.; Habeeb, M. A.; Habibullah, C. M. // Current Pharmaceutical Biotechnology;Feb2011, Vol. 12 Issue 2, p226 

    No abstract available.


Read the Article


Sign out of this library

Other Topics