Prediction of short- and long-term outcome in patients with autoimmune hepatitis.

UNLABELLED: Autoimmune hepatitis (AIH) is a chronic inflammatory disease characterized by a loss of tolerance toward the hepatocellular epithelium. Liver transplantation (LT) represents the ultimate therapeutic option for a fulminant course or end-stage liver disease. The aim of this study was to elucidate the clinical, serological, and genetic features of remission, relapse, and overall and LT-free survival. Between 2000 and 2014, 354 AIH patients from Hannover Medical School were included. Clinical, laboratory, and histological reports were analyzed. DRB1 allele analyses were performed in 264 AIH and 399 non-AIH patients. Cox's regression analysis was performed to identify factors significantly associated with survival. Patients diagnosed in childhood were at higher risk for relapses (P=0.003), requirement for LTs (P=0.014, log rank), and had a reduced life expectancy (P<0.001, log rank). Detection of soluble liver antigen/liver pancreas antigen (SLA/LP) antibodies was significantly associated with reduced overall and LT-free survival (P=0.037; P=0.021). Cirrhosis, which was evident in 25% at first diagnosis, was found to be a predictor of poor survival and requirement for LT (P=0.003; P=0.009). DRB1*04:01-positive phenotype was associated with a higher rate of complete remissions and with a lower frequency of cirrhosis and LTs. There were no significant differences for subsequent relapses or survival in patients achieving either partial or complete remission. CONCLUSION: Diagnosis<18 years, histological cirrhosis at first diagnosis and SLA/LP antibodies are major risk factors for a poor short- and long-term outcome. These patients are in need of high surveillance. Separating patients with positive SLA/LP antibodies into a third group may be reconsidered. DRB1*04:01 positivity has been identified in association with a favorable clinical outcome.

Long-term outcome of liver transplantation for autoimmune hepatitis.

BACKGROUND: Liver transplantation is the final therapeutic option for about 10% of patients with autoimmune hepatitis (AIH) who do not respond to medical therapy. The aim of this study was to evaluate the long-term outcome in serologically defined subgroups of AIH after transplantation. METHODS: Pre- and post-transplantation data of 28 patients with AIH transplanted between 1987 and 1999 were retrospectively analyzed and compared with 24 patients, who underwent liver transplantation because of Wilson's disease and glycogen storage disease type 1. RESULTS: Serological analyses identified patients with AIH type 1 (n = 13), type 2 (n = 5), and type 3 (n = 10). The 5-yr patient survival rate after liver transplantation was 78.2%, which was not significantly different from the control group. Six AIH patients and four control patients required re-transplantation because of initial non-function, chronic rejection or AIH recurrence. Patients transplanted for AIH (88%) had more episodes of acute rejection when compared with patients transplanted for genetic liver diseases (50%). Clinical and histological features of chronic rejection were present in four patients, which did not differ significantly from the controls. Recurrence of AIH was diagnosed in nine patients (32%) based upon the presence of autoantibodies, increased gamma-globulins, steroid dependency, and histological evidence of chronic hepatitis. These combined features were not found in any of the controls. CONCLUSIONS: Our data do not suggest that AIH subtypes influence prognosis after liver transplantation. Despite a high frequency of acute cellular rejection episodes and disease recurrence, transplantation for AIH has a 5-yr survival rate, which does not differ from that observed in patients transplanted for genetic liver diseases.