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

Abstract

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 5 0.003), requirement for LTs (P 5 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 5 0.037; P 5 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 5 0.003; P 5 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. (HEPATOLOGY 2015;62:1524-1535

The utility of repeat liver biopsy in autoimmune hepatitis: a series of 20 consecutive cases

“…16 These patients are also at increased risk for liver failure and the need for liver transplantation. 17 Hepatic fibrosis improves in more than half of AIH patients who received immunosuppressive therapy, while progressive fibrosis slows or is prevented in almost 80% of these patients. 4 Seven patients (35%) in our series showed progression of fibrosis, all showing newly diagnosed cirrhosis.…”

The management of childhood liver diseases in adulthood

“…Differences in autoimmune liver disease between children and adults is summarised in Table 5. Patients diagnosed with AIH under the age of 18 years have an increased risk for disease relapse, are more likely to need liver transplantation and have a reduced life expectancy compared to adults [94,95].…”

Immunopathogenic Mechanisms of Autoimmune Hepatitis: How Much Do We Know from Animal Models?

“…In addition, patients carrying DRB1*03:01 generate higher immunoglobulin G levels [19]. In contrast, individuals with HLA-DR4 display a higher rate of complete remissions alongside a lower frequency of cirrhosis and are thus associated with a more favorable clinical outcome [20]. …”