Early Predictors of Short‐Term Prognosis in Acute and Acute Severe Autoimmune Hepatitis

Abstract

Presentation of autoimmune hepatitis (AIH) can differ from nonacute to acute autoimmune hepatitis (A-AIH) with jaundice and acute severe autoimmune hepatitis (AS-AIH) with jaundice and coagulopathy. The aim of the study was to evaluate the short-term prognosis of different presentations of AIH and the influence of liver function improvement on short-term prognosis. In this single-center retrospective cohort study, AIH patients with repeatedly tested liver function at diagnosis and during at least 1 year of follow-up were included. A-AIH was defined as bilirubin >45 µmol and international normalized ratio (INR) <1.5. AS-AIH was defined as bilirubin level >45 µmol/L and INR ≥1.5. Of the 81 included patients, 17 (21%) presented with A-AIH, and 14 (17%) presented with AS-AIH. After the start of immunosuppressive therapy, bilirubin, albumin, and INR normalized in 70%, 77%, and 69%, respectively, in a median of 2.6 months, 3 months, and 4 weeks, respectively, in patients with A-AIH and AS-AIH. Liver transplantation (LT)-free survival rate was 100% in nonacute AIH, 94% in A-AIH, and 57% in AS-AIH at 12 months after diagnosis. An increase of INR or bilirubin at 2 weeks was the best predictive factor for the need of LT within 12 months with a Youden's index of 0.85. A-AIH was present in 21%, and AS-AIH was present in 17% of AIH patients. In the majority of patients, bilirubin, albumin, and INR normalized in the first months of treatment. Deterioration of liver function after 2 weeks of treatment should lead to rapid evaluation for LT and consideration of second-line medication.

Development and validation of a prognostic score for long‐term transplant‐free survival in autoimmune hepatitis type 1

“…After excluding patients with decompensated cirrhosis, the predictive value of the model remained similar (C‐statistic of 0.823). Patients presenting with acute severe AIH have also a decreased survival compared to AIH patients in general 32 . After excluding patients with acute severe AIH, the HR's remained similar.…”

“…31 The long term prognosis of AIH pa- compared to AIH patients in general. 32 After excluding patients with acute severe AIH, the HR's remained similar.…”

Autoimmune liver diseases, hepatocellular carcinoma, and recurrence of autoimmunity post-liver transplantation

“…A higher rate of non-response to corticosteroids is associated with a younger age at diagnosis, an acute presentation, higher bilirubin, a Model for End Stage Liver Disease (MELD) score greater than 12 at diagnosis, and the presence of HLA DRB1*03 [44] . For patients presenting with jaundice, failure of the MELD score to improve after 7 days is another predictor of corticosteroid treatment failure [45] . Accordingly, early triage of non-response to treatment is important to identify with acute AIH for patients who may progress to acute liver failure and require an urgent LT workup.…”

Aminotransferases During Treatment Predict Long-Term Survival in Patients With Autoimmune Hepatitis Type 1: A Landmark Analysis

“…For such patients, the 2-week response of international normalized ratio and bilirubin indicates short-term prognosis. 29 Values at especially 24 and 36 months were available in a smaller number of patients. Owing to the relatively good prognosis of AIH with treatment, only a limited number of events occurred, which limits the number of variables that could be included in multivariate analysis.…”