Published online Apr 7, 2018. doi: 10.3748/wjg.v24.i13.1410
Peer-review started: December 6, 2017
First decision: January 18, 2018
Revised: February 21, 2018
Accepted: March 3, 2018
Article in press: March 3, 2018
Published online: April 7, 2018
To investigate potential triggering factors leading to acute liver failure (ALF) as the initial presentation of autoimmune hepatitis (AIH).
A total of 565 patients treated at our Department between 2005 and 2017 for histologically-proven AIH were retrospectively analyzed. However, 52 patients (9.2%) fulfilled the criteria for ALF defined by the “American Association for the Study of the Liver (AASLD)”. According to this definition, patients with “acute-on-chronic” or “acute-on-cirrhosis” liver failure were excluded. Following parameters with focus on potential triggering factors were evaluated: Patients’ demographics, causation of liver failure, laboratory data (liver enzymes, MELD-score, autoimmune markers, virus serology), liver histology, immunosuppressive regime, and finally, outcome of our patients.
The majority of patients with ALF were female (84.6%) and mean age was 43.6 ± 14.9 years. Interestingly, none of the patients with ALF was positive for anti-liver kidney microsomal antibody (LKM). We could identify potential triggering factors in 26/52 (50.0%) of previously healthy patients presenting ALF as their first manifestation of AIH. These were drug-induced ALF (57.7%), virus-induced ALF (30.8%), and preceding surgery in general anesthesia (11.5%), respectively. Unfortunately, 6 out of 52 patients (11.5%) did not survive ALF and 3 patients (5.7%) underwent liver transplantation (LT). Comparing data of survivors and patients with non-recovery following treatment, MELD-score (P < 0.001), age (P < 0.05), creatinine (P < 0.01), and finally, ALT-values (P < 0.05) reached statistical significance.
Drugs, viral infections, and previous surgery may trigger ALF as the initial presentation of AIH. Advanced age and high MELD-score were associated with lethal outcome.
Core tip: Autoimmune hepatitis is considered to manifest as a chronic disease. In few cases, the clinician is challenged with patients revealing acute liver failure as their first manifestation of autoimmune hepatitis (AIH). The aim of our study was to investigate features of especially these patients with focus on potential triggering factors. We identified triggering factors in half of our patients (26 out of 52 patients with acute liver failure within a total cohort of 565 AIH patients). These were drugs, viral infections, and surgery. Advanced age and high MELD-score were associated with lethal outcome. Consequently, the clinician would be well-advised to document these underlying conditions.