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
Autoimmune hepatitis (AIH) is generally considered to manifest as a chronic liver disease. So far, only limited data are available investigating patients presenting a fulminant acute liver failure as a first manifestation of this autoimmune disorder. The significance of our study was therefore to investigate the circumstances leading to acute liver failure and onset of autoimmune hepatitis.
In the daily clinical setting, the hepatologist is frequently faced with patients demonstrating only a mild elevation of their liver enzymes. Routine work-up of these cases leads finally to the diagnosis of underlying AIH. However, in few cases, one is challenged with patients without any signs of hepatopathy but rapidly developing a life-threatening acute liver failure (ALF) as their first manifestation of AIH. We here presented potential triggering factors which may activate the “autoimmune machinery” leading to ALF.
The main objective of the present study was to gather more information with focus on potential triggering factors leading to acute presentation of AIH with consecutive liver failure. The clinician would be well-advised to accurately document these underlying conditions.
In our retrospective cohort study we investigated patients with histologically-proven AIH and further analyzed the patients who presented acute liver failure. Patients’ demographics, laboratory data, immunosuppressive regime, histology, and outcome were documented and studied.
We were able to identify potential triggering factors in 26/52 (50.0%) of our previously healthy patients presenting ALF as their first manifestation of AIH. These were drug-induced (e.g., non-steroidal anti-inflammatory drugs and antibiotics) ALF (57.7%), virus-induced (Epstein-Barr, Cytomegalovirus and HEV) ALF (30.8%), and surgery in general anesthesia (11.5%), respectively.
Approximately 9% of our patients were diagnosed with ALF as their initial presentation of AIH which may be potentially induced by drugs, viral infections, and surgery in general anesthesia. Consequently, the clinician would be well-advised to ask his patients for hepato-toxic drugs and accurately document these underlying conditions. Increases of age, MELD-score, and creatinine levels were associated with lethal outcome or need for urgent liver transplantation.
With our study and findings we hope to further attract the physician’s attention especially in cases of acute liver failure induced by autoimmune hepatitis. In some cases, these disorders may be triggered by drugs and hepato-tropic viruses. We hope that more studies investigating acute liver failure as a first manifestation of AIH will be available in future.