EASL Clinical Practice Guidelines

Introduction

Autoimmune hepatitis (AIH) was the first liver disease for which an effective therapeutic intervention, corticosteroid treatment, was convincingly demonstrated in controlled clinical trials. However, 50 years later AIH still remains a major diagnostic and therapeutic challenge. There are two major reasons for this apparent contradiction: Firstly, AIH is a relatively rare disease. Secondly, AIH is a very heterogeneous disease.

Like other rare diseases, clinical studies are hampered by the limited number of patients that can be included in trials. Possibly and more importantly, the interest of the pharmaceutical industry to develop effective specific therapies for rare diseases is limited due to the very restricted market for such products. The wide heterogeneity of affected patients and clinical manifestations of the disease limits both diagnostic and further therapeutic studies. AIH’s age spectrum is extremely wide, it can affect small infants and can manifest for the first time in octogenarians. AIH can run a very mild subclinical course or be very acute, rarely leading to fulminant hepatic failure. AIH sometimes demonstrates quite dramatic disease fluctuations with periods of apparent spontaneous remissions, acute flares and/or smouldering disease. AIH can be associated with a number of other hepatic conditions, in particular the cholestatic liver diseases; primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC), but also with drug-induced liver injury (DILI), alcoholic or non-alcoholic steatohepatitis (NASH) or viral hepatitis. Each condition provides special diagnostic and therapeutic challenges. Despite these challenges and complexities, diagnosis and treatment of AIH has seen striking progress, and now patients in specialised centres have an excellent prognosis, both in respect to survival and to quality of life.

The aim of the present Clinical Practice Guideline (CPG) is to provide guidance to hepatologists and general physicians in the diagnosis and treatment of AIH in order to improve care for affected patients. In view of the limited data from large controlled studies and trials, many recommendations are based on expert consensus. This is to some extent a limitation of this EASL-CPG, but at the same time it is its special strength: consensus in this guideline is based on intensive discussions of experts from large treatment centres. The core consensus group has experience of over one thousand AIH patients managed personally, and the recommendations have been reviewed by both the EASL Governing Board as well as external experts, who have a similarly wide personal experience. Therefore, the guidelines are a resource of information and recommendations based on the largest experience available thus far. At the same time, we formulate key scientific questions that result from the consensus discussions on the limitations of our knowledge. All recommendations of this CPG were agreed upon unanimously (100%) consensus. Grading of the recommendations is based on the GRADE system for evidence (Table 1) [1].

Table 1
Grading of recommendations.

Adapted from [1]