We read with great interest and agreement the review article by Czaja  who reported the management of autoimmune hepatitis (AIH) beyond consensus guidelines. As he described in the review, ‘highly individualised clinical judgments are required at decision points that are outside confident guidelines, and these judgments can be difficult and controversial’.
The aetiology of fulminant hepatitis in our unit was due to AIH in 29.3%, after the establishment of the international AIH criteria. In a recent Japanese nationwide survey, the number of patients with AIH showing the histological features of acute hepatitis, including acute liver failure (ALF), has been increasing. The US ALF Study Group reported that 58% of indeterminate ALF were considered autoimmune ALF. Therefore, AIH is a major aetiology of ALF worldwide.
The survival rate without liver transplantation (LT) of patients with fulminant AIH is poor, which is recognised everywhere around the world. LT should be considered, but in Japan where the serious problems of a shortage of donor livers exist, the treatment decision against immunosuppressive therapy is difficult. Czaja suggested that failure to improve within 2 weeks of immunosuppression indicates the need of emergency LT.
We applaud his efforts for diagnosing and treating this intractable liver disease, and dare to present one uncertain but important data based on ‘low-quality clinical evidence and conflicting experiences’, as hepatologists struggling against intractable liver diseases, including AIH, in Japan. Mean duration between introduction of immunosuppression and onset of sepsis was 14.8 ± 9.7 days in our autoimmune ALF patients (Figure 1). Therefore, we also suppose that the 2-week duration after introduction of immunosuppression is indeed a critical point for avoiding infectious complications and considering LT, although the most important treatment strategy is to diagnose and treat acute onset AIH before the development of ALF.