Science of the Month - July 2021
Noguchi F, Chu PS, Taniki N, Yoshida A, Morikawa R, Yamaguchi A, Ikura A, Yamataka K, Hoshi H, Usui S, Ebinuma H, Saito H, Kanai T, Nakamoto N
Autoimmune hepatitis (AIH) is an intractable immunological disease with an unknown pathogenesis, and its prevalence is increasing worldwide, including in Japan. In particular, severe acute-onset AIH (SA-AIH) often shows resistance to steroids, the standard medical treatment. There are no criteria for deciding whether to intensify immunosuppressive therapy or proceed with liver transplantation in such cases, and international guidelines lack clear evidence regarding recommended second-line therapeutic agents. In this study, we conducted a detailed examination of eight of the 26 cases of SA-AIH experienced in our department, in which steroid treatment was insufficiently effective and second-line therapy with the calcineurin inhibitor cyclosporine A (CsA) was introduced. We reported that on day 7 after the start of CsA, the MELD-Na score, an indicator of liver reserve and severity, improved rapidly, and all patients avoided liver transplantation and achieved long-term survival. This study represents a significant achievement in Japan, where a chronic shortage of donors is a particular challenge, and we intend to continue collaborating with the surgery transplant team to establish a treatment system with a high level of evidence and provide better care.
(Po-Sung Chu, Department of Internal Medicine (Gastroenterology), '86 equivalent)