%0 Journal Article
%T Belimumab is a promising third-line treatment option for refractory autoimmune hepatitis.
%A Arvaniti P
%A Giannoulis G
%A Gabeta S
%A Zachou K
%A Koukoulis GK
%A Dalekos GN
%J JHEP Rep
%V 2
%N 4
%D Aug 2020
%M 32671332
%F 9.917
%R 10.1016/j.jhepr.2020.100123
%X <B>UNASSIGNED: </B>Autoimmune hepatitis (AIH) is a disease of unknown aetiology with a favourable response to immunosuppression. However, in the clinic, it appears that <50% of patients achieve complete response on standard treatment. Serum B cell-activating factor (BAFF) levels are elevated in patients with AIH and are likely to contribute to disease pathogenesis. Given that belimumab, a BAFF inhibitor, has been shown to be effective in other autoimmune diseases, we investigated its use as a third-line add-on treatment option in patients with advanced AIH who did not respond to conventional treatment.<BR><B>UNASSIGNED: </B>Herein, we report for the first time two patients, a 27-year-old female and a 58-year-old male, both with AIH-related compensated cirrhosis at diagnosis, who were refractory to standard immunosuppressive therapies and received add-on third-line therapy with belimumab.<BR><B>UNASSIGNED: </B>Both patients achieved a complete response and remained in remission while receiving low-dose corticosteroids. No adverse events related to belimumab and/or disease decompensation were observed.<BR><B>UNASSIGNED: </B>These preliminary findings indicate belimumab as a promising treatment option for patients with AIH and refractory and advanced liver-related fibrosis.<BR><B>UNASSIGNED: </B>A small proportion of patients with autoimmune hepatitis (AIH) are refractory to standard treatments; these patients bear the highest probability of developing decompensated cirrhosis and hepatocellular carcinoma because third-line treatment options are not well established. In this case study, we showed that third-line add-on therapy with belimumab, a B cell-activating factor inhibitor, could be an alternative and promising treatment option in patients with advanced AIH who did not respond to conventional treatment.