Review article: unanswered clinical and research questions in autoimmune hepatitis‐conclusions of the International Autoimmune Hepatitis Group Research Workshop

Dyson, Jessica; Martin, Eléonora De; Dalekos, Georgios N.; Drenth, Joost P.H.; Herkel, Johannes; Hübscher, Stefan G.; Kelly, Déirdre; Lenzi, Marco; Milkiewicz, Piotr; Oo, Ye Htun; Heneghan, Michael A.; Lohse, Ansgar W.

doi:10.1111/apt.15111

Cited by 35 publications

(24 citation statements)

References 94 publications

(91 reference statements)

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“…Hepatitis Study Group emphasised that concerns regarding corticosteroid-related adverse events are ongoing. 28 The current study shows that even low-dose strategies frequently lead to substantial side effects are at odds with the assumption that adverse events are prevented by low-dose strategies. Yet, the finding that this effect mitigates after discontinuing predniso(lo)ne is reassuring.…”

Section: Discussionmentioning

confidence: 73%

“…With the introduction of azathioprine in the treatment of autoimmune hepatitis, both doses and corticosteroid‐related adverse events decreased . However, recently the International Autoimmune Hepatitis Study Group emphasised that concerns regarding corticosteroid‐related adverse events are ongoing . The current study shows that even low‐dose strategies frequently lead to substantial side effects are at odds with the assumption that adverse events are prevented by low‐dose strategies.…”

Section: Discussionmentioning

confidence: 76%

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Adverse events related to low dose corticosteroids in autoimmune hepatitis

Brand

Veen

Lissenberg‐Witte

et al. 2019

Aliment Pharmacol Ther

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Summary Background Autoimmune hepatitis requires long‐term therapy, and systemic corticosteroids are the backbone of therapeutic management. Prolonged use of corticosteroids may lead to adverse events but data from long‐term studies are mainly derived from studies in rheumatic diseases. Aim To assess cataract, diabetes and fractures in relation to corticosteroid doses in the long‐term maintenance treatment of patients with autoimmune hepatitis. Methods We retrospectively collected data on 476 patients (77% women) with an established diagnosis of autoimmune hepatitis. Binary logistic regression with a generalised estimating equation was used to analyse the association between current corticosteroid use and the incidence of cataract, diabetes and fractures with onset after autoimmune hepatitis diagnosis. We corrected for sex, age, cirrhosis at diagnosis and predniso(lo)ne use in the prior 3 years to account for possible ongoing effects. Results A total of 6634 years, with a median of 13 (range 1‐40) per patient were recorded. The median age at diagnosis was 44 years (range 2‐88). Adverse events were documented in 120 (25%) patients. Low‐dose predniso(lo)ne (0.1‐5.0 mg/d) increased the odds of fractures whereas higher doses (>5.0 mg/d) increased the odds of cataracts and diabetes. Budesonide increased the odds of cataract and fractures; this effect was independent of predniso(lo)ne use in the prior 1, 2 or 3 years. Conclusions Even low doses of corticosteroids frequently lead to substantial adverse events refuting the assumption that adverse events are prevented by administering low doses.

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Section: Discussionmentioning

confidence: 73%

Section: Discussionmentioning

confidence: 76%

Adverse events related to low dose corticosteroids in autoimmune hepatitis

Brand

Veen

Lissenberg‐Witte

et al. 2019

Aliment Pharmacol Ther

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“…The cross-sectional study performed in the UK adopted a criterion for the issue as more than mild steatosis regardless of hepatocyte ballooning [27]. A past report presented evidence that baseline liver status was more frequently cirrhotic, and that patient survival was worse in AIH with NASH than pure AIH [33]. In the current study, hepatic steatosis equal to or more than 5% accompanied by hepatocyte ballooning was de ned as NASH [17].…”

Section: Discussionmentioning

confidence: 80%

Prognosis of Probable Autoimmune Hepatitis Patients in a Retrospective Cohort Study

Fujita

Oura

Tadokoro

et al. 2020

Preprint

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Background: Autoimmune hepatitis (AIH) is an idiopathic inflammatory liver disease with genetic susceptibility and unknown environmental triggers, which results in failures of physiologic immunotolerance and destruction of the liver tissues. The gold standard for diagnosis is the International Autoimmune Hepatitis Group (IAIHG) scoring system: the disease is classified as definite or probable according to the scores. However, conventional research on probable AIH has focused on the Caucasian population and there is little data pertaining to the Asian population. Therefore, this study aimed to assess and compare the prognosis of Japanese patients with probable and definite AIH. Methods: Patients with probable and definite AIH diagnosed based on IAIHG scores between 1987 and 2018 were enrolled in this retrospective study. Results: Seventy-two patients with definite AIH and 49 patients with probable AIH were evaluated in the study. Univariate analysis revealed age, fibrosis stage 4, and the fibrosis-4 index were prognostic factors for overall survival. Multivariate analysis indicated that age and liver cirrhosis significantly affected the overall survival. When the cut off albumin-bilirubin score was set appropriately, cirrhosis was differentially diagnosed using albumin-bilirubin score with 100% sensitivity and 70.5% specificity. Classification of probable or definite disease did not alter overall survival with statistical significance. Conclusions: Our findings suggest that probable AIH should be managed as definite AIH is managed in Japanese population. The albumin-bilirubin score helps identify liver cirrhosis and is a prognostic biomarker for overall survival.

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“…Regardless, >50% of patients often do not achieve CR in the long-term by conventional therapy. 1 , 7 However, second- and third-line therapies for AIH are not well established and are mainly based on centre experience and small series of patients. 1 , 7 The major point arising from our case studies is that belimumab could be a promising option for difficult-to-treat patients, such as those with refractory AIH and advanced liver fibrosis, as seen in our case studies.…”

Section: Discussionmentioning

confidence: 99%

“…For these patients, alternative third-line treatment regimens have not yet been established and, thus, are urgently warranted. 7 …”

Section: Introductionmentioning

confidence: 99%

Belimumab is a promising third-line treatment option for refractory autoimmune hepatitis

et al. 2020

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Background & Aims 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. Methods 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. Results 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. Conclusions These preliminary findings indicate belimumab as a promising treatment option for patients with AIH and refractory and advanced liver-related fibrosis. Lay summary 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.

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Review article: unanswered clinical and research questions in autoimmune hepatitis‐conclusions of the International Autoimmune Hepatitis Group Research Workshop

Cited by 35 publications

References 94 publications

Adverse events related to low dose corticosteroids in autoimmune hepatitis

Adverse events related to low dose corticosteroids in autoimmune hepatitis

Prognosis of Probable Autoimmune Hepatitis Patients in a Retrospective Cohort Study

Belimumab is a promising third-line treatment option for refractory autoimmune hepatitis

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