Development and validation of a prognostic score for long‐term transplant‐free survival in autoimmune hepatitis type 1

Biewenga, Maaike; Verhelst, Xavier; Baven-Pronk, Martine; Putter, Hein; Berg, Aad P. van den; Nieuwkerk, Karin C M J van; Buuren, Henk R. van; Bouma, Gerd; Boer, Ynte S.; Simoen, Cedric; Colle, Isabelle; Schouten, Jeoffrey N.L.; Sermon, Filip; Steenkiste, Christophe Van; Vlierberghe, Hans Van; Meer, Adriaan J. van der; Nevens, Frederik; Hoek, Bart van

doi:10.1002/ueg2.12112

Cited by 10 publications

(10 citation statements)

References 38 publications

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“…In concordance with several studies [9][10][11][12]26,29,30] but with the exception of a few studies, [15][16][17] we found that cirrhosis at diagnosis adversely influences long-term outcomes. The finding in this report that cirrhosis is a risk factor for insufficient response is in line with earlier studies indicating that, in patients with AIH with cirrhosis, the complete biochemical response is more difficult to achieve, requiring more prolonged therapy with a higher risk of relapse.…”

Section: Discussionsupporting

confidence: 91%

Lack of complete biochemical response in autoimmune hepatitis leads to adverse outcome: First report of the IAIHG retrospective registry

Slooter,

van den Brand,

Lleo

et al. 2023

Hepatology

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Background and Aim: The International Autoimmune hepatitis (AIH) Group retrospective registry (IAIHG-RR) is a web-based platform with subjects enrolled with a clinical diagnosis of AIH. As prognostic factor studies with enough power are scarce, this study aimed to ascertain data quality and identify prognostic factors in the IAIHG-RR cohort. Methods: This retrospective, observational, multicenter study included all patients with a clinical diagnosis of AIH from the IAIHG-RR. Quality assessment consisted of external validation of completeness and consistency for 29 pre-defined variables. Cox regression was used to identify risk factors for liver-related death and liver transplantation (LT). Results: This analysis included 2559 patients across 7 countries. In 1700 patients, follow-up was available, with a completeness of individual data of 90% (range 30-100). During a median follow-up period of 10 (range 0-49) years, there were 229 deaths, of which 116 were liver-related and 143 patients underwent LT. Non-white ethnicity (HR 4.1 95% CI 2.3-7.1), cirrhosis (HR 3.5 95% CI 2.3-5.5), variant syndrome with primary sclerosing cholangitis (PSC) (HR 3.1 95% CI 1.6-6.2), and lack of complete biochemical response within 6 months (HR 5.7 95% CI 3.4-9.6) were independent prognostic factors. Conclusion: The IAIHG-RR represents the world’s largest AIH cohort with moderate-to-good data quality and a relevant number of liver-related events. The registry is a suitable platform for patient selection in future studies. Lack of complete biochemical response to treatment, non-white ethnicity, cirrhosis and PSC-AIH were associated with liver-related death and liver transplantation.

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Section: Discussionsupporting

confidence: 91%

Lack of complete biochemical response in autoimmune hepatitis leads to adverse outcome: First report of the IAIHG retrospective registry

Slooter,

van den Brand,

Lleo

et al. 2023

Hepatology

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“…First‐line medical therapy is based on corticosteroids (CST) and azathioprine (AZA) 1 and is efficient in the vast majority of patients. However, in the case of decompensated cirrhosis, hepatocellular carcinoma (HCC) or cortico‐resistant acute or sub‐acute liver failure, liver transplantation (LT) may be the only option; it is estimated that around 10% of patients suffering from AIH will require LT during their life 2–4 . LT for AIH (AIH‐LT) raises several specific issues in the early postoperative period.…”

Section: Introductionmentioning

confidence: 99%

“…However, in the case of decompensated cirrhosis, hepatocellular carcinoma (HCC) or cortico-resistant acute or sub-acute liver failure, liver transplantation (LT) may be the only option; it is estimated that around 10% of patients suffering from AIH will require LT during their life. [2][3][4] LT for AIH (AIH-LT) raises several specific issues in the early postoperative period. Notably, this population includes a large part of patients transplanted in the context of fulminant liver failure, which is known to be associated with a higher risk of early mortality.…”

Section: Introductionmentioning

confidence: 99%

Liver transplantation for autoimmune hepatitis: Pre‐transplant does not predict the early post‐transplant outcome

Chouik

Francoz

Martin

et al. 2023

Liver International

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Background and Aims: Autoimmune hepatitis (AIH) is a rare indication (<5%) for liver transplantation (LT). The aim of this study was to describe the early outcome after LT for AIH. Methods:A multicenter retrospective nationwide study including all patients aged ≥16 transplanted for AIH in France was conducted. Occurrences of biliary and vascular complications, rejection, sepsis, retransplantation and death were collected during the first year after LT.Results: A total of 344 patients (78.8% of women, 17.0% of (sub)fulminant hepatitis and 19.2% of chronic liver diseases transplanted in the context of acute-on-chronic liver failure [ACLF]) were included, with a median age at LT of 43.6 years. Acute rejection, sepsis, biliary and vascular complications occurred in respectively 23.5%, 44.2%, 25.3% and 17.4% of patients during the first year after LT. One-year graft and patient survivals were 84.3% and 88.0% respectively. The main cause of early death was sepsis. Pre-LT immunosuppression was not associated with an increased risk for early infections or surgical complications. Significant risk factors for septic events were LT

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“…The primary endpoint of this study was the normalisation of serum ALT and IgG levels after 24 weeks of treatment. With regard to the selection of compounds for clinical trials in AIH, there are two options: (1) the selection of repurposed compounds as monotherapy or as adjuncts for AIH17,72 ; and(2) the selection of novel immunomodulatory agents that specifically target dysregulated pathways in AIH, preferably individualised to the patient's immunological profile. To identify the best endpoints for clinical trials in AIH and simultaneously include the patient perspective are still challenging.…”

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confidence: 99%

Research gaps and opportunities in autoimmune hepatitis—Results of the international autoimmune hepatitis group research workshop 2022

Snijders

Sebode

et al. 2023

Liver International

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Autoimmune hepatitis (AIH) is a rare autoimmune liver disease that is characterised by a chronic inflammatory immune reaction directed against hepatocytes. The disease results in a substantial reduction in quality of life and potentially leads to liver-related complications or death. The International Autoimmune Hepatitis Group (IAIHG) initiated a series of research workshops to uncover the scientific gaps and opportunities in AIH. This review summarises the results of the latest workshop in Maastricht in 2022 and reviews the current challenges in adult AIH, particularly in relation to four important aspects of AIH: diagnostics; new immunomodulatory therapies; clinical trial design; and unmet clinical needs. This review also summarises the progress made since the AIH workshop in 2017. Patients and patient representatives were actively involved in the parallel working groups alongside clinicians and researchers. Despite 40 years of experience with diagnosing and treating AIH, false diagnoses occur and treatment is still based on nonselective immunosuppression. In addition to the need for more specific This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

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Development and validation of a prognostic score for long‐term transplant‐free survival in autoimmune hepatitis type 1

Cited by 10 publications

References 38 publications

Lack of complete biochemical response in autoimmune hepatitis leads to adverse outcome: First report of the IAIHG retrospective registry

Lack of complete biochemical response in autoimmune hepatitis leads to adverse outcome: First report of the IAIHG retrospective registry

Liver transplantation for autoimmune hepatitis: Pre‐transplant does not predict the early post‐transplant outcome

Research gaps and opportunities in autoimmune hepatitis—Results of the international autoimmune hepatitis group research workshop 2022

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