Liver and Bile
Hepatology. 2024;79(3):538–50
Lack of complete biochemical response in autoimmune hepatitis leads to adverse outcome: First report of the IAIHG retrospective registry
Background and aims: The International Autoimmune Hepatitis Group retrospective registry (IAIHG-RR) is a web-based platform with subjects enrolled with a clinical diagnosis of autoimmune hepatitis (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. The quality assessment consisted of external validation of completeness and consistency for 29 predefined 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.
Conclusions: The International Autoimmune Hepatitis Group retrospective registry (IAIHG-RR) represents the world's largest autoimmune hepatitis (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 primary sclerosing cholangitis-AIH were associated with liver-related death and liver transplantation.