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

• Published in: Hepatology (Baltimore, Md.) Vol. 79; no. 3; pp. 538 - 550
• Main Authors: Slooter, Charlotte D., van den Brand, Floris F., Lleo, Ana, Colapietro, Francesca, Lenzi, Marco, Muratori, Paolo, Kerkar, Nanda, Dalekos, George N., Zachou, Kalliopi, Lucena, M. Isabel, Robles-Díaz, Mercedes, Di Zeo-Sánchez, Daniel E., Andrade, Raúl J., Montano-Loza, Aldo J., Lytvyak, Ellina, Lissenberg-Witte, Birgit I., Maisonneuve, Patrick, Bouma, Gerd, Macedo, Guilherme, Liberal, Rodrigo, de Boer, Ynto S.
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 01.03.2024
• Subjects: Cholangitis, Sclerosing - complications; Hepatitis, Autoimmune - diagnosis; Humans; Liver Cirrhosis - complications; Liver Transplantation; Pathologic Complete Response; Retrospective Studies
• ISSN: 0270-9139; 1527-3350; 1527-3350
• DOI: 10.1097/HEP.0000000000000589

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 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 LT.