TY - JOUR
T1 - Hepatic Encephalopathy and MELD-Na Predict Treatment Benefit in Autoimmune Hepatitis-related Decompensated Cirrhosis
AU - Arvaniti, Pinelopi
AU - Rodríguez-Tajes, Sergio
AU - Padilla, Marlene
AU - Olivas, Ignasi
AU - Mauro, Ezequiel
AU - el Maimouni, Cautar
AU - Lytvyak, Ellina
AU - Verhelst, Xavier
AU - Engel, Bastian
AU - Taubert, Richard
AU - Lorente-Pérez, Sara
AU - Conde, Isabel
AU - Riveiro-Barciela, Mar
AU - Ruiz-Cobo, Juan-Carlos
AU - Álvarez-Navascués, Carmen
AU - Salcedo, Magdalena
AU - Gómez, Judith
AU - Janik, Maciej K.
AU - Mateos, Beatriz
AU - Efe, Cumali
AU - Granito, Alessandro
AU - Dajti, Elton
AU - Azzaroli, Francesco
AU - Horta, Diana
AU - Vila, Carmen
AU - Castello, Inmaculada
AU - Pérez-Medrano, Indhira
AU - Arencibia, Ana
AU - Gerussi, Alessio
AU - Bruns, Tony
AU - Colaprieto, Francesca
AU - Lleo, Ana
AU - van den Ende, Natalie
AU - Verbeek, Jef
AU - Díaz-González, Álvaro
AU - Morillas, Rosa Ma
AU - Torner-Simó, Maria
AU - Bernal, Vanesa
AU - Fernández, Eva-Maria
AU - Gevers, Tom J. G.
AU - Terziroli Beretta-Piccoli, Benedetta
AU - Gómez, Elena
AU - Cuenca, Paqui
AU - de Boer, Ynte S.
AU - Kerkar, Nanda
AU - Assis, David N.
AU - Liberal, Rodrigo
AU - Drenth, Joost P. H.
AU - International Autoimmune Hepatitis Group (IAIHG)
AU - Tana, Michele M.
AU - European Reference Network on Hepatological Diseases (ENR RARE-LIVER)
AU - Sebode, Marcial
AU - Schregel, Ida
AU - Spanish Registry for Autoimmune and Cholestatic Diseases (ColHai) Registry
AU - Schramm, Christoph
AU - Lohse, Ansgar W.
AU - Montano-Loza, Aldo J.
AU - Zachou, Kalliopi
AU - Villamil, Alejandra
AU - Dalekos, George N.
AU - Londoño, María-Carlota
N1 - Publisher Copyright:
© 2025 AGA Institute
PY - 2025
Y1 - 2025
N2 - Background & Aims: Management of patients with autoimmune hepatitis (AIH)-related decompensated cirrhosis is challenging because of the risk of treatment-related complications and lack of clinical recommendations. We investigated the predictive factors for treatment benefit in AIH-related decompensated cirrhosis at diagnosis and developed an algorithm to guide treatment decisions in clinical practice. Methods: This retrospective, international, multicenter study included 232 patients with histologically confirmed AIH-related decompensated cirrhosis at diagnosis. The sub-hazard ratio (SHR) of mortality was determined by competing risk analysis, considering liver transplantation (LT) as competing event. A decision tree analysis was used to develop a treatment algorithm. Results: At diagnosis, 89% of patients had ascites, and 41% had overt hepatic encephalopathy (OHE). Treated patients (n = 214; 92%) had higher aminotransferases, bilirubin, and modified hepatic activity index. The SHR of mortality was lower in treated patients (0.438; 95% confidence interval [CI], 0.196–0.981; P = .045). Patients without OHE grade 3/4 and Model for End-Stage Liver Disease-Sodium (MELD-Na) ≤28 at diagnosis were more likely to benefit from treatment. In these patients, a decline in MELD-Na ≥11 after 4 weeks of treatment had a 100% negative predictive value for death/LT. Forty-nine percent of treated patients recompensated during follow-up. Twenty percent of patients had to discontinue treatment, 65% during the first 4 weeks, and only 4% due to infectious complications. OHE ≥grade 2 and MELD-Na at diagnosis predicted the need for treatment discontinuation. Conclusions: Immunosuppression is beneficial in patients with AIH-related decompensated cirrhosis and active disease. OHE and MELD-Na at diagnosis, along with a decline in MELD-Na at 4 weeks of treatment, are the most important determinants of outcome and can guide treatment decisions.
AB - Background & Aims: Management of patients with autoimmune hepatitis (AIH)-related decompensated cirrhosis is challenging because of the risk of treatment-related complications and lack of clinical recommendations. We investigated the predictive factors for treatment benefit in AIH-related decompensated cirrhosis at diagnosis and developed an algorithm to guide treatment decisions in clinical practice. Methods: This retrospective, international, multicenter study included 232 patients with histologically confirmed AIH-related decompensated cirrhosis at diagnosis. The sub-hazard ratio (SHR) of mortality was determined by competing risk analysis, considering liver transplantation (LT) as competing event. A decision tree analysis was used to develop a treatment algorithm. Results: At diagnosis, 89% of patients had ascites, and 41% had overt hepatic encephalopathy (OHE). Treated patients (n = 214; 92%) had higher aminotransferases, bilirubin, and modified hepatic activity index. The SHR of mortality was lower in treated patients (0.438; 95% confidence interval [CI], 0.196–0.981; P = .045). Patients without OHE grade 3/4 and Model for End-Stage Liver Disease-Sodium (MELD-Na) ≤28 at diagnosis were more likely to benefit from treatment. In these patients, a decline in MELD-Na ≥11 after 4 weeks of treatment had a 100% negative predictive value for death/LT. Forty-nine percent of treated patients recompensated during follow-up. Twenty percent of patients had to discontinue treatment, 65% during the first 4 weeks, and only 4% due to infectious complications. OHE ≥grade 2 and MELD-Na at diagnosis predicted the need for treatment discontinuation. Conclusions: Immunosuppression is beneficial in patients with AIH-related decompensated cirrhosis and active disease. OHE and MELD-Na at diagnosis, along with a decline in MELD-Na at 4 weeks of treatment, are the most important determinants of outcome and can guide treatment decisions.
KW - Autoimmune Hepatitis
KW - Decompensated Cirrhosis
KW - Liver Transplant-free Survival
KW - Recompensation
UR - https://www.scopus.com/pages/publications/105005504800
U2 - 10.1016/j.cgh.2025.02.010
DO - 10.1016/j.cgh.2025.02.010
M3 - Article
C2 - 40210079
SN - 1542-3565
JO - Clinical gastroenterology and hepatology
JF - Clinical gastroenterology and hepatology
ER -
SDG 3 Good Health and Well-being