BACKGROUND AND AIMS: Autoimmune hepatitis presenting as acute on chronic liver failure (AIH-ACLF) is a novel entity with limited data on clinical course and management. We assessed outcomes in patients of AIH-ACLF with no extrahepatic organ dysfunction/failure when administered steroids. METHODS: In this retrospective analysis, clinical data, laboratory parameters, liver biopsy indices and prognostic scores such as model for end-stage liver disease (MELD) and Child-Turcotte-Pugh (CTP) scores at baseline were computed for patients with AIH-ACLF and compared across strata of incident infections and transplant-free survival. The primary outcome was 90-day transplant-free survival. Biochemical remission was assessed, and predictors of end points were identified. RESULTS: Twenty-nine patients of AIH-ACLF were included with a median follow-up of 4 months. The 90- and 180-day transplant-free survival rates of 55.2 [95% confidence interval (CI): 39.7-76.6]% and 30.2(95% CI: 16.7-54.6)%, respectively, were attained on steroids. Three patients (10.3%) underwent liver transplant while 16 (55.2%) deaths occurred. Infections developed in 12 patients (41.3%), leading to worsening prognostic scores, new onset organ dysfunction/failure and 11 deaths. Seven of ten patients (70%) in the transplant-free survivor group attained biochemical remission on follow-up. The MELD score<24 (sensitivity: 68.4%; specificity: 80%) and CTP<11 (sensitivity: 78.9%; specificity: 90%) had best predictive value for survival, in addition to decrease in the MELD score at 2 weeks (sensitivity: 78.9%; specificity: 70%). CONCLUSION: Patients with AIH-ACLF have a morbid disease course despite treatment with steroids. Patients with no extrahepatic organ failure with good baseline prognostic scores may be administered steroids with close monitoring for change in MELD over 2 weeks.
BACKGROUND AND AIMS: Autoimmune hepatitis presenting as acute on chronic liver failure (AIH-ACLF) is a novel entity with limited data on clinical course and management. We assessed outcomes in patients of AIH-ACLF with no extrahepatic organ dysfunction/failure when administered steroids. METHODS: In this retrospective analysis, clinical data, laboratory parameters, liver biopsy indices and prognostic scores such as model for end-stage liver disease (MELD) and Child-Turcotte-Pugh (CTP) scores at baseline were computed for patients with AIH-ACLF and compared across strata of incident infections and transplant-free survival. The primary outcome was 90-day transplant-free survival. Biochemical remission was assessed, and predictors of end points were identified. RESULTS: Twenty-nine patients of AIH-ACLF were included with a median follow-up of 4 months. The 90- and 180-day transplant-free survival rates of 55.2 [95% confidence interval (CI): 39.7-76.6]% and 30.2(95% CI: 16.7-54.6)%, respectively, were attained on steroids. Three patients (10.3%) underwent liver transplant while 16 (55.2%) deaths occurred. Infections developed in 12 patients (41.3%), leading to worsening prognostic scores, new onset organ dysfunction/failure and 11 deaths. Seven of ten patients (70%) in the transplant-free survivor group attained biochemical remission on follow-up. The MELD score<24 (sensitivity: 68.4%; specificity: 80%) and CTP<11 (sensitivity: 78.9%; specificity: 90%) had best predictive value for survival, in addition to decrease in the MELD score at 2 weeks (sensitivity: 78.9%; specificity: 70%). CONCLUSION: Patients with AIH-ACLF have a morbid disease course despite treatment with steroids. Patients with no extrahepatic organ failure with good baseline prognostic scores may be administered steroids with close monitoring for change in MELD over 2 weeks.
Authors: R Todd Stravitz; Jay H Lefkowitch; Robert J Fontana; M Eric Gershwin; Patrick S C Leung; Richard K Sterling; Michael P Manns; Gary L Norman; William M Lee Journal: Hepatology Date: 2011-01-05 Impact factor: 17.425
Authors: Javier Fernández; Joan Clària; Alex Amorós; Ferrán Aguilar; Miriam Castro; Mireia Casulleras; Juan Acevedo; Marta Duran-Güell; Laura Nuñez; Montserrat Costa; Mireia Torres; Raquel Horrillo; Luis Ruiz-Del-Árbol; Cándido Villanueva; Verónica Prado; Mireya Arteaga; Jonel Trebicka; Paolo Angeli; Manuela Merli; Carlo Alessandria; Niels Kristian Aagaard; German Soriano; François Durand; Alexander Gerbes; Thierry Gustot; Tania M Welzel; Francesco Salerno; Rafael Bañares; Victor Vargas; Agustin Albillos; Aníbal Silva; Manuel Morales-Ruiz; Juan Carlos García-Pagán; Marco Pavesi; Rajiv Jalan; Mauro Bernardi; Richard Moreau; Antonio Páez; Vicente Arroyo Journal: Gastroenterology Date: 2019-03-22 Impact factor: 22.682
Authors: Rajiv Jalan; Faouzi Saliba; Marco Pavesi; Alex Amoros; Richard Moreau; Pere Ginès; Eric Levesque; Francois Durand; Paolo Angeli; Paolo Caraceni; Corinna Hopf; Carlo Alessandria; Ezequiel Rodriguez; Pablo Solis-Muñoz; Wim Laleman; Jonel Trebicka; Stefan Zeuzem; Thierry Gustot; Rajeshwar Mookerjee; Laure Elkrief; German Soriano; Joan Cordoba; Filippo Morando; Alexander Gerbes; Banwari Agarwal; Didier Samuel; Mauro Bernardi; Vicente Arroyo Journal: J Hepatol Date: 2014-06-17 Impact factor: 25.083
Authors: Sung-Eun Kim; Ji Won Park; Hyung Su Kim; Myoung-Kuk Jang; Ki Tae Suk; Dong Joon Kim Journal: Int J Mol Sci Date: 2021-10-28 Impact factor: 5.923