BACKGROUND AND AIMS: Decompensated cirrhosis in autoimmune hepatitis has poor prognosis. Besides liver transplant, treatment for this entity is undefined. We explored the outcomes of autoimmune hepatitis (AIH)-related decompensated cirrhosis with active disease on treatment with steroids. METHODS: In this retrospective analysis, clinical data, laboratory parameters, and prognostic scores, such as baseline model for end-stage liver disease (MELD) scores, were compared among patients of AIH with decompensated cirrhosis with mild/no ascites (n = 38), gross ascites (n = 24), and compensated cirrhosis (n = 32) when administered steroids. The primary outcome was transplant-free survival at 12 months. Biochemical remission rates and other adverse events were also assessed and compared between these groups. RESULTS: Steroids were initiated at lower doses (25 mg/day-mild/no ascites, 20 mg/day-gross ascites) in patients with decompensated cirrhosis and at 40 mg/day in those with compensated cirrhosis. Transplant-free survival was 25.4%, 74.6%, and 96.9% (P = 0.001), and biochemical remission occurred in 5.1%, 49.0%, and 64.1% (P = 0.001) at 12 months in patients with gross ascites, mild/no ascites, and compensated cirrhosis, respectively. Infections were seen more frequently in decompensated cirrhosis, while other adverse events were comparable. Among decompensated cirrhosis, those with mild/no ascites had better prognostic scores, fewer posttreatment infections, and more frequent biochemical remission than those with gross ascites, achieving rates comparable to compensated cirrhosis. On multivariate analysis, the MELD score (subdistributional hazards ratio [sHR]; 95% confidence interval: 1.153 [1.07-1.24]; P = 0.001) and ascites (sHR: 2.556 [1.565-5.65]; P = 0.020) predicted survival. CONCLUSION: Type and severity of decompensation affect outcomes in patients with AIH-related cirrhosis. Those with mild/no ascites have comparable outcomes to those with compensated cirrhosis upon treatment with low-dose steroids.
BACKGROUND AND AIMS: Decompensated cirrhosis in autoimmune hepatitis has poor prognosis. Besides liver transplant, treatment for this entity is undefined. We explored the outcomes of autoimmune hepatitis (AIH)-related decompensated cirrhosis with active disease on treatment with steroids. METHODS: In this retrospective analysis, clinical data, laboratory parameters, and prognostic scores, such as baseline model for end-stage liver disease (MELD) scores, were compared among patients of AIH with decompensated cirrhosis with mild/no ascites (n = 38), gross ascites (n = 24), and compensated cirrhosis (n = 32) when administered steroids. The primary outcome was transplant-free survival at 12 months. Biochemical remission rates and other adverse events were also assessed and compared between these groups. RESULTS: Steroids were initiated at lower doses (25 mg/day-mild/no ascites, 20 mg/day-gross ascites) in patients with decompensated cirrhosis and at 40 mg/day in those with compensated cirrhosis. Transplant-free survival was 25.4%, 74.6%, and 96.9% (P = 0.001), and biochemical remission occurred in 5.1%, 49.0%, and 64.1% (P = 0.001) at 12 months in patients with gross ascites, mild/no ascites, and compensated cirrhosis, respectively. Infections were seen more frequently in decompensated cirrhosis, while other adverse events were comparable. Among decompensated cirrhosis, those with mild/no ascites had better prognostic scores, fewer posttreatment infections, and more frequent biochemical remission than those with gross ascites, achieving rates comparable to compensated cirrhosis. On multivariate analysis, the MELD score (subdistributional hazards ratio [sHR]; 95% confidence interval: 1.153 [1.07-1.24]; P = 0.001) and ascites (sHR: 2.556 [1.565-5.65]; P = 0.020) predicted survival. CONCLUSION: Type and severity of decompensation affect outcomes in patients with AIH-related cirrhosis. Those with mild/no ascites have comparable outcomes to those with compensated cirrhosis upon treatment with low-dose steroids.
Authors: Michael P Manns; Albert J Czaja; James D Gorham; Edward L Krawitt; Giorgina Mieli-Vergani; Diego Vergani; John M Vierling Journal: Hepatology Date: 2010-06 Impact factor: 17.425
Authors: Simon Pape; Tom J G Gevers; Michail Belias; Ilyas F Mustafajev; Jan Maarten Vrolijk; Bart van Hoek; Gerd Bouma; Carin M J van Nieuwkerk; Johannes Hartl; Christoph Schramm; Ansgar W Lohse; Richard Taubert; Elmar Jaeckel; Michael P Manns; Maria Papp; Felix Stickel; Michael A Heneghan; Joost P H Drenth Journal: Clin Gastroenterol Hepatol Date: 2019-01-06 Impact factor: 11.382
Authors: Floris F van den Brand; Koen S van der Veen; Ynto S de Boer; Nicole M van Gerven; Birgit I Lissenberg-Witte; Ulrich Beuers; Karel J van Erpecum; Henk R van Buuren; Jannie W den Ouden; Johannus T Brouwer; Jan M Vrolijk; Robert C Verdonk; Bart van Hoek; Ger H Koek; Joost P H Drenth; Marleen M J Guichelaar; Chris J J Mulder; Elisabeth Bloemena; Carin M J van Nieuwkerk; Gerd Bouma Journal: Clin Gastroenterol Hepatol Date: 2018-10-03 Impact factor: 11.382