Hepatic encephalopathy and MELD-Na predict treatment benefit in autoimmune hepatitis-related decompensated cirrhosis

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% 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%CI 0.196-0.981, p=0.045). Patients without OHE grade 3/4 and 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.