Liver failure as initial presentation of autoimmune hepatitis: Clinical characteristics, predictors of response to steroid therapy, and outcomes

Abstract

In a recent study, we had reported the impact of ethnicity on the natural history of autoimmune hepatitis (AIH).1 We did a further subgroup analysis of those with initial presentation with liver failure (LF), defined as either international normalized ratio (INR) ≥ 2 and or any degree of hepatic encephalopathy. Twenty patients (20/101, 19.8%) presented with LF, and they were more likely to be black (70% versus 7.4%, P = 0.001), require referral for liver transplantation (LT) (70% versus 24.6%, P < 0.0001), and have a higher mortality (45% versus 4.9%, P < 0.0001), compared to those without LF. Of the 14 (70%) patients with LF who were treated medically with steroids, seven (50%) responded (Table 1). Responders had a Model for End-Stage Liver Disease (MELD) score at admission ≤ 28 and were more likely to have cirrhosis (71.1% versus 28.5%); they showed a stable trend in serum bilirubin (mg/dL) (15.2 ± 13.2 versus 14.1 ± 14.1) and INR (1.8 ± 0.4 versus 1.8 ± 0.5) after a mean of 3.7 ± 0.6 days of steroid therapy. In contrast, nonresponders showed a worsening of serum bilirubin (22.5 ± 4.2 versus 23.1 ± 5.6) and INR (3.0 ± 1.5 versus 3.6 ± 2.1). All responders survived without need for a LT, whereas all nonresponders had a fatal outcome (of whom one died after LT). Six patients (30%) with LF were not treated medically, of whom five had submassive necrosis on liver biopsy. Five of these six patients underwent transplantation and all but one survived. One could not undergo transplantation due to development of fatal cholangitis. In conclusion, about 20% of patients with AIH have LF at initial presentation. A lower MELD score at admission (≤ 28), more severe hepatic fibrosis, and an early (within 4 days of steroid therapy) improvement or stabilization in serum bilirubin and INR identify those who are likely to respond to steroid therapy, and thus survive without LT. Our observations need further corroboration, but if confirmed, such a stratification strategy could optimize care of patients with AIH who present with LF. Sumita Verma*, Anurag Maheshwari , Paul Thuluvath , * Gastroenterology/Hepatology, Brighton and Sussex Medical School, University of Sussex, Falmer, Brighton, UK, Gastroenterology and Hepatology, Johns Hopkins University Hospitals, Baltimore, MD, Mercy Medical Center, Institute for Digestive Health and Liver Diseases, Baltimore, MD.