Aims: The definition of original acute severe autoimmune hepatitis (AS-AIH) is unclear. However, its rapid recognition and early treatment is potentially life-saving. Therefore, we present herein an open, real-world observational study for the assessment of the efficacy and safety of early high-dose i.v. corticosteroids in original AS-AIH patients.Methods: Prospectively collected data from 184 AIH patients were analyzed retrospectively. Original AS-AIH defined as an acute symptomatic presentation of newly diagnosed AIH (transaminases >10× upper limit of normal, bilirubin >4 mg/dL, and international normalized ratio [INR] ≥1.5) without histological lesions of chronic disease.Results: Thirty-four of 184 (18.5%) patients had original AS-AIH.These patients were promptly treated with i.v. corticosteroids (either 1 g methylprednisolone for 3 consecutive days followed by i.v. 1 mg/kg/day prednisolone or i.v. 1.5 mg/kg/day prednisolone from the beginning). Only 1/34 (2.9%) died due to sepsis; none required liver transplantation during follow-up (65 [1-175] months). No significant differences were detected in baseline characteristics between original AS-AIH patients and those with insidious presentation (not-AS-AIH; n = 117) apart from antinuclear antibodies negativity (P = 0.038), and higher immunoglobulin G, transaminases, INR, and bilirubin in original AS-AIH patients (P = 0.001 for all). Complete response and corticosteroids withdrawal (for patients treated >12 months) were significantly more frequent in original AS-AIH (n = 28) than in not-AS-AIH (n = 79; P = 0.026 and P = 0.016, respectively). Presence of original AS-AIH was the only independent predictor for achieving complete response.Conclusions: Prompt initiation of high-dose i.v. corticosteroids in original AS-AIH seems safe and efficient as it prevents disease deterioration and the need for liver transplantation. The long-term overall survival of these patients was high (97% for 5.3 years), and the long-term treatment response and corticosteroids withdrawal rates were higher compared to not-AS-AIH patients.

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BACKGROUND & AIMS:Patients usually receive a diagnosis of primary biliary cholangitis (PBC) at an early stage, based on biochemical analyses. We investigated the proportion of patients who progress to moderate or advanced PBC and factors associated with progression and patient survival. METHODS:We obtained data from 1615 patients (mean age, 55.4 y) with early stage PBC (based on their normal levels of albumin and bilirubin), collected at the time of initial evaluation or treatment, from the Global PBC Study Group database (comprising patients at 19 liver centers in North American and European countries). We collected data from health care evaluations on progression to moderate PBC (abnormal level of bilirubin or albumin) or advanced-stage PBC (abnormal level of both). The median follow-up time was 7.9 years. The composite end point was decompensation, hepatocellular carcinoma, liver transplantation, or death. RESULTS:Of the 1615 patients identified with early stage PBC, 904 developed moderate PBC and 201 developed advanced disease over the study period. The proportions of patients who transitioned to moderate PBC at 1, 3, and 5 years were 12.9%, 30.2%, and 45.8%. The a Authors share co-first authorship.