Prompt initiation of high-dose i.v. corticosteroids seems to prevent progression to liver failure in patients with original acute severe autoimmune hepatitis
Abstract
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.
