Clinical features of acute-onset autoimmune hepatitis: a systematic review and meta-analysis.

Abstract

Background and aims: Autoimmune Hepatitis (AIH), a rare chronic progressive inflammatory disease of the liver, and acute-onset AIH usually lack typical manifestations and have a poor prognosis. Hence, we have systematically examined all accessible data on the clinical features of acute-onset AIH to aid clinicians in deepening their understanding of the disease.

Methods: Eligible studies were gathered from a computer search of literature published from the inception of the database to June 2024 in PubMed and CNKI. Data on basic features, clinical manifestations, laboratory examinations, clinical outcomes, and pathological results were collected as relevant outcomes.

Results: Thirty-two retrospective studies encompassing 2,561 patients, and four cohort studies involving 419 patients were ultimately included. Approximately 31% of AIH patients present with acute disease onset, with 58% being acute non-severe, 25% acute severe, and 17% commencing with fulminant liver failure. Patients with acute-onset AIH tend to be younger (OR = 0.25, 95% CI: -0.449 to -0.057) and exhibit a diminishing female predilection that increases with severity compared to the more chronic classic AIH (OR = 0.65, 95% CI: 0.569‒0.727). The presence of comorbid extrahepatic autoimmune disease showed a potential protective effect when compared with C-AIH (OR = 0.63, 95% CI: 0.455‒0.874).

Conclusions: About 30% of patients with AIH exhibit an acute disease onset, understanding its proportion and clinical features aids clinicians in making timely diagnoses to prevent severe consequences. However, in existing studies, the results of laboratory tests and histologic examinations of A-AIH are influenced by AC-AIH to varying degrees, which somewhat restricts the clinicians' understanding.