Abstract Objectives Paediatric acute liver failure (PALF) and hepatitis of indeterminate aetiology with distinctive CD8+ T‐cell rich liver infiltrates is a recently recognised disorder of immune‐mediated dysregulation. We aimed to characterise the clinical phenotype, liver immunohistochemistry, response to immunosuppression (IS) therapy and clinical outcomes up to 24‐months in an Australian cohort. Methods Single‐centre retrospective study of patients aged 1–18 years who presented between 2017 and 2022 with hepatitis or acute liver failure with CD8+ rich infiltrates on liver biopsy. Results Of the 13 patients identified, 8/13 had CD8+ PALF and 5/13 had CD8+ hepatitis. Median age at presentation was 10.8 years (interquartile range IQR 7.8–12.3), and 85% were male. Peripheral lymphopenia was noted in 85% of patients. Corticosteroids (CS) were commenced in 10/13 (77%) patients at a median of 13 days post‐illness‐onset (IQR 9–14). Maintenance IS was initiated in 7/10 (70%) patients, at a median of 20 days post‐illness‐onset (IQR 19–37), with tacrolimus commenced in 4/7 (57%) patients. At 24‐month follow‐up, 100% of patients were alive, 9/13 (69%) survived with native liver (4/8 with PALF; 5/5 with hepatitis), 4/8 (50%) with PALF required liver transplantation and 2/13 (15%) developed aplastic anaemia and recovered without haematopoietic stem cell transplantation. Successful withdrawal of IS was achieved in 50% patients with their native liver. Conclusions Evaluation of liver immunohistochemistry and peripheral T‐cell subset analysis is essential for identification of patients with PALF and hepatitis with CD8+ T‐cell predominant liver injury. Early CS and adjunct IS are safe and effective in achieving favourable outcomes at 24 months.
Diksha et al. (Mon,) studied this question.