Case Report: Acute liver failure in systemic lupus erythematosus with autoimmune hepatitis-primary biliary cholangitis overlap: a belimumab-containing regimen and longitudinal follow-up.

Authors

Lina Zhang, Tong Zhang, Jing Zhang, Xiuhong Wang, Yihui Rong, Sheng-Guang Li

Published in

Frontiers in immunology. Volume 17. Pages 1915385. Epub Oct 06, 2026.

Abstract

Severe autoimmune liver injury as the first manifestation of systemic lupus erythematosus (SLE) is rare, and concomitant autoimmune hepatitis-primary biliary cholangitis (AIH-PBC) overlap is uncommon. B-cell-activating factor (BAFF) provides a biological rationale for adjunctive therapy, although clinical evidence remains limited.
A 72-year-old woman presented with 4 weeks of fatigue and anorexia and developed coagulopathy (international normalized ratio [INR] 1.8), ascites, and hepatic encephalopathy. Autoimmune testing showed antinuclear antibody 1:1280, high-titer anti-double-stranded DNA antibodies with hypocomplementemia, immunoglobulin G (IgG) 30.0 g/L, and antimitochondrial antibody positivity. Hepatitis B surface antigen, hepatitis C antibody, hepatitis E antibody, and Epstein-Barr virus and cytomegalovirus DNA tests were negative. Liver biopsy 42 days after corticosteroid initiation demonstrated interface hepatitis, destructive small-bile-duct injury, plasma cell-rich inflammation, and Batts-Ludwig stage 2 fibrosis without cirrhosis, supporting a clinicopathologic diagnosis of AIH-PBC overlap. Previously unrecognized chronic autoimmune liver disease could not be excluded. Prednisolone was started at 50 mg/day, followed 10 days later by intravenous belimumab, with concomitant ursodeoxycholic acid. Tacrolimus was added during maintenance. By 10 October 2025, liver biochemistry had improved, IgG was 9.42 g/L, and prednisolone had been reduced to 2.5 mg/day. Liver stiffness decreased from 21.5 to 9.7 kPa.
Sustained biochemical improvement occurred during a belimumab-containing multidrug regimen. This observation does not establish the independent efficacy of belimumab or histological regression of fibrosis.

PMID:
42903101
Bibliographic data and abstract were imported from PubMed on 22 Oct 2026.

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