{
  "abstract": "Background: 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.\n\nCase presentation: 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.\n\nConclusion: 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.",
  "abstract_shown": true,
  "affiliations": [
    "Department of Rheumatology and Immunology, Peking University International Hospital",
    "Department of Pathology, Peking University International Hospital",
    "Department of Pathology, China-Japan Friendship Hospital",
    "Department of Infectious Diseases, Peking University International Hospital"
  ],
  "article_type": null,
  "arxiv": null,
  "author_total": 6,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Lina Zhang"
    },
    {
      "affs": [
        1
      ],
      "name": "Tong Zhang"
    },
    {
      "affs": [
        0
      ],
      "name": "Jing Zhang"
    },
    {
      "affs": [
        2
      ],
      "name": "Xiuhong Wang"
    },
    {
      "affs": [
        3
      ],
      "name": "Yihui Rong"
    },
    {
      "affs": [
        0
      ],
      "name": "Sheng-Guang Li"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/8eyke6dj346bg.bib",
  "cited_by": 0,
  "doi": "10.3389/fimmu.2026.1915385",
  "fulltext_url": "https://europepmc.org/articles/PMC13680148",
  "id": "8eyke6dj346bg",
  "issns": [
    "1664-3224"
  ],
  "landing_url": "https://www.frontiersin.org/articles/10.3389/fimmu.2026.1915385/full",
  "language": "eng",
  "licence": "CC-BY-4.0",
  "licence_label": "CC BY 4.0",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "openalex",
      "2721|Liver Diseases and Immunity"
    ]
  ],
  "openalex": "W7220695878",
  "pdf_url": "https://europepmc.org/articles/PMC13680148?pdf=render",
  "pmcid": "PMC13680148",
  "pmid": "42903101",
  "published": "2026-10-06",
  "publisher": "Frontiers Media SA",
  "ris": "https://research.draconitic.com/paper/8eyke6dj346bg.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:19:15Z",
      "native_id": "10.3389/fimmu.2026.1915385",
      "source": "crossref",
      "url": "https://www.frontiersin.org/articles/10.3389/fimmu.2026.1915385/full"
    },
    {
      "first_seen": "2026-10-30T12:19:57Z",
      "native_id": "MED:42903101",
      "source": "europepmc",
      "url": "https://europepmc.org/article/MED/42903101"
    }
  ],
  "subjects": [
    {
      "field": "biology",
      "origin": "issn:1664-3224",
      "subfield": "immunology"
    }
  ],
  "title": "Case Report: Acute liver failure in systemic lupus erythematosus with autoimmune hepatitis–primary biliary cholangitis overlap: a belimumab-containing regimen and longitudinal follow-up",
  "title_text": "Case Report: Acute liver failure in systemic lupus erythematosus with autoimmune hepatitis–primary biliary cholangitis overlap: a belimumab-containing regimen and longitudinal follow-up",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T12:19:57Z",
  "url": "https://research.draconitic.com/paper/8eyke6dj346bg",
  "venue": "Frontiers in Immunology",
  "versions": []
}