In this report, we describe the case of a 28-yr-old woman with sickle cell anemia who presented with acute hepatic failure manifested by anorexia, malaise, painless jaundice, elevated aminotransferase activities, and severe coagulopathy. Liver biopsy revealed changes consistent with autoimmune hepatitis. Treatment with corticosteroids and azathioprine was followed by improvement in biochemical liver test results. The literature on sickle cell-associated liver diseases is reviewed.
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Cureus
December 2024
Internal Medicine, Tobago Regional Health Authority, Scarborough, TTO.
Autoimmune hepatitis (AIH) is rare, and the diagnosis is not always clear-cut. It is associated with increased morbidity and mortality for the pregnant mother and fetus. Frequent pregnancy testing of women in childbearing years who have a known diagnosis of AIH may be necessary, even after counseling on family planning takes place.
View Article and Find Full Text PDFImmunity
January 2025
Garvan Institute of Medical Research, Darlinghurst, NSW, Australia; St Vincent's Clinical School, UNSW Sydney, Sydney, NSW, Australia. Electronic address:
The unexplained association between infection and autoimmune disease is strongest for hepatitis C virus-induced cryoglobulinemic vasculitis (HCV-cryovas). To analyze its origins, we traced the evolution of pathogenic rheumatoid factor (RF) autoantibodies in four HCV-cryovas patients by deep single-cell multi-omic analysis, revealing three sources of B cell somatic mutation converged to drive the accumulation of a large disease-causing clone. A method for quantifying low-affinity binding revealed recurring antibody variable domain combinations created by V(D)J recombination that bound self-immunoglobulin G (IgG) but not viral E2 antigen.
View Article and Find Full Text PDFLiver Int
February 2025
Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Background And Aim: Discriminating between idiosyncratic drug-induced liver injury (DILI) and autoimmune hepatitis (AIH) is critical yet challenging. We aim to develop and validate a machine learning (ML)-based model to aid in this differentiation.
Methods: This multicenter cohort study utilised a development set from Beijing Friendship Hospital, with retrospective and prospective validation sets from 10 tertiary hospitals across various regions of China spanning January 2009 to May 2023.
J Clin Invest
January 2025
Institute for Research in Biomedicine (IRB), Bellinzona, Switzerland.
Autoimmune hepatitis (AIH) is a rare chronic inflammatory liver disease characterized by the presence of autoantibodies, including those targeting O-phosphoseryl-tRNA:selenocysteine-tRNA synthase (SepSecS), also known as soluble liver antigen (SLA). Anti-SepSecS antibodies have been associated with a more severe phenotype, suggesting a key role for the SepSecS autoantigen in AIH. To analyze the immune response to SepSecS in patients with AIH at the clonal level, we combined sensitive high-throughput screening assays with the isolation of monoclonal antibodies (mAbs) and T cell clones.
View Article and Find Full Text PDFJ Clin Invest
January 2025
How are autoreactive T cells induced and regulated in patients with autoimmune disease? This question lies at the core of understanding autoimmune disease pathologies, yet it has remained elusive due to host variability and the complexity of the immune system. In this issue of the JCI, Kramer and colleagues used autoimmune hepatitis (AIH) as a model to explore the maintenance of autoreactive CD4+ T cells specific to O-phosphoseryl-tRNA:selenocysteine tRNA synthase (SepSecS). The findings provide insight into the interaction between T cells and B cells in AIH pathogenesis that may reflect a shared mechanism among other autoimmune diseases.
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