Clonal analysis of SepSecS-specific B and T cells in autoimmunehepatitis. Autoimmunehepatitis (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
Elucidating the role of autoreactive T cells and B cells in autoimmunehepatitis. 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 autoimmunehepatitis (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.
Update in Clinical Science: AutoimmuneHepatitis. Autoimmunehepatitis (AIH) is an enigmatic, relatively rare disease with a variable spectrum of presentation whose pathogenesis, diagnosis and management remain a major challenge. Methods. We have performed a review incorporating recent developments in basic science, epidemiology, clinical science, therapeutics, regulatory science and evaluated
Ethyl acetate extract from Herpetospermun cardigerum Wall. Ameliorated Concanavalin A-induced autoimmunehepatitis in mice by reprofiling gut microenvironment to modulate IDO1/KYN and PI3K/AKT/NF-κB pathways. Autoimmunehepatitis (AIH) is an immunoinflammatory chronic liver disease with increasing prevalence worldwidely, lacking of effective medicine. Herpetospermum caudigerum Wall. (HC
Health-related quality of life is impaired in people with autoimmunehepatitis: Results of a multicentre cross-sectional study within the European Reference Network. Impaired health-related quality of life (HRQoL) contributes to the overall disease burden in autoimmunehepatitis (AIH). This study aimed to evaluate HRQoL in people with AIH and to identify potentially modifiable factors associated
British Society of Gastroenterology guidelines for diagnosis and management of autoimmunehepatitis. Autoimmunehepatitis (AIH) is a chronic inflammatory liver disease which, if untreated, often leads to cirrhosis, liver failure and death. The last British Society of Gastroenterology (BSG) guideline for the management of AIH was published in 2011. Since then, our understanding of AIH has advanced
Diagnosis and management of autoimmunehepatitis. Autoimmunehepatitis is an inflammatory disease of the liver of unknown cause that may progress to liver cirrhosis and end stage liver failure if diagnosis is overlooked and treatment delayed. The clinical presentation is often that of acute hepatitis, sometimes very severe; less frequently, it can be insidious or completely asymptomatic hepatitis and periportal necrosis are the diagnostic hallmarks of autoimmunehepatitis. Prompt response to treatment with corticosteroids and other immunomodulatory drugs is almost universal and supports the diagnosis. The aims of treatment are to induce and maintain long term remission of liver inflammation. Treatment can often even reverse liver fibrosis, thus preventing progression to advanced
AutoimmunehepatitisAutoimmunehepatitis - Symptoms, diagnosis and treatment | BMJ Best PracticeSkip to main contentSkip to search * About us * Help * Subscribe * Access through your institution * Log inBMJ Best Practice * Help * Getting started * FAQs * Contact us * Recent updates * Specialties * Calculators * Patient leaflets * Videos * Evidence * Drugs * Recent updates of circulating auto-antibodies with a high serum globulin concentration, inflammatory changes on liver histology, and a favourable response to immunosuppressive treatment.[1]Manns MP, Czaja AJ, Gorham JD, et al. Diagnosis and management of autoimmunehepatitis. Hepatology. 2010;51:2193-2213.http://onlinelibrary.wiley.com/doi/10.1002/hep.23584/fullhttp://www.ncbi.nlm.nih.gov/pubmed/20513004?tool
Lkb1 orchestrates γδ T-cell metabolic and functional fitness to control IL-17-mediated autoimmunehepatitis. γδ T cells play a crucial role in immune surveillance and serve as a bridge between innate and adaptive immunity. However, the metabolic requirements and regulation of γδ T-cell development and function remain poorly understood. In this study, we investigated the role of liver kinase B1 -cell subsets in both the thymus and peripheral lymphoid tissues. Notably, loss of Lkb1 inhibited the commitment of Vγ1 and Vγ4 γδ T cells, promoted the maturation of IL-17-producing Vγ6 γδ T cells, and led to the occurrence of fatal autoimmunehepatitis (AIH). Notably, clearance of γδ T cells or blockade of IL-17 significantly attenuated AIH. Mechanistically, Lkb1 deficiency disrupted metabolic
Management of hepatocellular carcinoma, an important cause of death in Japanese autoimmunehepatitis patients. Hepatocellular carcinoma (HCC) in autoimmunehepatitis (AIH) was considered rare but is increasing with prolonged prognosis. Its impact on the overall prognosis of AIH is unknown, and treatment has not been established. To investigate the risk factors and prognosis of HCC in patients
Treatment response and clinical event-free survival in autoimmunehepatitis: a Canadian multicentre cohort study. We sought to identify predictors of outcome for people living with autoimmunehepatitis (AIH). We evaluated the clinical course of people with AIH across 11 Canadian centres. Biochemical changes were analysed using linear mixed-effect and logistic regression. Clinical outcome ALT at 6 months was associated with worse clinical event-free survival. In people living with AIH, sustained elevated aminotransferase values, but not IgG, are associated with poorer long-term outcomes. Biochemical response and long-term survival are not associated with starting prednisone dose. Using clinical data from multiple Canadian liver clinics treating autoimmunehepatitis (AIH), we evaluate
Immunopathogenesis of Primary Biliary Cholangitis, Primary Sclerosing Cholangitis and AutoimmuneHepatitis: Themes and Concepts. Autoimmune liver diseases (AILDs) include primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmunehepatitis (AIH), a family of chronic immune-mediated disorders that target hepatocytes and cholangiocytes. Treatments remain non-specific
Advances in the evaluation and treatment of autoimmunehepatitis. The primary therapy of autoimmunehepatitis (AIH) has been established for over three decades. This review focuses on updates in the evaluation and management of patients with AIH. The evaluation of patients has recently been updated to include more definitive screening for other autoimmune diseases, including thyroid disease
Tacrolimus versus mycophenolate for AutoImmunehepatitis patients with incompLete response On first-line therapy (TAILOR study): a study protocol for a phase III, open-label, multicentre, randomised controlled trial. Autoimmunehepatitis (AIH) is a rare, chronic inflammatory disease of the liver. The treatment goal is reaching complete biochemical response (CR), defined as the normalisation
NMR-based metabolomic signature: An important tool for the diagnosis and to study pathogenesis of autoimmunehepatitis. Metabolomics are used to predict, diagnose, and monitor metabolic disorders but altered metabolomic signature has also been reported in diverse diseases, including autoimmune disorders. However, the metabolomic profile in autoimmunehepatitis (AIH), has not been investigated
Retrospective, single-center analysis of autoimmunehepatitis in Jordanian children: clinical features, treatments, and outcomes. This study describes clinical, biochemical, and histological features and long-term outcomes in pediatric patients diagnosed with autoimmunehepatitis (AIH) at King Abdullah University Hospital, Jordan. Retrospective, single-center study. King Abdullah University abnormalities included anemia (56.3%) and thrombocytopenia (37.5%). All patients underwent liver biopsy, with interface hepatitis present in 81.3% of cases. Treatment mainly involved prednisone and azathioprine. Three patients died, one discontinued therapy, two patients were lost to follow-up, and 10 remained on treatment. Autoimmunehepatitis affects Jordanian children, primarily female children. Jaundice
Isolated primary CNS lymphoma after liver transplantation for autoimmunehepatitis: a case report. Post-transplantation primary central nervous system lymphoma (PT-PCNSL) is a rare neoplasm that occurs in immunocompromised patients. It can manifest months or years after transplantation, presenting with various neurological symptoms. A 64-year-old woman, who had received a liver transplant due to autoimmunehepatitis, presented with generalized weakness, headache, and confusion. Further investigation revealed multiple ring-enhancing lesions in the right frontal and temporoparietal regions on brain MRI. A brain biopsy confirmed the diagnosis of PT-PCNSL. This case underscores the importance of considering PT-PCNSL in the differential diagnosis of contrast-enhancing brain lesions in post-transplant
Real-world evidence for factors associated with maintenance treatment practices among U.S. adults with autoimmunehepatitis. While avoidance of long-term corticosteroids is a common objective in the management of autoimmunehepatitis (AIH), prolonged immunosuppression is usually required to prevent disease progression. This study investigates the patient and provider factors associated
Efficacy and safety of infliximab in patients with autoimmunehepatitis. A limited number of drugs are used as standard or alternative therapies in autoimmunehepatitis (AIH). No specific-recommendations are available for patients failing to respond to these therapies. We analyzed the efficacy and safety of infliximab in patients with AIH. We performed a retrospective study of 42 patients