Health-related quality of life in autoimmune hepatitis

Autoimmune hepatitis(AIH)is a severe chronic autoimmune disease and has a significant impact on the patient’s quality of life,in particular regarding psychological problems such as anxiety and depression.Consistent evidence on which patient-related,disease-related or physician-related factors cause health-related quality of life(HRQoL)impairment in patients with AIH is lacking.Current studies on HRQoL in AIH are mainly single-centered,comprising small numbers of patients,and difficult to compare because of the use of different questionnaires,patient populations,and cutoff values.Literature in the pediatric field is sparse,but suggests that children/adolescents with AIH have a lower HRQoL.Knowledge of HRQoL and cohesive factors in AIH are important to improve healthcare for AIH patients,for example by developing an AIH-specific chronic healthcare model.By recognizing the importance of quality of life beyond the concept of biochemical and histological remission,clinicians allow us to seek enhancements and possible interventions in the management of AIH,aiming at improved health.

Antigen presentation,autoantibody production,and therapeutic targets in autoimmune liver disease

The liver is an important immunological organ that controls systemic tolerance.The liver harbors professional and unconventional antigen-presenting cells that are crucial for tolerance induction and maintenance.Orchestrating the immune response in homeostasis depends on a healthy and well-toned immunological liver microenvironment,which Is maintained by the crosstalk of liver-resident antigen-presenting cells and intrahepatic and liver-infiltrating leukocytes.In response to pathogens or autoantigens,tolerance is disrupted by unknown mechanisms.Intrahepatic parenchymal and nonparenchymal cells exhibit unique antigen-presenting properties.The presentation of microbial and endogenous lipid-,metabolite-and peptide-derived antigens from the gut via conventional and nonconventional mechanisms can educate intrahepatic immune cells and elicit effector responses or tolerance.Perturbation of this balance results in autoimmune liver diseases,such as autoimmune hepatitis,primary biliary cholangitis,and primary sclerosing cholangitis.Although the exact etiologies of these autoimmune liver diseases are unknown,it is thought that the disruption of tolerance towards self-antigens and microbial metabolites and lipids,as well as alterations in bile acid composition,may result in changes in effector cell activation and polarization and may reduce or impair protective antiinflammatory regulatory T and B cell responses.Additionally,the canonical and noncanonical transmission of antigens and antigen:MHC complexes via trogocytosis or extracellular vesicles between different(non)immune cells in the liver may play a role in the induction of hepatic inflammation and tolerance.Here,we summarize emerging aspects of antigen presentation,autoantibody production,and the application of novel therapeutic approaches in the characterization and treatment of autoimmune liver diseases.