A-LiNK: Improving Outcomes in Autoimmune Liver Disease
Autoimmune HepatitisPrimary Sclerosing CholangitisThe Autoimmune Liver disease Network for Kids (A-LiNK) is a multi-institutional group with the mission to deliver the best care to kids with pediatric autoimmune liver disease (AILD). This study will establish a shared clinical registry and a learning health network for the participating sites focusing on collecting and transmitting clinical measurement data, information about processes, and participation in an improvement collaborative. Pediatric Autoimmune Hepatitis (AIH) and Primary Sclerosing Cholangitis (PSC), represent a spectrum of AILD which present unique diagnostic and therapeutic challenges.A lack of accepted guidelines for disease monitoring or symptom management results in wide treatment variation with liver transplants indicated in refractory, progressive disease. The aims of A-LiNK are to: 1.) Create a learning health network focused on patient-centered outcomes research characterized by transparent sharing among centers, common priorities, and feasible plans for implementing new practices; 2) shift from traditional investigator-driven study to a patient and family-centered approach, and 3.) improve clinical outcomes and quality of life for pediatric AILD patients.
Study of the Clinical Features of Autoimmune Hepatitis
HepatitisAutoimmuneThe purpose of this study is to explore the pathogenes, clinical characteristics, laboratory and histological examination results, treatment and prognosis of autoimmune hepatitis(AIH). At phase 1, the investigators focus on studying of the clinical characteristics of acute autoimmune hepatitis, and then will study the difference about treatment effects between acute autoimmune hepatitis and chronic AIH. Morever, the investigators have noticed that drug induced AIH have some special characteristics that may be beneficial to distinguish it with durg induced liver disease. Therefore the investigators will do some studies about drug induced AIH or other disease which maybe related to the onset of AIH.
PredIcting sterOid depeNdEnt livEr injuRy With Polyreactive Immunoglobulin G
Autoimmune HepatitisAutoimmune Liver DiseaseThe investigators identified polyreactive immunoglobulin G (pIgG) in adults (published in Hepatology: https://doi.org/10.1002/hep.32134) and children (in preparation). Quantification of these pIgG using a "home-made" ELISA facilitates the diagnosis of autoimmune hepatitis (AIH) as compared to non-AIH liver diseases and healthy controls. Positivity for pIgG was independent from ANA/SMA positivity and equally diagnostic for AIH even when conventional autoantibodies (ANA/SMA/SLA/LKM) were negative. Additionally, the frequency of pIgG was lower than conventional autoantibodies (ANA, SMA) in vaccinia/drug associated severe liver injury in a retrospective multicenter study after Covid-19 vaccination (https://doi.org/10.1016/j.jhepr.2022.100605). Aims of the study The study aims to evaluate the diagnostic capacity of pIgG to predict AIH in comparison to other liver diseases prospectively. To avoid diagnostic inaccuracy between AIH with long-term need for an immunosuppression and drug induced liver injury with autoimmune features, which can be indistinguishable from AIH at baseline and which has a very low relapse rate after a short steroid course, a follow-up after six months is obligatory for inclusion. Therefore, the investigators will collect one serum sample from every patient (without immunosuppressive treatment) that presents to the respective hospital for evaluation of liver disease by liver biopsy within one year after initiation of the study and that provided written informed consent. Follow-up for evaluation of steroid dependency at six months after diagnosis is obligatory.
Swiss Autoimmune Hepatitis Cohort Study
HepatitisAutoimmuneResearch project in which biological material is sampled and health-related personal data is further used and collected. Coded data are used.
A Single-Site Tissue Repository Providing Annotated Biospecimens for Approved Investigator-directed...
Age-Related Macular DegenerationAllergies45 moreTo collect, preserve, and/or distribute annotated biospecimens and associated medical data to institutionally approved, investigator-directed biomedical research to discover and develop new treatments, diagnostics, and preventative methods for specific and complex conditions.
MRI Based Biomarkers in Pediatric Autoimmune Liver Disease
Autoimmune Liver DiseasePrimary Sclerosing Cholangitis1 moreAutoimmune liver diseases (AILD), which include Primary Sclerosing Cholangitis (PSC) and Autoimmune Hepatitis (AIH) are a common etiological factors for chronic liver disease among adolescents. In all these conditions, autoimmune lymphocyte responses are thought to orchestrate inflammatory injury against hepatocytes (primarily in AIH) or cholangiocytes (in PSC). In this proposal we aim to evaluate the Magnetic Resonance Imaging (MRI) modalities; MR cholangiopancreatography (MRCP) and MR elastography (MREL), as non-invasive biomarkers to assess two primary pathophysiological processes of AILD: bile duct damage and liver fibrosis. In this cross-sectional study MRI based findings of bile duct injury and liver fibrosis will be correlated with both liver histology and circulating biomarkers of these disease processes.
Predictive Models of Hepatic Decompensation and Survival Outcomes in Pediatric Patients With Cirrhosis...
CirrhosisBiliary Atresia5 moreThe aim of this study was to developed and validated models to predict hepatic decompensation and survivals in pediatric patients with cirrhosis and compared these models with currently available models.
AIH Risk Stratification With Multiparametric MRI
Autoimmune HepatitisThe primary aim of this study is to investigate whether the baseline cT1 can predict those whose condition relapses following treatment withdrawal. The secondary aim is to investigate correlation of cT1 with histology to explore utility as a monitoring tool. A total of 97 patients with AIH will be recruited and divided into 2 arms. 20 of which will be treatment naive and the other 77 will have been on treatment for the past 18-24 months and will be coming in for therapy cessation review.
Abatacept for Treatment of Recurrent or de Novo Autoimmune Hepatitis
Autoimmune HepatitisThe purpose of this study is to evaluate whether Orencia® (Abatacept) improves outcomes in liver transplant patients with recurrent or de novo AIH (autoimmune hepatitis) that has not responded to previous therapy. AIH that does not respond to steroids or conventional immunotherapy often affects young patients and leads to irreversible liver damage. There is currently no effective therapy for this condition.
A Phase 2a Study to Evaluate the Safety and Efficacy of Cannabidiol Only as Maintenance Therapy...
Autoimmune HepatitisSubjects with stable autoimmune hepatitis disease currently being administered corticosteroids with or without azathioprine (AZA) treatment will be be treated with Cannabidiol instead of standard of care treatment with corticosteroids