The “National Primary Biliary Cholangitis Database” project aims to establish a nationwide research infrastructure to investigate the biology of PBC and, in particular, why a significant group of patients fail primary therapy with UDCA and are at risk of developing progressive disease and requiring liver transplantation. This research infrastructure is an invaluable resource for successful translational research in this field and would also be critical for drug development, in particular to assess the feasibility of clinical trials, support patient recruitment and evaluate the impact of new interventions.

Investigators will recruit patients and organise the collection of relevant clinical and biological data, including the optional collection of biological samples. Data will be collected using electronic case report forms (REDCap cloud) completed at baseline and annually thereafter. The clinical information will be used to identify clinical profiles of patients, while the biological samples will allow the study of key aspects of genetic and immunological make-up, as well as differences between different clinical phenotypes.

The main objective of the National Autoimmune Hepatitis Database is to collect all incident and prevalent cases of autoimmune hepatitis in Italy, making it a unique and privileged epidemiological observatory. Additionally, it aims to describe the various phenotypes of the disease and the risk factors associated with its progression.
The therapies currently available for the treatment of AIH demonstrate an efficacy exceeding 80%, but they present significant short- and long-term side effects, making the identification of new therapeutic options necessary. The database aims to provide real-world epidemiological data on the efficacy and safety of currently available drugs for the treatment of autoimmune hepatitis. Additionally, the database will allow ancillary studies aimed at correlating the impact of chronic therapy on the patients’ psychophysical well-being.
The investigators will recruit patients and organize the collection of relevant clinical and biological data, including the optional collection of biological samples. Data will be collected using electronic case report forms (REDCap cloud) completed at baseline and annually thereafter. The clinical information will be used to identify clinical profiles of patients, while the biological samples will allow the study of key aspects of genetic and immunological make-up, as well as differences between different clinical phenotypes.

The aim of the study is to conduct a nationwide data collection on this rare disease to describe the incidence and prevalence of PSC in Italy, to identify and define distinct phenotypes and subphenotypes of PSC patients, to identify factors that influence PSC progression and mortality, and to evaluate the long-term safety and efficacy of new therapies.
Despite the high mortality associated with PSC and efforts to optimise its management, there is no medical therapy that can halt the progression of PSC or prevent its serious complications.
Investigators will recruit patients and organise the collection of relevant clinical and biological data, including the optional collection of biological samples. Data will be collected using electronic case report forms (REDCap cloud) completed at baseline and annually thereafter. The clinical information will be used to identify clinical profiles of patients, while the biological samples will allow the study of key aspects of genetic and immunological make-up, as well as differences between different clinical phenotypes.