CaNAL is a longitudinal observational cohort study of patients diagnosed with Primary Biliary Cholangitis (PBC), Autoimmune Hepatitis (AIH), or overlap syndrome. This study creates a nationwide registry and network focusing on high quality long-term follow-up of individual patient data from major Canadian centers.

Primary Biliary Cholangitis (PBC) and Autoimmune Hepatitis (AIH) are rare and slowly progressive liver diseases associated with development of cirrhosis, liver cancer (HCC) and liver failure requiring liver transplantation or leading to premature death. The rarity and slowly progressive nature of these autoimmune liver diseases make them difficult to study and only a large scale approach combining patient data from multiple centers across Canada will allow new insights. The primary aim of the Canadian Network for Autoimmune Liver Disease is to build a Canadian registry of patients with PBC, AIH, and overlap syndrome. We capture patient characteristics, laboratory assessments and natural history, patient-reported outcomes including quality of life measures and environmental exposures, response to treatment, and pre- and post-transplant outcomes. We will then identify risk factors associated with critical outcomes for the patient, including response to treatment, progression to transplant, risk of liver cancer, and recurrent disease after transplant. We can identify biomarkers (biochemical indicators of progression of disease) to help diagnose autoimmune liver disease at its earliest stages, ensuring timely treatment and preventing disease progression. CaNAL will provide a better understanding of autoimmune liver diseases, biomarkers predictive of disease progression or non-response to therapy as well as better knowledge of the etiology and pathogenesis. CaNAL will also help to serve as a platform for conducting clinical trials or targeted lab-studies to answer important questions that are unlikely to be evaluated by the pharmaceutical industry.

Inclusion Criteria:

• Diagnosis of Primary Biliary Cholangitis and/or Autoimmune Hepatitis

Exclusion Criteria:

• Less than 18 years of age

Patients with a diagnosis of Primary Biliary Cholangitis and/or Autoimmune Hepatitis across Canada.

Patient data will be anonymized. A standardized electronic case record (e-CRF) form will be used to capture the data. The e-CRF form has been designed in REDCap, a secure web based application supporting data capture for research studies. REDCap is designed to comply with HIPAA security regulations. The CaNAL REDCap database (REDCap project CaNAL) has been established at the University of Alberta under the jurisdiction of the Women and Children's Health Research Institute (WCHRI).

Individual centres will have access only to their own data and patient identifiers will only be visible to the individual centres. The steering committee will have access to all anonymized data within the REDCap database, that is without any patient identifiers. Main centres in Toronto and Edmonton will have access to de-identified data.

Results will be disseminated via publication in international literature and presentation at international liver conferences. Patient data will be anonymous.