Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC) are the three major autoimmune liver diseases (AILDs). AIH, PBC, and PSC are all complex disorders resulting from genetic factors in combination with as yet, unknown environmental factors.
There is no curative treatment for all three disorders, and a significant number of patients eventually progress to end-stage liver disease requiring liver transplantation (LT). Liver transplantation in this context has a favorable overall outcome, with a current patient and graft survival of more than 80% at five years. However, all three conditions can recur after transplantation and are associated with an increased risk of acute cellular and chronic ductopenic rejection. Crosstalk between the alloimmune and autoimmune responses may perpetuate each other. An immunological response to auto- or alloantigens is well recognized after LT in patients transplanted for non-autoimmune indications and is termed de novo autoimmune hepatitis.
In this Research Topic, we aim to review new findings on pathological processes and mechanisms leading to autoimmunity in the liver and their possible involvement in the immune response against graft after LT.
Further goals of this collection are to identify recent advances in diagnosis, treatment, and understanding of autoimmune hepatitis and primary biliary cholangitis, providing an up-to-date perspective on the epidemiology, clinical practice, research, and current knowledge on autoimmune liver disease.
All three disorders have a progressive course that, if left untreated, evolves into liver failure requiring liver transplantation (LT). The aim of treatment is to abolish or reduce inflammation, cholestasis, and the progression to cirrhosis. Standard therapy in AIH consists of a combination of corticosteroids and azathioprine, which is effective in 80% of patients; however, progression may occur despite apparently adequate treatment. Other immunosuppressive agents such as mycophenolate mofetil, tacrolimus, cyclosporine, and T-cell therapies have been used in refractory cases with limited success. The only approved therapy for PBC and PSC is ursodeoxycholic acid (UDCA). In PBC, response to UDCA has a favorable effect on long-term survival and fibrosis progression. However, those who do not respond to UDCA have poor survival (10-year survival is >95% and <80% in UDCA responders and non-responders, respectively). Other drugs such as obeticholic acid (OCA), an agonist of the Farsenoid X receptor involved in the metabolism and enterohepatic circulation of bile acids and bezafibrate, are beneficial as second-line therapies in patients not responding to ursodeoxycholic acid.
In PSC, treatment with UDCA improves serum liver tests but does not improve survival. Thus, there is a subgroup of patients who do not respond to current treatments for these autoimmune liver diseases and have a poor prognosis, for whom new treatment options are needed.
However, the development of new agents is currently hampered by the inadequate understanding of the etiology and pathogenesis of these autoimmune conditions.
We are interested in receiving Review and Original articles related to, but not limited to, the following areas:
• Critical overviews of the current diagnostic criteria
• New immunoserological and imaging tools for the early diagnosis of major autoimmune liver diseases and optimal prognostic assessment during follow-up
• Current available treatment options with an overview of potential new therapeutic agents
• Comprehensive and extensive approaches to managing autoimmune liver diseases, including; adequate symptom control, early recognition of extra-hepatic manifestations, and surveillance of complications
Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC) are the three major autoimmune liver diseases (AILDs). AIH, PBC, and PSC are all complex disorders resulting from genetic factors in combination with as yet, unknown environmental factors.
There is no curative treatment for all three disorders, and a significant number of patients eventually progress to end-stage liver disease requiring liver transplantation (LT). Liver transplantation in this context has a favorable overall outcome, with a current patient and graft survival of more than 80% at five years. However, all three conditions can recur after transplantation and are associated with an increased risk of acute cellular and chronic ductopenic rejection. Crosstalk between the alloimmune and autoimmune responses may perpetuate each other. An immunological response to auto- or alloantigens is well recognized after LT in patients transplanted for non-autoimmune indications and is termed de novo autoimmune hepatitis.
In this Research Topic, we aim to review new findings on pathological processes and mechanisms leading to autoimmunity in the liver and their possible involvement in the immune response against graft after LT.
Further goals of this collection are to identify recent advances in diagnosis, treatment, and understanding of autoimmune hepatitis and primary biliary cholangitis, providing an up-to-date perspective on the epidemiology, clinical practice, research, and current knowledge on autoimmune liver disease.
All three disorders have a progressive course that, if left untreated, evolves into liver failure requiring liver transplantation (LT). The aim of treatment is to abolish or reduce inflammation, cholestasis, and the progression to cirrhosis. Standard therapy in AIH consists of a combination of corticosteroids and azathioprine, which is effective in 80% of patients; however, progression may occur despite apparently adequate treatment. Other immunosuppressive agents such as mycophenolate mofetil, tacrolimus, cyclosporine, and T-cell therapies have been used in refractory cases with limited success. The only approved therapy for PBC and PSC is ursodeoxycholic acid (UDCA). In PBC, response to UDCA has a favorable effect on long-term survival and fibrosis progression. However, those who do not respond to UDCA have poor survival (10-year survival is >95% and <80% in UDCA responders and non-responders, respectively). Other drugs such as obeticholic acid (OCA), an agonist of the Farsenoid X receptor involved in the metabolism and enterohepatic circulation of bile acids and bezafibrate, are beneficial as second-line therapies in patients not responding to ursodeoxycholic acid.
In PSC, treatment with UDCA improves serum liver tests but does not improve survival. Thus, there is a subgroup of patients who do not respond to current treatments for these autoimmune liver diseases and have a poor prognosis, for whom new treatment options are needed.
However, the development of new agents is currently hampered by the inadequate understanding of the etiology and pathogenesis of these autoimmune conditions.
We are interested in receiving Review and Original articles related to, but not limited to, the following areas:
• Critical overviews of the current diagnostic criteria
• New immunoserological and imaging tools for the early diagnosis of major autoimmune liver diseases and optimal prognostic assessment during follow-up
• Current available treatment options with an overview of potential new therapeutic agents
• Comprehensive and extensive approaches to managing autoimmune liver diseases, including; adequate symptom control, early recognition of extra-hepatic manifestations, and surveillance of complications
