Systemic Complications of Primary Biliary Cholangitis
This article discusses the clinical presentation, pathophysiology, and management of pruritus (itching), fatigue, osteoporosis, hyperlipidemia, fat-soluble vitamin deficiencies, malignancies, cardiac dysfunction, bacterial cholangitis, cholemic (bile cast) nephropathy, and Sicca syndrome. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - August 26, 2023 Category: Gastroenterology Authors: Mariana Zapata, Hendrick Pagan-Torres, Marlyn J. Mayo Source Type: research
Post-Transplant Management and Complications of Autoimmune Hepatitis, Primary Biliary Cholangitis, and Primary Sclerosing Cholangitis including Disease Recurrence
Autoimmune liver diseases have unique post-transplant considerations. These recipients are at increased risk of rejection, and recurrent disease may also develop, which can progress to graft loss and increase mortality. Monitoring for and managing these complications is therefore important, though data on associated risk factors and immunosuppression strategies has in most cases been mixed. There are also other disease-specific complications that require management and may impact these decisions, including inflammatory bowel disease in PSC. Further work to better understand the optimal management strategies for these patie...
Source: Clinics in Liver Disease - August 26, 2023 Category: Gastroenterology Authors: Jacqueline B. Henson, Lindsay Y. King Source Type: research
Treatment of Autoimmune Hepatitis
The goal of autoimmune hepatitis treatment is to achieve clinical and biochemical remission, which is associated with significantly improved outcomes. Induction treatment with corticosteroids and the subsequent addition of steroid-sparing therapy with gradual tapering of corticosteroids remains the standard of care. Several alternatives to azathioprine and second-line agents, such as mycophenolate mofetil, tacrolimus, cyclosporine, sirolimus, or rituximab, have been evaluated in those with intolerance or inadequate response to standard-of-care therapy. Treatment withdrawal is achievable in less than 20% of patients after 2...
Source: Clinics in Liver Disease - August 25, 2023 Category: Gastroenterology Authors: Aparna Goel, Paul Kwo Source Type: research
Primary Sclerosing Cholangitis
This article discusses the epidemiology, clinical presentation, and diagnostic workup in patients with PSC. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - August 21, 2023 Category: Gastroenterology Authors: Aalam Sohal, Sanya Kayani, Kris V. Kowdley Source Type: research
Autoimmune Hepatitis
Autoimmune hepatitis (AIH) is a chronic immunologic disorder in which the immune system targets the liver. The disease has a genetic basis and this accounts for the epidemiologic variation observed in serologic testing and clinical presentation across different populations. The incidence of AIH increases with age into the 70s and seems to be increasing in prevalence. Most patients test positive for antinuclear antibody, ASMA, or anti-LKM but about 20% of patients do not have these serologic markers. At clinical presentation, patients may be asymptomatic, symptomatic, have acute liver failure, or decompensated cirrhosis. (S...
Source: Clinics in Liver Disease - August 17, 2023 Category: Gastroenterology Authors: Mitchell L. Shiffman Source Type: research
Autoimmune Markers in Primary Biliary Cholangitis
This article describes various methods used to evaluate antibodies as well as describe the antibodies present in PBC. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - August 17, 2023 Category: Gastroenterology Authors: Shivani K. Shah, Christopher L. Bowlus Source Type: research
Phenotypes of Primary Sclerosing Cholangitis and Differential Diagnosis
Primary sclerosing cholangitis is a heterogenous immune-mediated disorder characterized by chronic inflammation and stricturing of the bile ducts. Though the driving pathophysiologic mechanisms remain elusive, there are several observed clinical phenotypes of the disease. The distribution of bile duct involvement, presence of concomitant inflammatory bowel disease, significant infiltration of IgG4-positive plasma cells, and overlapping features with other autoimmune disease has significant implications for prognosis and treatment. As there remains no pathognomonic finding for primary sclerosing cholangitis, a broad differe...
Source: Clinics in Liver Disease - August 17, 2023 Category: Gastroenterology Authors: Brian H. Horwich, Douglas T. Dieterich Source Type: research
Treatment of Primary Sclerosing Cholangitis Including Transplantation
Primary sclerosing cholangitis is a progressive cholestatic liver disease that causes stricturing of the intra and extrahepatic bile ducts that can lead to cirrhosis and end stage liver disease. Effective medical therapy has been elusive, but a course of ursodeoxycholic acid may be prescribed at doses of 17-23 mg/kg/day for up to a year to determine if a reduction in serum alkaline phosphatase is observed. A number of drugs are under investigation, including FXR agonists with choleretic and antimicrobial properties. Liver transplantation for PSC has one of the highest survival rates, but recurrent PSC i s seen in up to 2...
Source: Clinics in Liver Disease - August 17, 2023 Category: Gastroenterology Authors: William H. Wheless, Mark W. Russo Source Type: research
Autoimmune Hepatitis
Genome-wide association analyses suggest that HLA genes including HLA-DRB*0301, HLA-DRB*0401, and HLA-B*3501 as well as non-HLA genes including CD28/CTLA4/ICOS and SYNPR increased AIH susceptibility. The destruction of hepatocytes is the result of the imbalance between proinflammatory cells and immunosuppressive cells, especially the imbalance between Tregs and Th17 cells. The microbiome in patients with AIH is decreased in diversity with a specific decline in Bifidobacterium and enrichment in Veillonella and Faecalibacterium. Recent evidence has demonstrated the pathogenic role of E. gallinarum and L.reuteri in inducing...
Source: Clinics in Liver Disease - August 12, 2023 Category: Gastroenterology Authors: Zhou Yuming, Tang Ruqi, Merrill Eric Gershwin, Ma Xiong Source Type: research
Primary Biliary Cholangitis
Using ursodeoxycholic acid as a standard treatment and for its ability to test for antimitochondrial antibody to accelerate diagnosis, survival of primary biliary cholangitis patients has approached that of the general population, leading to a change in nomenclature from primary biliary cirrhosis to primary biliary cholangitis to more accurately describe the disease. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - August 12, 2023 Category: Gastroenterology Authors: Muhammad Salman Faisal, Humberto C. Gonzalez, Stuart C. Gordon Source Type: research
Primary Biliary Cholangitis
Primary biliary cholangitis (PBC) is the most common of the autoimmune liver diseases, in which there is chronic small bile duct inflammation. The pathophysiology of PBC is multifactorial, involving immune dysregulation and damage to biliary epithelial cells, with influences from genetic factors, epigenetics, the gut-liver axis, and environmental exposures. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - August 12, 2023 Category: Gastroenterology Authors: Inbal Houri, Gideon M. Hirschfield Source Type: research
Diagnostic Tests in Primary Sclerosing Cholangitis
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by progressive inflammation and fibrosis of the biliary tree leading to biliary strictures, cholangitis, and cirrhosis. Early in presentation, patients may have normal liver tests, though over time develop a cholestatic pattern of liver injury. Diagnosis is made radiographically with magnetic resonance or endoscopic cholangiography. While several autoantibodies are associated with PSC, none have proven to have adequate diagnostic utility. Liver biopsy is rarely recommended unless to evaluate for small-duct PSC or overlap syndrome. Ela...
Source: Clinics in Liver Disease - August 12, 2023 Category: Gastroenterology Authors: Clara Y. Tow, Erica Chung, Bindu Kaul, Amarpreet Bhalla, Brett E. Fortune Source Type: research
The “B and D” Times, They Are A-Changin’
The state of hepatitis B and delta infection is changing rapidly. Since the advent 1 year ago of the Advisory Committee on Immunization Practices ’ recommendation to vaccinate all adults for hepatitis B, we have met other milestones. This great advance was followed in the spring of 2023 by the Centers for Disease Control and Prevention’s recommendation to test all adults for hepatitis B with the hepatitis B virus (HBV) triple panel. More recently, in June of 2023, the European Association for the Study of the Liver (EASL)’s guidelines came out with the recommendation to test all HBsAg-positive individuals for delta i...
Source: Clinics in Liver Disease - August 12, 2023 Category: Gastroenterology Authors: Robert G. Gish Tags: Preface Source Type: research
Autoimmune Hepatitis Including Primary Biliary Cholangitis and Primary Sclerosing Cholangitis
Autoimmune hepatitis, Primary Biliary Cholangitis (PBC), and Primary Sclerosing Cholangitis (PSC) are all chronic, progressive, noncurable conditions affecting the liver whose pathogenesis remains incompletely understood and whose diagnosis is often delayed or missed in our patient population. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - August 12, 2023 Category: Gastroenterology Authors: David Bernstein Tags: Preface Source Type: research
Diagnosis of Autoimmune Hepatitis
This article presents the currently adopted approach to AIH diagnosis and explores the challenges with accurately identifying this disease entity. AIH offers no pathognomonic findings, instead relies on clinical presentation, serology, and histology to make the diagnosis. Diagnostic scoring systems support clinical judgment and serve as valuable tools in diagnosis and research. Histological analysis remains the cornerstone of diagnosis and to this day biopsy is essential to make the diagnosis. (Source: Clinics in Liver Disease)
Source: Clinics in Liver Disease - August 8, 2023 Category: Gastroenterology Authors: Ben Flikshteyn, Kamal Amer, Zaid Tafesh, Nikolaos T. Pyrsopoulos Source Type: research
