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Autoimmune Hepatitis Primary Biliary Cholangitis

Remission Induction Of Primary Biliary Cholangitis

Autoimmune Hepatitis and Primary Biliary Cholangitis | Gina Choi, MD | UCLA Digestive Diseases
The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government.Know the risks and potential benefits of clinical studies and talk to your health care provider before participating. Read our disclaimer for details.
First Posted : October 18, 2016Last Update Posted : October 27, 2021
  • Study Details
Condition or disease
Hepatitis, AutoimmuneCholangitisLiver Cirrhosis, BiliaryCholestasisDrug: Ursodeoxycholic acid combination of immunosuppressive agentsDrug: Ursodeoxycholic AcidNot Applicable
Layout table for study information

Study Type :
Treatment
Official Title:Biochemical Response of Primary Biliary Cholangitis-autoimmune Hepatitis Overlap Syndrome Induced by Ursodeoxycholic Acid Only or Combination Therapy of Immunosuppressive Agents
Study Start Date :
Experimental: Ursodeoxycholic acid + immunosuppressive agents groupUrsodeoxycholic acid + immunosuppressive agents Drug: Ursodeoxycholic acid combination of immunosuppressive agentsUrsodeoxycholic acid combination of immunosuppressive agents
Active Comparator: Ursodeoxycholic acid groupUrsodeoxycholic acid

How Is Pbc Treated

Patients most often respond to bile acid therapy taken by mouth daily for life. The medication has minimal side effects, improves the livers ability to function and retards progression of cirrhosis. Treatment has been shown to extend life expectancy in patients with or without cirrhosis, delaying or preventing the need for a liver transplant.

A Randomised Clinical Trial Assessing The Efficacy And Safety Of Mycophenolate Mofetil Versus Azathioprine For Induction Of Remission In Treatment Primary Biliary Cholangitis

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government.Know the risks and potential benefits of clinical studies and talk to your health care provider before participating. Read our disclaimer for details.
First Posted : June 21, 2021Last Update Posted : June 21, 2021
  • Study Details
Condition or disease
Autoimmune HepatitisPrimary Biliary CirrhosisDrug: Methylprednisolone and Mycophenolate mofetilDrug: Methylprednisolone and azathioprinePhase 4
Layout table for study information

Study Type :
Treatment
Official Title:A Randomised Clinical Trial Assessing the Efficacy and Safety of Mycophenolate Mofetil Versus Azathioprine for Induction of Remission in Treatment Primary Biliary Cholangitis-Autoimmune Hepatitis Overlap Syndrome
Actual Study Start Date :
Experimental: Methylprednisolone and Mycophenolate mofetil Drug: Methylprednisolone and Mycophenolate mofetilMethylprednisolone combination of mycophenolate mofetil
Active Comparator: Methylprednisolone and Azathioprine Drug: Methylprednisolone and azathioprine
  • Biochemical remission The percentage of patients in remission, defined as normalization of serum transaminase and IgG levels after 6 months of treatment, per treatment group
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    Other Antibodies Associated With Aih

    Anti-Liver Cytosol Type 1 Antibodies

    Anti-LC1 antibodies directed against liver cytosol 1 target epitopes of the enzyme formiminotransferase cyclodeaminase . They are present in about 30% of type 2 AIH patients, alone or in combination with anti-LKM-1 . In IFT, anti-LC1 stain hepatocytes but spare the centrilobular areas of the liver. By contrast, anti-LKM-1 stain hepatocytes throughout the liver lobule. When both antibodies are coexistent, anti-LKM-1 cover the spared areas of anti-LC1. Thereby, anti-LKM-1 can mask the presence of anti-LC1. Solid-phase assays help to identify anti-LC-1 . When anti-LC1 are the sole antibodies being detected, they strongly support the diagnosis of type 2 AIH. However, anti-LC1 are not AIH specific and can also be detected in patients with HCV .

    Antineutrophil Cytoplasmatic Antibodies With a Perinuclear Staining Pattern

    The presence of p-ANCA can support the diagnosis of AIH, especially in the absence of other autoantibodies . However, p-ANCA can also be detected in chronic viral hepatitis, inflammatory bowel disease , PSC or microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis . p-ANCA mainly react with myeloperoxidase. Atypical p-ANCA , which are characterized by the retention of a perinuclear staining on formaldehyde-fixed neutrophils seem to be more specific for autoimmune liver diseases and IBD .

    Anti-Asialoglycoprotein Receptor Antibodies

    Journal Of Medical Cases

    Primary Sclerosing Cholangitis: Autoimmune Hepatitis and ...

    QUICK LINKS

    Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
    Article copyright, the authors Journal compilation copyright, J Med Cases and Elmer Press Inc
    Journal website https://www.journalmc.org

    Volume 12, Number 4, April 2021, pages 131-133

    Primary Biliary Cholangitis Overlap With Autoimmune Hepatitis? A Case Report

    Vitor Costaa, c, Marta Moreirab, Raquel Costaa, Joana Fontesa

    aDepartment of Medicine, Unidade Local de Saude do Alto Minho, Largo Conde de Bertiandos, Ponte de Lima 4990-041, PortugalbDepartment of Gastroenterology, Unidade Local de Saude do Alto Minho, Largo Conde de Bertiandos, Ponte de Lima 4990-041, PortugalcCorresponding Author: Vitor Costa, Department of Medicine, Unidade Local de Saude do, Alto Minho, Largo Conde de Bertiandos, Ponte de Lima 4990-041, Portugal

    Manuscript submitted November 22, 2020, accepted December 1, 2020, published online February 8, 2021Short title: Cholangitis Overlap With Autoimmune Hepatitisdoi: https://doi.org/10.14740/jmc3623

    Top

    Keywords: Primary biliary cholangitis Ursodeoxycholic acid Anti-mitochondrial antibodies Cirrhosis Autoimmune hepatitis Overlap syndrome

    IntroductionTop

    The major autoimmune disorders of the liver are the autoimmune hepatitis , primary biliary cholangitis and primary sclerosing cholangitis .

    Case ReportTop

    On physical examination, he was anicteric, without adenopathies, hepatomegaly/splenomegaly, without stigmas of chronic liver disease.

    DiscussionTop

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    Can Primary Biliary Cholangitis Be Prevented

    Because doctors do not know the cause of PBC, it cannot be prevented. However, you can take steps to lessen liver damage, including:

    • Quit smoking, stop drinking alcohol and stop using illegal drugs
    • Take all medicines as directed by your doctor.
    • Eat a healthy, well balanced diet.
    • Get regular exercise, such as walking.

    Healthy food choices could include eating foods rich in vitamins A, D, E and K and/or supplements of these vitamins. Also, foods high in vitamin D and calcium could help prevent osteoporosis. Ask your doctor for specific foods high in these vitamins and minerals.

    Foods to avoid include raw shellfish plus food high in salt, fat, and carbohydrates especially added sugars.

    Your Digestive System & How It Works

    The digestive system is made up of the gastrointestinal tractalso called the digestive tractand the liver, pancreas, and the gallbladder. The GI tract is a series of hollow organs joined in a long, twisting tube from the mouth to the anus.

    This content is provided as a service of the National Institute of Diabetes and Digestive and Kidney Diseases, part of the National Institutes of Health. The NIDDK translates and disseminates research findings to increase knowledge and understanding about health and disease among patients, health professionals, and the public. Content produced by the NIDDK is carefully reviewed by NIDDK scientists and other experts.

    The NIDDK would like to thank:John Moore Vierling, M.D., Baylor College of Medicine

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    About Primary Biliary Cholangitis

    The principal roles of the liver include removal of toxins from the blood and processing food nutrients into proteins, fats and carbohydrates. The liver produces bile, which is stored in the gall bladder and added to the digestive tract via bile ducts to help break down dietary fats.

    Primary biliary cholangitis is an autoimmune condition characterised by chronic inflammation and subsequent scarring of the bile ducts within the liver. The cause is unknown, although researchers have ruled out alcohol and diet as possible triggers. Women are 10 times more likely to develop PBC than men, for reasons unknown.

    The disease is usually diagnosed later in life, between the ages of 35 and 60 years. There is no cure, but treatment can slow the progression of the disease and alleviate symptoms. PBC is associated with other autoimmune diseases, including rheumatoid arthritis, scleroderma and Sjogrens syndrome.

    Paediatric And Young Adults

    Addressing Challenges in the Management of Primary Biliary Cholangitis

    From early reports of children with PSC it was clear that there were many clinical similarities between PSC and AIH, and in fact these patients had often initially been managed as having AIH.40,41 In approximately a third of these PSC cases, the diagnosis was only made after subsequent investigations, including cholangiography, which revealed features of PSC.

    A study that systematically evaluated consecutive children with liver disease and positive antibodies consistent with AIH, with screening cholangiogram and liver biopsy, found ~50% had abnormal cholangiograms.42 The term ASC was used for those with abnormal cholangiograms and positive autoantibodies, who had different characteristics to those with normal cholangiograms. The condition has only been described in paediatric populations.

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    Overview On Clinical And Laboratory Features

    Fatigue and pruritus are the most common symptoms in PBC patients, and often have a quite negative effect on quality of life ,. Patients with pruritus might have excoriations or bleeding as a result of chronic scratching. Melanin deposition may occur, causing hyperpigmentation of the skin in up to 50% of cases. Long-term complications of the disease include osteopenia and osteoporosis, hyperlipidemia and vitamin deficiencies . In late stages, typical signs of cirrhosis and portal hypertension might be present, as well as an increased risk of hepatocellular carcinoma ,. In the cirrhotic stage of the disease, radiological HCC surveillance is therefore mandatory . Patients with PBC, particularly women, have a higher likelihood of other concomitant autoimmune disorders, and up to 55% have an additional ongoing autoimmune process, such as autoimmune Hashimotos thyroiditis, Sjogrens syndrome and Raynauds disease ,.

    PBC may coexist with other liver autoimmune disorders, particularly AIH .

    In the last few years, efforts have been made to improve prognostic tools in PBC patients. The Global PBC Study Group and the UK-PBC consortium developed two new continuous scoring systems, the GLOBE Score and the UK-PBC Risk Score, considering both measures of treatment response and parameters of disease severity ,,. Both showed better performance in the prediction of death or OLT than previous criteria ,.

    Antibodies To Biliary Epithelial Cells

    Few studies found antibodies of different subtypes in sera of patients with PSC directed against BEC . Levels of IgA antibodies directed against BEC were correlated with adverse patient outcomes . Furthermore, it has been demonstrated that antibodies against BEC and bacterial lipopolysaccharides co-activate cytokine release by BEC and therefore induce biliary immune responses . This provides further evidence for the involvement of microbiota in the pathogenesis of PSC. Testing for anti-BEC antibodies has not been introduced into clinical practice.

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    Efficacy And Safety Of Immunosuppressive Therapy For Pbcaih Overlap Syndrome Accompanied By Decompensated Cirrhosis: A Real

    Li Yang

    1Department of Gastroenterology & Hepatology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, China

    2Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, China

    Abstract

    1. Introduction

    Autoimmune liver disease comprises a group of immune-mediated liver diseases that include autoimmune hepatitis , primary biliary cirrhosis , and primary sclerosing cholangitis . The occurrence of overlapping syndromes at different disease stages is not rare, with PBC-AIH overlap syndrome being the most common . The prevalence of PBC-AIH overlap syndrome is approximately 810% in adult patients with either PBC or AIH , and this low incidence contributes to imprecise diagnostic criteria furthermore, no standard therapy is currently available. According to the most recent guidelines based on the results of small studies, a combination of ursodeoxycholic acid and immunosuppressants is often recommended for PBC-AIH in clinical practice .

    2. Materials and Methods

    2.1. Inclusion Criteria

    West China Hospital is a 4300-bed tertiary teaching hospital affiliated with Sichuan University. The hospital has a liver transplant unit and is the leading hospital in the western areas of China.

    Cirrhosis was diagnosed according to the histological analysis, unequivocal imaging, or endoscopic examination . Decompensation was diagnosed by the presence of clinical complications, including ascites, variceal hemorrhage, and hepatic encephalopathy .

    What Causes Primary Biliary Cholangitis

    Autoimmune Hepatitis, Overlap Syndrome, Primary Sclerosing ...

    The cause of this disease is unknown. It may be related to problems in the immune system. Although PBC is technically not a hereditary disease, meaning a disease caused by a specific gene or genetic defect that is passed from parent to child, there appears to be some family link. PBC is more common among siblings and in families where one member has been affected.

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    What Is Primary Biliary Cholangitis

    Primary biliary cholangitis is a chronic autoimmune disease of the liver that slowly destroys its small to medium-sized bile ducts. This causes bile to remain in the liver, which can damage cells and cause scarring that can lead to cirrhosis if unrecognized and untreated.

    Bile is a liquid that is made in the liver and travels down the bile ducts to enter the duodenum, the portion of the small bowel next to the stomach. Bile contains acids, which are needed to digest fats and absorb fat-soluble vitamins A, D, E and K. These acids also act as hormones with important effects in maintaining health.

    PBC advances slowly, providing doctors an opportunity for early treatment. Many patients lead active and productive lives for more than 10 to 15 years after diagnosis. In fact, patients who show no symptoms at the time of diagnosis often remain symptom-free for years. And the good news is that patients whose liver tests return to normal on treatment can expect a normal life expectancy.

    If cirrhosis develops, PBC may lead to life-threatening complications. Cirrhosis occurs when scar tissue blocks the flow of blood through the liver and impairs its ability to function normally.

    If the liver cannot filter waste from the intestine, confusion and altered levels of consciousness can result.

    The goals of therapy are to prevent development of cirrhosis in patients with earlier stages of disease and slow progression and prevent deterioration in patients who already have cirrhosis.

    The Spinal Itch Pathway

    After the pruriceptive primary afferent has been activated, the signal is transmitted from the skin into the spinal dorsal horn. In this area, a number of interneurons will either be inhibited or activated to promote activation of projection neurons, mediating the puriceptive signal to the brain. The GRP-GRPR interneuron system has been found to be important for mediating both histaminergic and non-histaminergic itch, where the GRP neurons activate GRPR neurons to promote itch

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    What Lifestyle Changes May Be Helpful For Pbc Patients

    Maintaining a healthy lifestyle helps patients feel better, prevents loss of calcium from bones and may relieve or prevent some of the secondary symptoms of PBC. Your doctor may give you recommendations about:

    • A reduced sodium diet
    • Taking calcium and vitamin D supplements
    • Avoiding or reducing the consumption of alcohol
    • Reducing stress

    Antibodies Found In Patients With Psc And Additional Aih

    Primary Sclerosing Cholangitis (PSC) Care at Mayo Clinic

    Additional features of AIH are found in approximately 5% of adult patients with PSC but are prevalent in 3565% of children with PSC . In children and adolescents, the primary manifestation of disease may be that of typical AIH, together with cholangiographic and histological changes of sclerosing cholangitis . Testing patients with PSC for ANA, anti-SMA using IFT and for anti-SLA/LP using ELISA should be performed when additional AIH is suspected. Liver histology is mandatory to establish suspected AIH in patients with PSC, which will affect treatment decisions since AIH should be treated with immunosuppression .

    Antibodies Against Glycoprotein 2

    Recently, IgA-class antibodies against glycoprotein 2 , which were formerly linked to severe types of Crohns disease , were detected in sera of patients with PSC at a prevalence of 46.771.5%. The presence of anti-GP2 IgA was strongly associated with large bile-duct involvement, development of cholangiocarcinoma, and increased mortality . Therefore, anti-GP2 might serve as a novel biomarker of risk stratification in patients with PSC. Moreover, evidence for the involvement of GP2 in immune responses of the intestinal mucosa to gut bacteria provides a further pathophysiological link to the recently discovered aberrant community structure of the gut microbiota in patients with PSC .

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    Autoantibodies In Autoimmune Liver Diseaseclinical And Diagnostic Relevance

    • 11st Department of Medicine, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
    • 2Martin Zeitz Centre for Rare Diseases, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany

    Testing for liver-related autoantibodies should be included in the workup of patients with hepatitis or cholestasis of unknown origin. Although most of these autoantibodies are not disease specific, their determination is a prerequisite to diagnose autoimmune hepatitis and primary biliary cholangitis , and they are components of the diagnostic scoring system in these diseases. In primary sclerosing cholangitis , on the other hand, autoantibodies are frequently present but play a minor role in establishing the diagnosis. In PSC, however, data on antibodies suggest a link between disease pathogenesis and the intestinal microbiota. This review will focus on practical aspects of antibody testing in the three major autoimmune liver diseases AIH, PBC, and PSC.

    How Pbc Is Treated

    PBC is a progressive condition, which means the damage to the liver can steadily get worse over time.

    The rate at which PBC progresses varies between individuals. Sometimes, it can take decades.

    Without treatment, the liver can become so badly damaged that it no longer works properly. This is known as liver failure and can be fatal.

    Liver failure can be prevented in the majority of people being treated for PBC with current treatments such as ursodeoxycholic acid and obeticholic acid.

    Other medicines can help relieve the itchiness associated with PBC. Occasionally, if the liver is severely damaged, a liver transplant may be needed.

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