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*Corresponding Author: Ahed J Alkhatib, Department of Legal Medicine, Toxicology and Forensic Medicine, Jordan University of Science & Technology, Jordan.
Citation: Ahed J Alkhatib, (2023), Autoimmunity and liver disease: Updates of the literature, J New Medical Innovations and Research, 4(5); DOI:10.31579/2767-7370/056
Copyright: © 2023, Ahed J Alkhatib. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Received: 05 August 2023 | Accepted: 14 August 2023 | Published: 21 August 2023
Keywords: autoimmune liver diseases; immune system; liver cells; autoimmune hepatitis; primary biliary cholangitis
Progressive immune-mediated inflammation and eventual destruction of hepatocytes and biliary epithelial cells characterize autoimmune liver diseases (AILDs). The primary objective of this study was to conduct a literature review on AILDs. In a nutshell, autoimmune liver diseases are a group of disorders in which the body's immune system assaults the liver cells in error, causing inflammation and damage. Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis are the three most prevalent autoimmune liver diseases (PSC). AIH is a chronic disease characterized by inflammation and liver cell injury, which, if left untreated, can progress to cirrhosis and liver failure. PBC is a chronic disease that affects the small bile ducts within the liver and can contribute to cirrhosis, liver failure, and other complications. Chronic PSC, which affects the larger bile ducts, can cause liver failure and bile duct carcinoma. The precise cause of autoimmune liver diseases is unknown, but genetic and environmental factors are believed to play a role. Typically, blood tests, imaging, and liver biopsies are used for diagnosis. Medications that suppress the immune system, such as corticosteroids, immunosuppressants, or biologics, may be used in the treatment of a specific disease. In some instances, liver transplantation may be required. Autoimmune liver diseases can have a significant impact on a person's quality of life and require ongoing management and monitoring. It is essential for individuals with these conditions to work closely with their healthcare providers to manage symptoms, prevent complications, and preserve liver health overall.
Both autoimmune illness and liver disease are conditions that are closely related to one another and can have a substantial impact on an individual's quality of life as well as their overall health [19]. The liver is an important organ that is responsible for several key tasks in the body, including the process of metabolism, detoxification, and the production of proteins [3]. These functions are hindered whenever the liver is injured or diseased, which in turn leads to a wide variety of symptoms and problems. On the other side, autoimmunity is a disorder in which the immune system erroneously assaults the body's own tissues, including the liver. This can lead to serious health complications. We shall investigate the connection between autoimmunity and liver disease, as well as their respective causes, symptoms, diagnoses, and treatments, in the following article [4]
Autoimmune liver diseases (AILDs)
The term "autoimmune liver disorders" refers to a collection of ailments in which the liver is attacked by the body's immune system, resulting in inflammation and damage to the organ. Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are the three most common forms of autoimmune liver diseases (AILDs) [14]. AIH, also known as autoimmune hepatitis, is a chronic inflammatory disease of the liver that mostly affects women and has the potential to result in cirrhosis and liver failure. PBC is a chronic liver disease that worsens with time and primarily affects women. It is distinguished by the degeneration of the liver's tiny bile ducts. PBC is also known as polycystic liver disease. PSC is a chronic disease that worsens over time and can affect either men or women. It is distinguished by inflammation and scarring of the bile ducts both inside and outside of the liver [6]. The increasing immune-mediated inflammation and eventual death of hepatocytes and biliary epithelial cells that characterize autoimmune liver disorders (AILDs) set these conditions apart from other liver conditions [30]. Although overlap syndromes are possible, it most commonly refers to autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) [31]. IgG4-sclerosing cholangitis and IgG4-autoimmune hepatitis are examples of the immunoglobulin G (IgG4)-related hepatobiliary illnesses that make up a minor percentage of autoimmune liver diseases (AILDs) [32]. Patients who have damage to their livers that cannot be reversed have the option of undergoing liver transplantation, often known as LT. The indications for liver transplantation in individuals with AILDs are, with a few notable differences, comparable to those in patients with other chronic liver illnesses [33]. These autoimmune hepatobiliary illnesses account for around 24 percent of all liver transplants, which places them in the third spot on the list of most transplant centers' most prevalent liver transplant indications [34]. Although the therapeutic approach for each autoimmune liver disease must be different, the goals of treatment remain the same: to increase the recipient's chance of survival, to prevent the failure of a liver graft, and to lower the likelihood that the disease would return. In order to accomplish these objectives, there are a number of factors that need to be taken into consideration, such as determining post-LT risk factors that correlate with liver graft failure and disease recurrence, choosing the immunosuppressive regimen that is the most appropriate, and implementing additional cancer surveillance depending on the LT indication [33].
Autoimmune hepatitis (AIH)
AIH is a complicated inflammatory liver disease that has an unknown cause and is caused by a mix of immunologic, genetic, and environmental variables. The exact cause of AIH is unknown. The incidence of ischemic heart disease in adults is extremely variable around the globe, ranging from 4.2 occurrences per 100,000 people in Singapore to 42.9 cases per 100,000 people in Alaska [32]. In the United States, a population-based national survey that was conducted not long ago found an estimated prevalence of 31.2 cases per 100,000 individuals [38]. The clinical presentation might take many different forms. Individuals may exhibit no symptoms, acute symptoms (such as abrupt severe or acute liver failure), or mild symptoms that may progress to chronic manifestations of the disease. The diagnosis is challenging and requires certain test signs such as increased total immunoglobulin G levels and raised aminotransferase levels; lymphoplasmacytic interface hepatitis on histology; and the presence of autoantibodies in the serum (antinuclear antibodies, anti-smooth muscle antibody, and anti-liver kidney microsomal antibodies). It is important to rule out other liver illnesses such as viral hepatitis, Wilson's disease, genetic liver injuries, and metabolic liver injuries [39]. Even if the AIH care guidelines are updated on a regular basis, a sizeable portion of patients who have AIH may eventually develop liver disease of the end-stage and will be in need of an LT. Five percent of all LT operations carried out in the United States are classified as AIH [33]. The patient survival rate is 76 percent after five years, whereas the transplant survival rate is 70.9 percent [40]. These rates are quite good; nevertheless, when compared to those of other LT indications, there is still a larger chance of late acute rejection (9%) and chronic rejection (16%), as well as recurrent sickness (36% to 68
Both autoimmune illness and liver disease are disorders that can affect the liver and, if left untreated, can cause persistent inflammation as well as damage to the organ. An examination of the patient's physical condition, along with blood tests, imaging scans, and possibly even a liver biopsy, are the standard diagnostic procedures. An autoimmune response can cause autoimmune liver disorders (AILDs), which include autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis, among others. Immunosuppressant drugs might be used in the treatment of AILDs. Hepatitis, which is caused by viruses, alcoholic liver disease, and nonalcoholic fatty liver disease are additional potential triggers for liver disease. The treatment for these disorders varies, but some of the options include taking antiviral medicine, quitting drinking alcohol, making changes to one's way of life, and in rare instances, getting a liver transplant. While developing an unique treatment strategy for each patient, it is frequently important to use a multidisciplinary approach and involve a group of healthcare specialists working together as a team.
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