Materiały źródłowe

• #1 Autoimmune Hepatitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459186/

  Autoimmune hepatitis refers to chronic and progressive inflammation of the liver from an unknown cause. This activity reviews the pathophysiology, causes, presentation, and diagnosis of autoimmune hepatitis and highlights the role of the interprofessional team in its management. […] Diagnosis of autoimmune hepatitis requires exclusion of other chronic causes of liver disease including Wilson disease, drug-induced hepatitis, nonalcoholic hepatosteatosis (NASH), chronic viral hepatitis, primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC. […] A multi-pronged approach is used to make a diagnosis. This approach includes determining symptoms, laboratory tests, and biopsies, as no single diagnostic test is pathognomonic for autoimmune hepatitis. […] The serologic markers required for the diagnosis of autoimmune hepatitis include antinuclear antibody (ANA), smooth muscle antibodies (SMA), and antibodies to liver-kidney microsome type 1 (anti-LKM1).

• #2 Management of Autoimmune Hepatitis | AASLD

  https://www.aasld.org/practice-guidelines/management-autoimmune-hepatitis

  Autoimmune Hepatitis (AIH) is an immune-mediated inflammatory liver disease of uncertain cause which affects all ages, both genders, and all ethnicities. […] The diagnosis must be considered in all patients with acute or chronic liver inflammation, including patients with graft dysfunction after LT. […] AIH does not have a signature diagnostic feature, and the diagnosis requires the presence of a constellation of typical features which can vary between patients with the same disease and can occur in other liver diseases.

• #3 Diagnosis of Autoimmune Hepatitis – NIDDK

  https://www.niddk.nih.gov/health-information/liver-disease/autoimmune-hepatitis/diagnosis

  Doctors diagnose autoimmune hepatitis based on a combination of information from your medical history, a physical exam, and tests. […] Your doctor may order blood tests, imaging tests, and a liver biopsy to diagnose autoimmune hepatitis. No single test can diagnose autoimmune hepatitis. In most cases, doctors order a combination of tests, including a liver biopsy, to make a diagnosis. […] Doctors order antibody tests to check for autoantibodies that attack your healthy tissues and cells by mistake such as antinuclear antibody (ANA) and anti-smooth muscle antibody (SMA). […] A doctor can use a liver biopsy to look for the features of autoimmune hepatitis and to check the amount of scarring to find out if you have cirrhosis.

• #4 Autoimmune Hepatitis| Cleveland Clinic

  https://my.clevelandclinic.org/departments/digestive/medical-professionals/hepatology/autoimmune-hepatitis

  No single test is diagnostic for AIH. The diagnosis of AIH is based on a combination of characteristic clinical features and typical laboratory abnormalities. Other causes of chronic hepatitis should be excluded, including alcohol induced hepatitis, drug-induced hepatitis, and viral hepatitis. […] In the majority of cases, AIH diagnosis can be made by using Table 2. When patients do not meet all the criteria in Table 2 or have atypical features, the diagnosis of AIH becomes less certain and they should be referred to a gastroenterologist or hepatologist. […] Autoimmune hepatitis is diagnosed when all of the above diagnostic criteria are met. Liver biopsy under these circumstances can be relevant for assessing severity of AIH. If some of the criteria above are not met, diagnosis of AIH is less certain and patients should be referred to a gastroenterologist or hepatologist before starting treatment.

• #4 Autoimmune Hepatitis| Cleveland Clinic

  https://my.clevelandclinic.org/departments/digestive/medical-professionals/hepatology/autoimmune-hepatitis

  The major laboratory abnormalities encountered in AIH are elevation of liver transaminases and gamma globulins and the presence of autoantibodies. […] Liver biopsy should be considered for the diagnosis of AIH and it can provide valuable information regarding severity of the disease. The American Association for Study of Liver Diseases recommends a liver biopsy for diagnostic purposes. The hallmark of AIH on histology is interface hepatitis characterized by lymphocytic infiltrate in the portal triad that goes beyond the limiting plate and reaches the bordering hepatocytes. […] There is no role for routine imaging in the diagnosis of AIH. Because patients with inflammatory bowel disease (IBD) and AIH have high rates of PSC, they should have a cholangiographic study once AIH is diagnosed to rule out PSC.

• #5 Autoimmune Hepatitis: A Diagnostic and Therapeutic Overview

  https://www.mdpi.com/2075-4418/14/4/382

  Unfortunately, there is no pathognomonic marker of AIH, and its diagnosis requires the exclusion of other liver diseases that may resemble it. […] The typical biochemical profile in AIH is predominantly a hepatocellular pattern, with bilirubin and aminotransferases concentrations ranging from just above ULN to >50 times these levels. […] Autoantibodies are the hallmark of AIH and play a major role in diagnosis. […] A liver biopsy with a compatible histologic finding of AIH is required to establish diagnosis and should be performed before treatment is started (unless contraindicated). […] The International Autoimmune Hepatitis Group (IAIHG) created a diagnostic scoring system that was later revised to aid in the diagnosis of AIH. […] The revised scoring system exhibits a greater sensitivity for AIH when compared to the simplified scoring system (100% versus 95%), while the simplified version has greater specificity (90% versus 73%) and accuracy (92% versus 82%).

• #5 Autoimmune Hepatitis: A Diagnostic and Therapeutic Overview

  https://www.mdpi.com/2075-4418/14/4/382

  Autoimmune hepatitis is an immune-mediated inflammatory condition of the liver of undetermined cause that affects both sexes, all ages, races, and ethnicities. The diagnosis is based on histological abnormalities (interface hepatitis), characteristic clinical and laboratory findings (increased aspartate aminotransferase, alanine aminotransferase, and serum IgG concentration), and the presence of one or more characteristic autoantibodies. […] An adequate and timely diagnosis allows an early start of immunosuppression, thus preventing the progression of liver injury. […] The diagnosis of AIH remains to this day a challenging task, considering the heterogenous clinical manifestation and broad age of presentation. […] The diagnosis of AIH can be established when characteristic histologic abnormalities (interface hepatitis), clinical and laboratory findings (elevated AST, ALT, and serum IgG) are present, along with one or more circulating antibodies.

• #6 How Autoimmune Hepatitis Is Diagnosed

  https://www.verywellhealth.com/how-autoimmune-hepatitis-is-diagnosed-5195513

  Autoimmune hepatitis is an autoimmune disease that occurs when the body’s immune system attacks the liver by mistake. The exact cause is unknown but believed to be due to a combination of genetic and environmental factors. […] There is no one test to diagnose autoimmune hepatitis. The diagnostic process usually involves a physical exam, detailed history, lab tests, imaging studies, and a liver biopsy. […] Once your doctor has performed a physical exam and asked for a detailed health history, they will likely order blood tests. These tests can tell you if your liver is inflamed or has been damaged. […] Blood tests used to diagnose autoimmune hepatitis include: Liver enzymes: Elevated levels of alanine aminotransferase (ALT) and aspartate aminotransferase (AST) indicate inflammation in the liver. Liver function tests: Levels of bilirubin, clotting factors (INR), and albumin can indicate if there is compromise to the function of your liver. Anti-nuclear antibody (ANA): A positive ANA may be seen, but is not enough for diagnosis of AIH, and there can be false positives. Serum IgG and other gamma globulins: Elevated levels without the presence of cirrhosis usually indicate autoimmune hepatitis. Anti-liver kidney microsome type 1 antibody (anti LKM-1): Elevated levels can indicate autoimmune hepatitis. Anti-smooth muscle antibody (SMA): Elevated levels can indicate autoimmune hepatitis.

• #6 How Autoimmune Hepatitis Is Diagnosed

  https://www.verywellhealth.com/how-autoimmune-hepatitis-is-diagnosed-5195513

  If any of your blood tests come back abnormal, the next step may be to order imaging tests to visualize your liver. To view your liver, your doctor may recommend a CT scan, ultrasound, or magnetic resonance imaging (MRI). […] If your doctor strongly suspects that you have autoimmune hepatitis, the last step in the diagnostic process is to obtain a liver biopsy. The doctor will remove small tissue samples from your liver with a needle. These samples are then sent to the lab to be studied under a microscope. This test can help your medical team determine which kind of liver disease you have. They can also determine if you have any evidence of long-term damage or fibrosis in the liver. […] There is no one test to diagnose autoimmune hepatitis. Your doctor will have to rule out other conditions in a process of elimination to arrive at the accurate diagnosis. They will order liver enzymes and liver function tests to see whether there is inflammation in your liver. They will also order tests that detect certain antibodies that have been associated with autoimmune hepatitis. If your doctor thinks you may have autoimmune hepatitis, they will also order imaging and a liver biopsy to better understand your condition.

• #7 Can blood tests diagnose autoimmune hepatitis?

  https://www.medicalnewstoday.com/articles/autoimmune-hepatitis-blood-test

  Blood tests can help with the diagnosis of autoimmune hepatitis. These include tests for increased liver enzymes or antibody levels. However, other tests are necessary to confirm the diagnosis. […] An accurate diagnosis can guide suitable treatment. To help diagnose the condition, a doctor will likely recommend blood tests. However, other tests are necessary to rule out differential diagnoses, such as other conditions that may affect the liver. […] Doctors administer several blood tests to support an autoimmune hepatitis diagnosis. They refer to these series of blood tests as liver function tests. These help assess whether the liver is functioning correctly and can help distinguish between a variety of liver diseases. […] Elevated liver enzyme levels can suggest inflammation or damage to the liver. For example, if a person has high levels of alanine aminotransferase (ALT) and aspartate aminotransferase (AST), this can indicate autoimmune hepatitis.

• #8 Autoimmune Hepatitis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/172356-overview

  Autoimmune hepatitis is a chronic disease of unknown cause. It is characterized by continuing hepatocellular inflammation and necrosis, and it has the potential to progress to cirrhosis. […] Laboratory findings in autoimmune hepatitis include the following: Elevated serum aminotransferase levels (1.5-50 times reference values), Elevated serum immunoglobulin (Ig) levels, primarily IgG, Mild to moderately elevated serum bilirubin and alkaline phosphatase: Marked hyperbilirubinemia can be seen in patients with acute severe autoimmune hepatitis, Positive tests for antinuclear antibodies (ANAs) and anti-smooth-muscle antibodies (ASMAs): Tests for antibodies to liver-kidney microsome type 1 (anti-LKM-1), soluble liver antigen (anti-SLA), and liver cytosol type 1 (anti-LC1) may be helpful if ANA and ASMA test results are negative.

• #8 Autoimmune Hepatitis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/172356-overview

  Liver biopsy remains an important part of the diagnostic workup of patients with suspected autoimmune hepatitis. […] Clinicians must consider the diagnosis of autoimmune hepatitis in any patient who has acute hepatitis or acute liver failure (defined by the new onset of coagulopathy and hepatic encephalopathy). The workup of such patients should include testing for serum autoantibodies, serum protein electrophoresis, and quantitative immunoglobulins. Urgent liver biopsy, transjugular if appropriate, may help to confirm the clinical suspicion of acute autoimmune hepatitis. […] Rapid institution of treatment with high-dose corticosteroids may rescue patients whose autoimmune hepatitis ultimately would have progressed to either acute liver failure or cirrhosis.

• #9 Autoimmune Hepatitis | Choose the Right Test

  https://arupconsult.com/content/autoimmune-hepatitis

  AIH is associated primarily with hypergammaglobulinemia, which is found in approximately 85% of patients with AIH and may be detected by either IgG testing or serum protein electrophoresis. […] An initial autoantibody assessment tailored for AIH includes ANAs, SMAs, LKM type 1 (LKM1) autoantibodies, liver cytosol type 1 (anti-LC1) autoantibodies, and SLA/liver-pancreas (LP) autoantibodies. […] AIH is associated with various autoantibodies; however, most of these autoantibodies are not specific to AIH, and their presence is not necessary for an AIH diagnosis. […] Anti-SLA/LP autoantibodies are highly specific to AIH and thus have a high diagnostic value. […] The heterogeneous presentation seen in AIH creates a diagnostic challenge. The simplified International Autoimmune Hepatitis Group (IAIHG) diagnostic criteria can be used in clinical settings to help address this challenge. These criteria have a reported sensitivity of 80% and a reported specificity of 95% (with a cutoff of 7 points).

• #10 Liver Function Tests for Autoimmune Hepatitis | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/liver-kidneys-and-urinary-system/autoimmune-hepatitis/diagnosis/liver-function-tests.html

  A series of special blood tests can often determine whether or not the liver is functioning properly. These tests can also distinguish between acute and chronic liver disorders and between hepatitis and cholestasis. […] The most commonly performed blood tests include the following: […] Serum albumin test: This test is used to measure the level of albumin (a protein in the blood) and aides in the diagnosis of liver disease. […] Alanine transaminase (ALT) test: This test measures the level of alanine aminotransferase (an enzyme found predominantly in the liver) that is released into the bloodstream after acute liver cell damage. This test may be performed to assess liver function, and/or to evaluate treatment of acute liver disease, such as hepatitis. […] Mitochondrial antibodies test: The presence of these antibodies can indicate primary biliary cirrhosis, chronic active hepatitis, and certain other autoimmune disorders.

• #11 Recent advances in the diagnosis and management of autoimmune hepatitis – Polish Archives of Internal Medicine

  https://www.mp.pl/paim/issue/article/16334/

  In 2008, the IAIHG simplified the diagnostic criteria by issuing a scoring system comprising only 4 parameters (liver histology, autoantibody titers, serum levels of -globulin or IgG, and absence of viral hepatitis). The score was found to bear 97% specificity and 88% sensitivity for diagnosing probable AIH and 99% specificity and 81% sensitivity for definite AIH. […] The need for identifying new biomarkers for the diagnosis of AIH is of particular importance. A meta-analysis showed that ANA have moderate specificity (62%) and sensitivity (65%), while SMAs offer high specificity (93%) but moderate sensitivity (59%). On the other hand, anti-SLA/LP exhibit very low sensitivity (19%) but the highest specificity (99%). […] The therapeutic aim is to achieve remission of the disease at the histological level with as few drug-induced complications as possible. Biochemical remission usually occurs 6 to 12 months earlier than the histological remission and its maintenance prevents further progression of liver disease.

• #11 Recent advances in the diagnosis and management of autoimmune hepatitis – Polish Archives of Internal Medicine

  https://www.mp.pl/paim/issue/article/16334/

  Detection of autoantibodies is essential not only for the diagnosis but also for the classification of AIH. Patients with AIH type 1 (AIH-1) are positive for antinuclear autoantibodies (ANA) and / or smooth muscle autoantibodies (SMA). Adults with a titer of at least 1:40 and children with a titer of at least 1:20 on indirect immunofluorescence (IIF) are considered positive. Individuals with AIH type 2 (AIH-2) are positive for antiliver kidney microsomal type 1 (anti-LKM1) or, rarely, for antiliver kidney microsomal type 3 (anti-LKM3), and / or antiliver cytosol type 1 (anti-LC1) autoantibodies. The presence of soluble liver antigens / liver pancreas antibodies (anti-SLA/LP) was formerly considered a third category of AIH. However, such a classification was abandoned when it became clear that the characteristics of AIH patients positive for anti-SLA/LP do not differ from those of individuals with AIH-1. Anti-SLA/LP levels should be tested by an enzyme-linked immunosorbent assay (ELISA) or immunoblotting, and they are detected in 20% to 30% of AIH cases. These antibodies are highly specific for AIH and the discovery of their presence is particularly helpful in patients who are seronegative for the conventional antibodies.

• #11 Recent advances in the diagnosis and management of autoimmune hepatitis – Polish Archives of Internal Medicine

  https://www.mp.pl/paim/issue/article/16334/

  Autoimmune hepatitis (AIH) is diagnosed using a combination of clinical, serological, biochemical, and histological indicators. Exclusion of any other cause of liver disease (viral hepatitis A to E, drug-induced hepatitis, alcoholic liver disease, primary sclerosing cholangitis, primary biliary cholangitis, variant forms of AIH, Wilsons disease, nonalcoholic fatty liver disease, -1 antitrypsin deficiency, hemochromatosis, and celiac disease) is obligatory. A liver biopsy is necessary as part of the diagnostic process, provided there are no contraindications. […] The clinical presentation of AIH in adults is highly heterogeneous. The most prevalent clinical phenotype in about two-thirds of the patients is marked by an insidious onset, either totally asymptomatic or with the presence of nonspecific symptoms (malaise, fatigue, anorexia, arthralgias, weight loss, etc). Therefore, it is critical to include AIH in the differential diagnosis in all individuals with transaminasemia of any severity, regardless of their ethnicity, sex, or age. Although an increased IgG level is characteristic of AIH patients, about one-tenth have a normal IgG concentration at presentation. About one-third of the patients present with acute disease, either with acute exacerbation of chronic AIH or with genuine acute AIH with no histological evidence of chronic liver disease. Due to the fact that liver autoimmune serology tests may be negative and IgG levels can be normal in such cases of acute presentation, clinicians may overlook AIH, although detailed testing for autoantibodies in specialist laboratories may be helpful. Unfortunately, owing to a delay in the diagnosis, one-third of the patients are already cirrhotic at presentation.

• #11 Recent advances in the diagnosis and management of autoimmune hepatitis – Polish Archives of Internal Medicine

  https://www.mp.pl/paim/issue/article/16334/

  Every patient with suspected AIH should have a liver biopsy performed unless there is a contraindication. Apart from facilitating the diagnosis, liver biopsy is essential to the diagnostic scoring systems. According to the simplified criteria for AIH diagnosis, the presence of 3 histological characteristics is essential for defining a case as typical: (1) interface hepatitis (inflammation of hepatocytes at the portal-lobular interface with lymphoplasmacytic cells spreading into the lobule), (2) emperipolesis (presence of a plasma cell or lymphocyte inside the cytoplasm of hepatocytes), and (3) rosettes (a small group of hepatocytes arranged around a small central lumen). Recent studies, however, raised doubts about the usefulness of hepatocyte rosettes and emperipolesis as typical AIH characteristics as both findings were suggested to be more indicative of liver cell damage.

• #12 Final Diagnosis — Case 554

  https://path.upmc.edu/cases/case554/dx.html

  The anti-LKM-1 antibody is a serologic marker of Type 2 AIH. […] Anti-soluble liver antigen (anti-SLA) antibodies can be detected in 10-50% of Type 1 AIH patients. […] In cases when serology for anti-SMA, ANA, and anti-LKM is negative anti-SLA can help to establish the diagnosis. […] The hepatic „overlap syndromes” are entities where histological and serological parameters show an intermediate distribution between AIH and primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). […] The majority of AIH patients show excellent response to immunosuppressive therapy with corticosteroids. […] The preferred treatment for AIH is prednisone in combination with azathioprine. […] Therapeutic success in AIH is marked by the disappearance of serum ANA and SMA antibodies, as well as by resolution of clinical and histological findings. […] In summary, autoimmune hepatitis is a relatively uncommon disorder associated with chronic hepatic inflammation. Occurring in patients with a well characterized genetic background, the diagnosis of AIH is greatly facilitated by the international AIH scoring system.

• #12 Final Diagnosis — Case 554

  https://path.upmc.edu/cases/case554/dx.html

  Moreover, liver biopsy continues to remain the „gold standard” for grading and staging disease activity. […] The diagnosis of AIH primarily relies on the scoring system which was established by the International Autoimmune Hepatitis Group in 1993, and was revised in 1999. […] For definitive diagnosis of AIH a cumulative score greater than 15 in pre-treatment and greater than 17 in post-treatment patients is required. […] The scoring system is highly sensitive (89%) in detecting AIH patients. […] Since the introduction of the revised criteria, the system can better differentiate between cholestatic and hepatocytic disorders, and therefore its specificity (89.5%) has also significantly improved. […] Based on the differences in clinical, serological and genetic characteristics AIH can be divided into at least two major subtypes.

• #13 Autoimmune hepatitis – Wikipedia

  https://en.wikipedia.org/wiki/Autoimmune_hepatitis

  The diagnosis of autoimmune hepatitis is best achieved with a combination of clinical, laboratory, and histological findings after excluding other etiological factors (e.g. viral, hereditary, metabolic, cholestatic, and drug-induced liver diseases). The requirement for histological examination necessitates a liver biopsy, typically performed with a needle by the percutaneous route, to provide liver tissue. […] A number of specific antibodies found in the blood (antinuclear antibody (ANA), anti-smooth muscle antibody (SMA), anti-liver kidney microsomal antibodies (LKM-1, LKM-2, LKM-3), anti soluble liver antigen (SLA), liverpancreas antigen (LP), and anti-mitochondrial antibody (AMA)) are of use, as is finding an increased immunoglobulin G level. The presence of anti-mitochondrial antibody is more suggestive of primary biliary cholangitis. Hypergammaglobulinemia is also of diagnostic value.

• #14 Back to Basics: ANA-lyzing Autoimmune Hepatitis | AASLD

  https://www.aasld.org/liver-fellow-network/core-series/back-basics/back-basics-ana-lyzing-autoimmune-hepatitis

  Autoimmune hepatitis (AIH) is a chronic, progressive immune-mediated inflammatory liver disease. […] AIH is divided into subtypes based on patterns of specific autoantibodies with type 1 (approximately 96% of US adults with AIH) and type 2 AIH as outlined in Table 1. […] An elevation in liver enzymes is usually the first signal to suspect AIH. […] For patients in whom AIH is suspected due to elevated serum aminotransferase levels and/or elevated serum IgG, AASLD guidelines suggest first excluding other competing causes of liver disease. […] If AIH is still suspected, anti-nuclear antibody (ANA) and anti-smooth muscle antibody (ASMA) should be assessed in adults. […] If positive, liver biopsy confirms the diagnosis with characteristic histologic findings of interface hepatitis with plasma cell infiltration. […] Though the laboratory workup can be highly suggestive of AIH, liver biopsy is required to confirm the diagnosis based on the above criteria. […] The diagnosis of AIH is made through laboratory and histologic evaluation as outlined in the Simplified Diagnostic Criteria for AIH.

• #15 Autoimmune hepatitis, is it difficult to diagnose? | Clinic FEHV

  https://fehv.org/en/autoimmune-hepatitis-is-it-difficult-to-diagnose/

  Autoimmune hepatitis is diagnosed by the presence of autoantibodies in the patients blood (anti-smooth muscle, antinuclear, anti-LKM, etc.) and there is also usually an increase in IgG immunoglobulin. However, in around 5% of cases of autoimmune hepatitis, no autoantibodies or increased IgG levels are detected and the disease is very difficult to diagnose. […] In this sense, Dr. Carreo and his team at the Foundation take into account all these disease onset profiles to diagnose autoimmune hepatitis. They also always recommend doing a liver biopsy to confirm the diagnosis.

• #16 Autoimmune Hepatitis Workup: Approach Considerations, Autoantibody Assays, Serum Proteins and Immunoglobulins

  https://emedicine.medscape.com/article/172356-workup

  Clinicians must consider the diagnosis of autoimmune hepatitis in any patient who presents with abnormal liver chemistries, acute hepatitis, or acute liver failure (defined by the new onset of coagulopathy and hepatic encephalopathy). […] While serologic tests may help the clinician make a correct diagnosis of autoimmune hepatitis, it should be recalled that liver biopsy plays a pivotal role in the evaluation. The 2019 American Association for the Study of Liver Diseases (AASLD) practice guideline states: The diagnosis of autoimmune hepatitis cannot be made without liver biopsy and compatible histologic findings. […] Liver biopsy is the most important diagnostic procedure in patients with autoimmune hepatitis. This procedure can be performed percutaneously, with or without ultrasonographic guidance, or by the transjugular route.

• #16 Autoimmune Hepatitis Workup: Approach Considerations, Autoantibody Assays, Serum Proteins and Immunoglobulins

  https://emedicine.medscape.com/article/172356-workup

  Liver biopsy should be performed as early as possible in patients with acute hepatitis who are thought to have autoimmune hepatitis. Confirmation of the diagnosis enables initiation of treatment at an early stage in the disease process. […] Histopathologic findings on liver biopsy specimens are crucial to determining the diagnosis of autoimmune hepatitis and the disease severity. Liver biopsy findings can help to differentiate autoimmune hepatitis from chronic hepatitis C virus (HCV) infection, alcohol-induced hepatitis, drug-induced liver disease, primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC). […] The simplified IAHIG diagnostic scoring system is as follows: If the antinuclear antibody (ANA) or smooth muscle antibody (SMA) cutoff is 1:40, assign 1 point. If the ANA or SMA cutoff is 1:80 — Or, soluble liver antigen (SLA) is positive — Or, if the liver-kidney microsome (LKM) cutoff is 1:40, assign 2 points (addition of points achieved for all autoantibodies, for a maximum of 2 points). If immunoglobulin G (IgG) is over the upper limit of normal (ULN), assign 1 point; if it is over 1.10 times the ULN, assign 2 points. If the liver histology (evidence of hepatitis is required) is compatible with autoimmune hepatitis, assign 1 point; if it is typical of autoimmune hepatitis, assign 2 points. If viral hepatitis is absent, assign 2 points. If the total score is at least 6 points, autoimmune hepatitis is probable. If it is at least 7, then autoimmune hepatitis is definite.

• #17 Autoimmune Hepatitis: Diagnosis, Imaging, and Treatment

  https://www.maxhealthcare.in/blogs/facts-about-autoimmune-hepatitis

  Imaging studies like ultrasound, or Magnetic Resonance Imaging (MRI), computed tomography (CT) may be performed to assess the liver’s structure and rule out other causes of liver disease. While these imaging techniques can provide valuable information, they are not specific to AIH and mainly serve to exclude other conditions. […] Liver biopsy remains the gold standard for confirming the diagnosis of AIH. It involves the removal of a small sample of liver tissue for microscopic examination. The biopsy helps evaluate the severity of liver inflammation, fibrosis (scarring), and the presence of characteristic histological features consistent with AIH. The biopsy can also exclude other liver diseases with similar clinical presentations. […] Once the diagnosis of AIH is established, it is important to classify the disease as type 1 or type 2 AIH, as this affects treatment strategies and prognosis.

• #18 Autoimmune Hepatitis | NewYork-Presbyterian

  https://www.nyp.org/digestive/liver-diseases/autoimmune-hepatitis/treatment

  How is Autoimmune Hepatitis Diagnosed? Diagnosis To receive an autoimmune hepatitis diagnosis, your doctor will perform a physical exam to check for any signs of the disease. They will ask after your symptoms and review your medical history medications you are currently taking, your alcohol use, and whether you have an autoimmune disease. Your physician may order certain autoimmune hepatitis tests, including: Autoimmune hepatitis blood tests. These blood tests can evaluate how well the liver is functioning, check for inflammation, and determine the presence of antibodies associated with the disease. Imaging tests. An ultrasound, CT scan, or an MRI can help detect cirrhosis of the liver or other signs of autoimmune hepatitis. Liver biopsy. A sample of liver tissue is extracted using a thin needle that’s passed through a small incision. The sample is sent to a lab and analyzed under a microscope. […] Early detection is essential in the fight against autoimmune hepatitis. At NewYork-Presbyterian, our doctors are experts in identifying the symptoms of autoimmune hepatitis, so you can get an accurate diagnosis and start your treatment plan.

• #19 Diagnosis | AIHA – Autoimmune Hepatitis Association

  https://aihep.org/diagnosis/

  The presence of autoantibodies can help diagnose autoimmune disorders. In the case of AIH, antinuclear antibodies (ANA) and/or smooth muscle antibodies (SMA)/anti-actin antibodies are often, but not always, present. […] A liver biopsy is commonly ordered to help establish a diagnosis of AIH and to see if there is any scarring of the liver. […] During the procedure, a small piece of liver tissue is removed, and a pathologist examines this tissue to determine if any scarring (fibrosis) is present and whether the patient has AIH. […] A FibroScan is not a reliable indication of fibrosis level when the liver has significant inflammation, which is why its not typically used when a patient is being diagnosed with AIH.

• #20 Diagnosis and management of autoimmune hepatitis | The BMJ

  https://www.bmj.com/content/380/bmj-2022-070201

  The first scoring system for the diagnosis of autoimmune hepatitis was proposed in 1993, revised in 1999, and simplified in 2008 to assist and standardize the diagnostic process. […] Autoantibodies are a hallmark of autoimmune hepatitis and have been the key components of all versions of the diagnostic scoring system, even though most of them are not disease specific. […] Liver histology is mandatory for the diagnosis of autoimmune hepatitis. […] A recent consensus proposes liver biopsy to be considered as: Likely autoimmune hepatitis if a portal lymphoplasmacytic infiltrate is present with at least one of the following two features: more than mild interface hepatitis or more than mild lobular hepatitis. […] The increased frequency of autoimmune hepatitis with acute onset highlights the problem of the differential diagnosis between classic autoimmune hepatitis and drug induced liver injury (DILI) with autoimmune features. […] Several reports have recently described acute liver injury following vaccination against SARS-CoV-2 with clinical, serologic, and histological features suggestive of autoimmune hepatitis.

• #21 Diagnosis and Management of Autoimmune Hepatitis: Current Status and Future Directions

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl15352

  The diagnostic criteria of the IAIHG require the presence of compatible laboratory (serum aspartate [AST] and alanine aminotransferase [ALT] abnormalities, hypergammaglobulinemia, and increased serum IgG level), serological (ANA, SMA or anti-LKM1 positivity) and histological findings (interface hepatitis with or without plasma cell infiltration). Diseases that can resemble autoimmune hepatitis must also be excluded by appropriate tests, and these include virus-related, drug-induced, alcoholic, hereditary (Wilson disease, hereditary hemochromatosis), metabolic (nonalcoholic fatty liver disease [NAFLD]), and immune-mediated cholestatic diseases (PBC and PSC). […] The revised original scoring system is a comprehensive template that evaluates 13 clinical categories and renders 27 possible grades. This comprehensive scoring system was originally developed as a research tool by which to ensure the homogeneity of patient populations in clinical studies. It has emerged subsequently as a template by which to ensure the systematic evaluation of patients, and it can serve as a mechanism by which to bolster clinical judgment.

• #22 Autoimmune Hepatopathies Diagnostics

  https://www.diagnostic.grifols.com/en/-/learning/autoimmune-hepatopathies-diagnostics

  A diagnosis of AIH should be considered in all individuals with raised blood liver enzyme (transaminase) levels. […] Early diagnosis is challenging because of disease heterogeneity, and lack of a precise test for all patients. […] The 5-year mortality rate for untreated AIH exceeds 50%, and the 10-year mortality rate is 90%. […] The International Autoimmune Hepatitis Group (IAIHG) established a simplified, 8-point scoring system for AIH in 2008. […] An overall score 6 indicates probable AIH, and a score 7 indicates definite AIH, in both children and adults. […] Anti-SLA/LP antibodies have the highest specificity of all autoantibodies for AIH, although present in only about 20%30% of patients, and should therefore be tested routinely by ELISA or Western blot. […] Considering AITH collectively, initial diagnosis involves the assessment of clinical symptoms, biochemical laboratory findings, and immunoserologic parameters hepatitis serology and autoantibodies.

• #23

  https://journals.lww.com/hep/fulltext/2020/08000/diagnosis_and_management_of_autoimmune_hepatitis.24.aspx

  The diagnosis of AIH is based on histological abnormalities (interface hepatitis), characteristic clinical and laboratory findings (elevated serum aspartate aminotransferase [AST] and alanine aminotransferase [ALT] levels and increased serum IgG concentration), and the presence of one or more characteristic autoantibodies. AIH lacks a signature diagnostic marker, and the diagnosis requires characteristic features and the exclusion of other diseases that may resemble it (e.g., viral hepatitis, drug-induced liver injury, Wilson’s disease, hereditary hemochromatosis). […] The diagnosis of AIH requires compatible histological findings and is further supported by the following features: (1) elevated serum aminotransaminase levels; (2) elevated serum IgG level and/or positive serological marker(s); (3) exclusion of viral, hereditary, metabolic, cholestatic, and drug-induced diseases that may resemble AIH.

• #23

  https://journals.lww.com/hep/fulltext/2020/08000/diagnosis_and_management_of_autoimmune_hepatitis.24.aspx

  Initial serological testing should include determinations of ANA and SMA in adults and ANA, SMA, and anti-LKM1 in children; consider additional autoantibody tests if warranted to secure the diagnosis. […] Diagnostically challenging cases should be reviewed by or referred to an experienced liver center prior to initiating therapy.

• #24 Autoimmune Hepatitis Diagnosis and Treatment Market to Hit $20.05 Bn by 2034

  https://www.towardshealthcare.com/insights/autoimmune-hepatitis-diagnosis-and-treatment-market-sizing

  Early diagnosis and appropriate treatment are crucial for several reasons. AIH can progress over time and lead to severe complications such as cirrhosis (scarring of the liver), liver failure, and even liver cancer if left untreated. Early detection allows for prompt initiation of treatment, which can help slow down or stop the progression of the disease, reduce inflammation, and prevent further liver damage. […] It’s essential for individuals with autoimmune hepatitis to follow their treatment plan carefully and regularly monitor their liver function with blood tests. They may also need ongoing medical care and regular follow-up appointments with a hepatologist (liver specialist) or gastroenterologist to ensure the disease remains under control and to address any complications or side effects of treatment.

• #25 Autoimmune Hepatitis: What It Is, Symptoms, Stages & Treatment

  https://my.clevelandclinic.org/health/diseases/17867-autoimmune-hepatitis

  The standard treatment is to begin with a high dose of corticosteroids to calm the inflammation and suppress the autoimmune response, then slowly taper off. […] Prednisone is the most commonly prescribed and the most studied medication for autoimmune hepatitis. […] The goal of the medications is to make the disease go into remission. […] The American Association for the Study of Liver Diseases recommends staying on steroids for at least three years before discontinuing. […] When you’ve been in remission for at least two years, they’ll consider discontinuing the immunosuppressants. […] Most people (80%) who discontinue their medications will eventually have a relapse and need to start them again. […] Without treatment, life expectancy is 50% within five years. But with treatment, life expectancy is 90% in 10 years and 70% in 20. […] Your healthcare provider will need to continue to monitor your liver for the rest of your life. […] If you do begin to have symptoms again, don’t hesitate to contact your healthcare provider.

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