Materiały źródłowe

• #1 Autoimmune hepatitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-hepatitis/diagnosis-treatment/drc-20352158

  The goal of treatment for autoimmune hepatitis is to slow or stop the immune system attack on the liver. This may help increase the time before the disease gets worse. To meet this goal, you’ll likely need medicines that lower immune system activity. The first treatment is usually prednisone. A second medicine, azathioprine (Azasan, Imuran), may be recommended in addition to prednisone. […] Prednisone, especially when taken long term, can cause a wide range of serious side effects, including diabetes, weakened or broken bones, high blood pressure, cataracts, glaucoma, and weight gain. […] Healthcare professionals typically prescribe prednisone at a high dose for about the first month of treatment. Then, to reduce the risk of side effects, they gradually reduce the dose over the next several months until reaching the lowest possible dose that controls the disease. Adding azathioprine also helps you avoid prednisone side effects.

• #2 Treatment for Autoimmune Hepatitis – NIDDK

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

  Doctors treat autoimmune hepatitis with medicines that suppress or decrease the activity of your immune system, reducing your immune system’s attack on your liver. The medicines doctors most often prescribe are corticosteroids, also called steroids, and immunosuppressants. […] Treatment can relieve symptoms and prevent or reverse liver damage in many people with autoimmune hepatitis. Early treatment of autoimmune hepatitis can lower the chances of developing cirrhosis and other complications. […] With treatment, you may experience remission. Remission is a period when you don’t have any symptoms and your test results show that your liver is working better and is no longer being damaged. […] If you relapse, your doctor will restart or adjust your medicines to treat autoimmune hepatitis.

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

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

  Autoimmune hepatitis causes chronic inflammation in your liver, the same way other types of hepatitis do. But instead of a virus or alcohol damaging your liver, its your own immune system attacking it. This type of disease has no cure, but you can manage it with medication. You may even go into remission. […] Medical treatment can help reduce the inflammation and prevent complications from developing. […] 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. It works well for most people, but it can have side effects. Alternatives, such as budesonide, seem to have fewer. […] Your healthcare provider may prescribe an immunosuppressant called azathioprine along with steroids, or they may prescribe it after you’ve finished your course of steroid therapy. Since azathioprine has fewer side effects than steroids, it works better for long-term maintenance therapy, to keep your immune system from becoming overactive again. You may need to take it on and off for life.

• #4 Autoimmune Hepatitis | Causes, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/a/autoimmune-hepatitis

  With the right treatment, autoimmune hepatitis usually can be controlled. Recent studies show that continued treatment not only stops the disease from getting worse, but it may reverse some of the damage. […] Medicine helps to slow down an overactive immune system. Both type 1 and type 2 autoimmune hepatitis are treated with daily doses of a steroid called prednisone. […] Patients who have no liver scarring and a mild case of autoimmune disease may be given budesonide. This drug is a form of steroid. It does not have the same level of side effects as prednisone. […] Another medication, azathioprine, is also used to treat this disease. Like prednisone and budesonide, azathioprine slows down the immune system, but in a different way. […] Treatment with azathioprine helps lower the dose of prednisone needed, thereby reducing steroid side effects. Your doctor may prescribe azathioprine in addition to prednisone once the disease is under control.

• #5 Autoimmune Hepatitis: Treatment Options and Management Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8207545/

  Autoimmune hepatitis (AIH) is an inflammatory pathology of the liver which leads to liver cirrhosis and death if left untreated and affects a large population across the world with no ethnic discrimination. […] The management of AIH is still a crucial concern and after 50 years of efforts and trials, AHI is still one of the significant challenges of diagnosis and treatment. However, it was the first hepatic pathology for which treatment intervention of corticosteroid was strongly exhibited in clinical control trials. […] The initial therapy for AIH was given by Prednisolone 0.5 and 1 mg/kg/day and added azathioprine after two weeks of Prednisolone. The treatment should be adjusted according to the advancement of the disease and only remission patients required close follow-up of three to six months; treatment may not be required in this case. Early treatment by intravenous corticosteroids (1 mg/kg) in acute severe AIH patients is a recommended choice, in case of no improvement within seven days of treatment patient should be referred for an emergency liver transplant. An increase in the dose of prednisolone and azathioprine or alternative therapeutics should be used in case of the sub-optimal outcome.

• #6 Autoimmune Hepatitis Treatment & Management: Approach Considerations, Initial Therapy for Adults and Children, Treatment Endpoints

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

  The American Association for the Study of Liver Diseases (AASLD) published detailed guidelines regarding the management of autoimmune hepatitis in 2010 and 2019. […] The 2010 AASLD guideline delineated absolute and relative indications for immunosuppressive treatment. […] Note that many patients with cirrhosis and active autoimmune hepatitis respond well to immunosuppression. […] The 2010 AASLD guideline also described the following contraindications to treatment: […] In addition, treatment might not be appropriate in patients with decompensated liver disease (eg, a patient with massive ascites or severe hepatic encephalopathy). Such individuals might be better served by liver transplantation. […] The 2010 AASLD guideline also recommended initial treatment strategies for adults as shown in Table 3, below.

• #7 Treatment for autoimmune hepatitis – British Liver Trust

  https://britishlivertrust.org.uk/information-and-support/liver-conditions/autoimmune-hepatitis/treatment/

  Most people with autoimmune hepatitis need treatment. […] This is called immunosuppression and uses a combination of medicines: corticosteroids and another immunosuppressant. […] Immunosuppression reduces how active your immune system is. This stops it attacking your liver and reduces the inflammation. […] The main aim of treatment is to improve your symptoms and your blood tests. It will also prevent or reduce scarring and long-term liver damage and failure. […] You are likely to take medicines for at least two years. […] For many people this could be for life. It might be possible to stop treatment completely, but some people can become ill again (relapse) and need further treatment. […] If your liver is very badly damaged, a liver transplant could be life-saving. It is usually only recommended if other treatments are no longer helpful, and your life is threatened by end stage liver disease.

• #8 Autoimmune Hepatitis | Causes, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/a/autoimmune-hepatitis

  Most people with autoimmune hepatitis will need to take prednisone, with or without azathioprine, for years. Some people take it for life. These steroids may slow down the disease, but everyone is different. […] In about one out of every four people, treatment eventually can be stopped. However, it is important to carefully watch your child’s condition and report any new symptoms to the doctor. The disease may return and be even more severe, especially during the first few months after stopping treatment. […] People who progress to end stage liver disease (liver failure) may need a liver transplant. The outcome for patients with autoimmune hepatitis is excellent. Survival rates at transplant centers for this condition are well over 90%, with a good quality of life after recovery. […] The outlook for children with autoimmune hepatitis is generally favorable. In about seven out of 10 people, the disease goes into remission, with symptoms becoming less severe within two years of starting treatment. […] However, some people whose disease goes into remission will see it return within three years, so treatment may be necessary on and off for years, if not for life.

• #9 Autoimmune Hepatitis | Causes, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/a/autoimmune-hepatitis

  With the right treatment, autoimmune hepatitis usually can be controlled. Recent studies show that continued treatment not only stops the disease from getting worse, but it may reverse some of the damage. […] Medicine helps to slow down an overactive immune system. Both type 1 and type 2 autoimmune hepatitis are treated with daily doses of a steroid called prednisone. […] Patients who have no liver scarring and a mild case of autoimmune disease may be given budesonide. This drug is a form of steroid. It does not have the same level of side effects as prednisone. […] Another medication, azathioprine, is also used to treat this disease. Like prednisone and budesonide, azathioprine slows down the immune system, but in a different way. […] Treatment with azathioprine helps lower the dose of prednisone needed, thereby reducing steroid side effects. Your doctor may prescribe azathioprine in addition to prednisone once the disease is under control.

• #10 Patient education: Autoimmune hepatitis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/autoimmune-hepatitis-beyond-the-basics/print

  Budesonide is a glucocorticoid that has more liver-specific effects and is associated with fewer side effects. It is an alternative to prednisone in selected situations. It should only be used in people who have no evidence of cirrhosis. […] Azathioprine or 6-mercaptopurine – A second medication, such as azathioprine (brand names: Azasan, Imuran) or 6-mercaptopurine (brand names: Purixan, Purinethol) and, less commonly, methotrexate or mycophenolate mofetil, may be recommended in addition to prednisone. The benefit of adding a second medication is that it may be possible to reduce or eliminate prednisone, helping to minimize the potential side effects of prednisone. […] Azathioprine and 6-mercaptopurine can also cause side effects, including allergic reactions, a low white blood cell count, inflammation of the pancreas, nausea, and abnormal liver blood tests (which can sometimes cause confusion as to whether the abnormal results are from the AIH or the drugs used to treat it). There may be a small increased risk of certain types of cancer (such as lymphoma). Blood tests to monitor for these conditions are performed regularly while taking these medications.

• #11 Current and Emerging Treatments for Autoimmune Hepatitis – Gastroenterology & Hepatology

  https://www.gastroenterologyandhepatology.net/archives/march-2024/current-and-emerging-treatments-for-autoimmune-hepatitis/

  Autoimmune hepatitis (AIH) is a composite of diseases marked by immune dysregulation targeting hepatocytes in the absence of infection. […] The current therapies tend to be broad-based immunosuppressants that impair or inhibit T-cell activation. For decades, the first-line AIH treatment was a systemic corticosteroid plus azathioprine. […] In 2010, a landmark study demonstrated that budesonide, a corticosteroid with significant first-pass metabolism in the liver, could be used instead of conventional systemic corticosteroids in patients without cirrhosis. […] What are typically used now are corticosteroids with either mycophenolate mofetil or azathioprine, and those regimens tend to be effective in many patients. […] In December 2023, results from the CAMARO study were released online, which I consider to be the most recent major breakthrough in AIH.

• #12 Advancements in autoimmune hepatitis management: Perspectives for future guidelines

  https://www.wjgnet.com/1948-5182/full/v16/i2/135.htm

  The first-line treatment for autoimmune hepatitis involves the use of prednisone or prednisolone either as monotherapy or in combination with azathioprine (AZA). […] Budesonide has shown promise in inducing a complete biochemical response (CBR) with fewer adverse effects and is considered an optional first-line treatment, particularly for patients without cirrhosis; however, it is worth noting that the design of that study favored budesonide. […] Current guidelines recommend mycophenolate mofetil (MMF) for patients who are intolerant to AZA. […] Nevertheless, MMF has demonstrated superiority to AZA in the context of renal transplant. […] Recent comparative studies have shown higher CBR rates, lower therapeutic failure rates, and reduced intolerance in the MMF group. […] Until recently, the only alternative to corticosteroids was lifelong maintenance treatment with AZA, which comes with notable risks, such as skin cancer and lymphoma.

• #13 Autoimmune Hepatitis | UCSF Department of Surgery

  https://hpbsurgery.ucsf.edu/condition/autoimmune-hepatitis

  A health care provider may gradually reduce the dose of medication in people who show improvement, although the symptoms can return. […] People who do not respond to standard immune therapy or who have severe side effects from the medications may benefit from other immunosuppressive agents such as mycophenolate mofetil (CellCept), cyclosporine, or tacrolimus (Hecoria, Prograf). […] In some people, autoimmune hepatitis progresses to cirrhosis and end-stage liver failure, and a liver transplant may be necessary. […] Liver transplant is surgery to remove a diseased or an injured liver and replace it with a healthy one from another person, called a donor. […] Liver transplant surgery for autoimmune hepatitis is successful in most cases.

• #14 Autoimmune Hepatitis| Cleveland Clinic

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

  The treatment of AIH consists of 2 phases: 1) induction of remission and 2) maintenance of remission. […] The goal of treatment is to prevent liver failure and end stage liver disease. Response to treatment is classified into remission, incomplete response or treatment failure. […] Liver transplantation is an option when patients present with acute liver failure, decompensated cirrhosis with a Model for End Stage Liver Disease score 15 or hepatocellular carcinoma that meets criteria for liver transplantation. Approximately 10% to 15% of AIH patients will require liver transplantation. […] Alternative treatment options are generally used when there is intolerance or contraindications to azathioprine or when treatment failure ensues. The most commonly used alternative agents are mycophenolate mophetil or calcineurin inhibitors (cyclosporine or tacrolimus) alone or in combination with prednisone.

• #15

  https://www.xiahepublishing.com/m/2310-8819/JCTH-2024-00193

  For untreated adult AIH patients who are not experiencing an acute severe flare-up or cirrhosis, predniso(lo)ne combined with AZA or predniso(lo)ne alone has long been considered the first-line treatment. […] The Chinese guidelines for the diagnosis and treatment of autoimmune hepatitis (2021) recommend predniso(lo)ne combined with AZA as the initial first-line treatment. […] Budesonide is an effective alternative to predniso(lo)ne for induction treatment. […] Second-line treatments are indicated for patients who experience nonresponse, insufficient response, or intolerance to first-line therapy. […] MMF is the most widely used alternative immunosuppressant in patients with an inadequate response to first-line therapy. […] Calcineurin inhibitors (CNIs), including cyclosporine A (CsA) and tacrolimus (TAC), inhibit lymphocyte proliferation and interfere with T-cell-mediated responses, thereby reducing liver inflammation in AIH patients.

• #16 Autoimmune Hepatitis Management: Recent Advances and Future Prospects

  https://www.mdpi.com/2673-4389/4/2/17

  After four to eight weeks, if there is evidence of a positive biochemical response with improved liver function tests and gamma globulin levels, a gradual steroid reduction can be initiated. […] A minority of patients experience treatment failure or an incomplete response (approximately 8% and 15% of those undergoing treatment, respectively) and may require second-line options. […] If both azathioprine and 6-mercaptopurine are intolerable, mycophenolate mofetil is the preferred second-line therapy at a standard dose of 2 g/day. […] Recently, biological agents have shown promising results in refractory AIH. […] Budesonide, a synthetic steroid, has been shown to induce less systemic side effects when compared with other agents, due to its high first-pass hepatic clearance rate. […] It will be important to consider that for patients reaching AIH end-stage chronic liver disease, the AASLD guidelines recommend semi-annual ultrasounds for HCC detection for patients with compensated AIH liver cirrhosis, similar to any other cause of liver cirrhosis.

• #17 Advancing Autoimmune Hepatitis Treatment and Research, with Craig Lammert, MD

  https://www.hcplive.com/view/advancing-autoimmune-hepatitis-treatment-research-craig-lammert-md

  Results showed mycophenolate mofetil with prednisolone led to a significantly higher rate of biochemical remission at 24 weeks with fewer adverse events compared to azathioprine combined with prednisolone. […] Still, Lammert points out a significant proportion of patients fail to achieve biochemical response with currently available therapies and face long-term corticosteroid dependence. […] Anything we can do to herald in a new wave of clinical trials, and how we design those trials to not suffer from what we’ve seen in the PBC space, but also to identify agents and test them in a way to get full approval, would be tremendously impactful for autoimmune hepatitis patient populations, he said. […] In the meantime, Lammert describes efforts to optimize current standard of care and assess tools for long-term disease monitoring, including FibroScan.

• #18

  https://www.xiahepublishing.com/m/2310-8819/JCTH-2024-00193

  AZA is a purine antimetabolite with cytotoxic properties. […] Allopurinol can be an effective and relatively safe alternative immunosuppressive therapy. […] 6-TG can be a viable second-line treatment option for AZA-intolerant patients. […] MTX may have a role in AIH treatment, but its efficacy appears lower compared to other second-line therapies, and further validation is needed. […] For AIH patients who fail first- and second-line therapies, reevaluation of the diagnosis is necessary, and third-line treatments may be initiated. […] Rituximab can be effective for some AIH patients, but it can also lead to serious side effects, such as infections and hematologic or lymphatic abnormalities. […] Infliximab treatment reduced hepatic inflammation, as evidenced by lower serum transaminase and immunoglobulin levels.

• #19

  https://link.springer.com/article/10.1007/s10620-021-07378-4

  Biologic agents may satisfy an unmet clinical need for treatment of refractory autoimmune hepatitis. […] Rituximab (monoclonal antibodies against CD20 on B cells), infliximab (monoclonal antibodies against tumor necrosis factor-alpha), low-dose recombinant interleukin 2 (regulatory T cell promoter), and belimumab (monoclonal antibodies against B cell activating factor) have induced laboratory improvement in small cohorts with refractory autoimmune hepatitis. […] Ianalumab (monoclonal antibodies against the receptor for B cell activating factor) is in clinical trial. […] Fully human antibodies, expanded target options, improved targeting precision, more effective delivery systems, and biosimilar agents promise to improve efficacy, safety, and accessibility. […] In conclusion, biologic agents target key pathogenic pathways in autoimmune hepatitis, and early experiences in refractory disease encourage clarification of the preferred target, rigorous clinical trial, and comparative evaluations.

• #20 Advancements in autoimmune hepatitis management: Perspectives for future guidelines

  https://www.wjgnet.com/1948-5182/full/v16/i2/135.htm

  Single-center studies using chloroquine diphosphate have shown promising results in significantly reducing relapse rates compared to placebo. […] Additionally, second-line drugs, such as tacrolimus, rituximab, and infliximab, should be subjected to controlled trials for further evaluation. […] Autoimmune hepatitis guidelines consider corticosteroids as first-line treatment, including budesonide as an option in patients without cirrhosis. […] Azathioprine is recommended to reduce corticosteroid doses and side effects. […] Recent publications suggest that these guidelines may be outdated. […] The potential superiority of mycophenolate mofetil to azathioprine is under scrutiny. […] The suspension of treatment remains a topic of controversy in the management of AIH. […] Encouraging results have emerged from single-center studies involving the use of chloroquine diphosphate, demonstrating a significant reduction in relapse rates compared to a placebo. […] Promising therapeutic agents, such as those acting on cytokine, chemokine, and signaling pathways, cell-based therapy, microbiome modulation, or nanomedicine, are still in the early stages of research.

• #21 Autoimmune Hepatitis Treatment & Management: Approach Considerations, Initial Therapy for Adults and Children, Treatment Endpoints

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

  Treatment of overlap syndromes: […] Patients may achieve 1 of 4 treatment end points, as follows: […] Remission is defined as a combination of the following: […] Treatment withdrawal: […] Relapse occurs in 50-85% of adults and 60-80% of children after treatment withdrawal. […] Treatment failure is defined as deterioration in a patient’s clinical condition, laboratory test results, or histologic features after starting standard therapy. […] Incomplete response is defined as an improvement that is insufficient to satisfy remission criteria. […] Drug toxicity is not uncommon during treatment for autoimmune hepatitis. […] Liver transplantation is a life-saving therapy for many severely ill patients with autoimmune hepatitis. […] Patients transplanted due to autoimmune hepatitis appear to have a higher incidence of acute T-cell mediated rejection than patients transplanted for other diagnoses.

• #22 Patient education: Autoimmune hepatitis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/autoimmune-hepatitis-beyond-the-basics/print

  AUTOIMMUNE HEPATITIS TREATMENT […] Not everyone with autoimmune hepatitis (AIH) needs treatment immediately. The decision to treat is based on the severity of symptoms, the severity of the disease (based upon results of blood tests and the liver biopsy), and the potential side effects of treatment. The guidelines for treatment can be found online at the American Association for the Study of Liver Diseases (https://www.aasld.org). […] Medications — AIH is usually treated first with a glucocorticoid (steroid medication) such as prednisone; budesonide may be used in people without severe liver scarring. […] Glucocorticoids – Glucocorticoids such as prednisone control the inflammation in the liver, thereby preventing further scarring. The main drawback of prednisone is side effects, which can include weight gain, acne, bone loss, elevated blood glucose levels (potentially leading to diabetes), an increased risk of infections, cataracts, high blood pressure, and mood and sleep disturbance, among others. People who require long-term prednisone are monitored carefully for these side effects. To minimize the risks of side effects, the lowest possible dose of prednisone is used.

• #23 Autoimmune Hepatitis: Symptoms & Treatments

  https://liverfoundation.org/liver-diseases/autoimmune-liver-diseases/autoimmune-hepatitis-aih/

  Autoimmune hepatitis is a disease in which the bodys own immune system attacks the liver and causes it to become inflamed. The disease is chronic, meaning it lasts many years. If untreated, it can lead to cirrhosis and liver failure. […] The goal of treatment is to stop the bodys attack on itself by suppressing the immune system. This is accomplished with a medicine called prednisone, a type of steroid. Often times, a second drug, azathioprine (Imuran) is also used. Treatment starts with a high dose of prednisone. When symptoms improve, the dosage is lowered and azathioprine may be added. In most cases, autoimmune hepatitis can be controlled but not cured. That is why most patients will need to stay on the medicine for years, and sometimes for life. Unfortunately, long-term use of steroid can cause serious side effects including diabetes, osteoporosis, high blood pressure, glaucoma, weight gain and decreased resistance to infection. Other medications may be needed to control these side effects.

• #24 Patient education: Autoimmune hepatitis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/autoimmune-hepatitis-beyond-the-basics/print

  Budesonide is a glucocorticoid that has more liver-specific effects and is associated with fewer side effects. It is an alternative to prednisone in selected situations. It should only be used in people who have no evidence of cirrhosis. […] Azathioprine or 6-mercaptopurine – A second medication, such as azathioprine (brand names: Azasan, Imuran) or 6-mercaptopurine (brand names: Purixan, Purinethol) and, less commonly, methotrexate or mycophenolate mofetil, may be recommended in addition to prednisone. The benefit of adding a second medication is that it may be possible to reduce or eliminate prednisone, helping to minimize the potential side effects of prednisone. […] Azathioprine and 6-mercaptopurine can also cause side effects, including allergic reactions, a low white blood cell count, inflammation of the pancreas, nausea, and abnormal liver blood tests (which can sometimes cause confusion as to whether the abnormal results are from the AIH or the drugs used to treat it). There may be a small increased risk of certain types of cancer (such as lymphoma). Blood tests to monitor for these conditions are performed regularly while taking these medications.

• #25 Treatment for autoimmune hepatitis – British Liver Trust

  https://britishlivertrust.org.uk/information-and-support/liver-conditions/autoimmune-hepatitis/treatment/

  The length of treatment depends on each person. It is likely to be for several years, and longer (sometimes for life). […] This type of medicine can act slowly, so it may be up to three months before you feel any benefits. […] You are more likely to experience side effects when you first start taking azathioprine or when your dose is increased. […] If you are struggling with other side effects or they are not going away, speak to your doctor, liver specialist or specialist nurse. They can sometimes adjust your treatment to help. […] If you have liver disease you are more vulnerable to infections. And if you do get ill, you are more likely to become severely ill. It is important to have your Hepatitis A and B, flu and COVID-19 vaccinations.

• #26 Autoimmune Hepatitis Treatment & Management: Approach Considerations, Initial Therapy for Adults and Children, Treatment Endpoints

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

  After making a diagnosis of autoimmune hepatitis, it is incumbent upon the physician to educate the patient as to their risk of progressing to advanced liver disease. It is important to review the following goals of treatment with the patient: […] For more than four decades, corticosteroids, either alone or in combination with azathioprine, have been the mainstays of drug therapy for patients with autoimmune hepatitis. […] Treatment must be individualized for patients with autoimmune hepatitis. […] Patients with autoimmune hepatitis who are asymptomatic should undergo the same treatment strategy as symptomatic patients, because both groups experience a similar course of disease progression and clinical response to immunosuppressive medications. […] Azathioprine is metabolized to 6-mercaptopurine (6-MP). One of the enzymes responsible for this is thiopurine methyltransferase (TPMT).

• #27 Autoimmune Hepatitis | Choose the Right Test

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

  A liver biopsy with compatible histologic findings is required for the diagnosis of autoimmune hepatitis (AIH). […] Testing for AIH should be considered in all individuals who present with acute or chronic liver disease (including acute liver failure) or abnormal liver function tests (elevated aspartate aminotransferase [AST] and alanine aminotransferase [ALT]), particularly when hypergammaglobulinemia or features of other autoimmune disorders are present. […] Testing is also indicated in patients with AIH to monitor the disease before, during, and after treatment. […] Because azathioprine therapy can cause severe myelosuppression in those with thiopurine methyltransferase (TPMT) deficiency, phenotyping or genotyping testing should be considered to assess the risk of such complications before initiating treatment.

• #28 British Society of Gastroenterology guidelines for diagnosis and management of autoimmune hepatitis | Gut

  https://gut.bmj.com/content/early/2025/04/01/gutjnl-2024-333171

  We recommend that patients with AIH and ALF (including encephalopathy) should be referred promptly to a transplant centre). Grade of evidence: low. Strength of recommendation: strong. […] We recommend that patients with AIH and decompensated cirrhosis, and those with jaundice but with a MELD score of 27, treatment can be started on prednisolone (after a negative septic screen), but should be discussed with a transplant centre if the MELD score does not fall progressively. Grade of evidence: low. Strength of recommendation: strong. […] We recommend that confirmed normalisation of serum ALT/AST and serum IgG should be the aim of treatment. Grade of evidence: moderate. Strength of recommendation: strong. […] We recommend that response to treatment be assessed at the following time points, with inadequate response being defined as: (a) After 1 month: 50% decrease in ALT/AST (b) After 6 months: failure of normalisation of ALT/AST and IgG. Grade of evidence: moderate. Strength of recommendation: strong.

• #29 Patient education: Autoimmune hepatitis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/autoimmune-hepatitis-beyond-the-basics/print

  Duration of treatment — As a general rule, treatment is continued until the disease is in remission, the treatment fails, or the person develops severe side effects from treatment. […] Remission is defined as a lack of symptoms, normal or near normal levels of liver blood tests, and improvement in the appearance of liver tissue (based upon a biopsy). The initial period of remission generally occurs 12 or more months after treatment begins. The majority of people achieve remission by 18 months to three years of treatment. […] Approximately 50 percent of people remain in remission or have only mild disease activity for months to years after treatment is stopped. However, most people (75 to 80 percent) must eventually restart treatment because the disease becomes active again (relapse). Relapse typically occurs within the first 6 to 12 months after treatment is stopped. Relapse is more likely in those who have cirrhosis on the initial liver biopsy.

• #30

  https://www.xiahepublishing.com/m/2310-8819/JCTH-2024-00193

  Therefore, guidelines from China, Europe, and the United States all recommend initiating immunosuppressive therapy for patients with active AIH (serum aminotransferase levels 3 the upper limit of normal [ULN], IgG 1.5 ULN, and/or moderate to severe interface hepatitis). The treatment regimen and drug dosage can be adjusted based on disease activity. […] Immunosuppressive treatment should continue for at least two years after achieving complete biochemical remission, which is defined by the normalization of serum transaminases and IgG levels. […] Despite some patients maintaining remission after discontinuation, research has shown that the majority experience disease relapse after stopping medication. […] The goal of first-line treatment is to alleviate symptoms, prevent disease progression, achieve biochemical remission, and reduce complications.

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

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

  The goal of the medications is to make the disease go into remission. You may have to take them for several months to years before this happens. […] The American Association for the Study of Liver Diseases recommends staying on steroids for at least three years before discontinuing. […] No. It can go into remission. That means the inflammatory process goes away for a time, sometimes for a long time. But after you stop the treatment, it can come back. This is called relapse. […] Autoimmune hepatitis is serious, but most people respond well to treatment. The disease can go into remission and all but disappear for a long time. When it returns, the same treatment is likely to work again.

• #32 Autoimmune hepatitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-hepatitis/diagnosis-treatment/drc-20352158

  Although you may experience remission a few years after starting treatment, the disease often returns if the drug is discontinued. Depending on your situation, you may need lifelong treatment. […] When medicines don’t stop the disease from getting worse or you get scarring that can’t be reversed called cirrhosis or liver failure, the remaining option is a liver transplant. […] During a liver transplant, your diseased liver is removed and replaced with a healthy liver from a donor.

• #33 Autoimmune Hepatitis: Treatment Options and Management Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8207545/

  Immunosuppressive treatment is another approach and needs long-term management of approximately two to three years until normal levels of IgG and transaminases are achieved. […] The core treatment concern of AIH should be response-dependent and followed on an individualized basis. […] AIH treatment should be focused to settle all biochemical and histopathological markers to avoid disease development in the future. […] Liver transplantation is the last option in AIH management and about 10-20% of AIH patients pursue this option. Liver transplantation is advisable in AIH patients with decompensation presentation, severe AIH with no improvement of transaminases after therapeutic intervention, fulminant liver failure, the MELD score of 15, and patients with HCC, meeting the transplantation criteria.

• #34 Autoimmune Hepatitis Treatment & Management: Approach Considerations, Initial Therapy for Adults and Children, Treatment Endpoints

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

  Despite this, the long-term outlook after liver transplantation is excellent, with 10-year survival reported as greater than 70%. […] Patients with acute autoimmune hepatitis and symptoms of nausea and vomiting may require intravenous fluids and even total parenteral nutrition; however, most patients can tolerate a regular diet. […] Patients with cirrhosis secondary to autoimmune hepatitis may develop ascites. […] Most patients do not need hospitalization, although this may be required for clinically severe illness.

• #35 An Acute Severe Dilemma | AASLD

  https://www.aasld.org/liver-fellow-network/core-series/clinical-pearls/acute-severe-dilemma

  A 61 year old male with a history of recently diagnosed autoimmune hepatitis presents with fatigue and generalized weakness for 1.5 weeks. […] The correct answer is B, start 1 mg/kg per day of prednisolone. This patient is presenting with an acute severe autoimmune hepatitis (AIH) flare. […] The recommended treatment for an acute severe AIH flare is 0.5-1mg/kg of prednisolone per day, with close observation over the next several days to monitor for a response to the treatment. […] Azathioprine and mycophenolate mofetil are not first line treatments for an acute severe AIH flare. […] The first line therapy for AS-AIH is corticosteroids. […] The recommended medication and dose is prednisolone 0.5-1mg/kg/day. […] If there is a lack of biochemical improvement after one to two weeks of steroid therapy, or the patient develops hepatic encephalopathy, urgent liver transplant evaluation should be initiated.

• #36

  https://www.xiahepublishing.com/m/2310-8819/JCTH-2024-00193

  Belimumab may be a promising option for treating AIH patients. […] LT is indicated for AIH-related liver failure or decompensated cirrhosis. […] Post-LT AIH patients often receive low-dose corticosteroids (predniso(lo)ne) combined with immunosuppressants, as this regimen reduces AIH recurrence, lowers rejection rates, and increases graft survival while minimizing corticosteroid side effects. […] Advances in understanding the pathogenesis have led to the development of new treatments such as IL-2, PIF, and Tregs. […] In summary, the optimal treatment plan for AIH patients should be individualized to slow disease progression, reduce side effects, and improve treatment efficacy and quality of life.

• #37 Patient education: Autoimmune hepatitis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/autoimmune-hepatitis-beyond-the-basics/print

  If medications are not used — Close follow-up is recommended for people who are not initially treated with medications. Follow-up generally includes a physical examination and blood tests every few months. […] Self care — Taking medication and seeing a health care provider on a regular basis can help to ensure that the liver remains as healthy as possible. […] Diet — No specific diet has been shown to improve the outcome in people with AIH. The best advice is to eat a normal, healthy and balanced diet and to avoid becoming obese; obesity can increase the risk of fatty liver disease and may complicate AIH. […] Alcohol — Alcohol should be avoided since it can cause fatty liver and other liver damage. All types of alcoholic beverages can be harmful to the liver, including beer, wine, and liquor. People with liver disease may worsen with even small amounts of alcohol.

• #38 Autoimmune Hepatitis | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/a/autoimmune-hepatitis.html

  Treatment works best when autoimmune hepatitis is found early. The goal of treatment is to control the disease and to reduce or get rid of any symptoms (be in remission). […] To do this, medicines (corticosteroids and immune system suppressors) are used to help slow down or suppress your overactive immune system. They also stop your body from attacking your liver. […] Once you have started treatment, it can take 6 months to a few years for the disease to go into remission. Some people can stop taking medicine, but often the disease comes back. You may need treatment now and then for the rest of your life. Some people need to remain on treatment if they have relapsed many times or if their disease is severe. […] In some cases, autoimmune hepatitis may go away without taking any medicines. But for most people, autoimmune hepatitis is a chronic disease. […] If you have liver failure, a liver transplant may be needed. […] Be sure to ask your healthcare provider about recommended vaccines. These include vaccines for viruses that can cause liver disease.

• #39 Autoimmune Hepatitis: Symptoms, and Treatment | Doctor

  https://patient.info/doctor/autoimmune-hepatitis-pro

  Autoimmune hepatitis requires lifelong treatment in most patients in order to prevent development of cirrhosis and end-stage liver disease. […] The cornerstone of treatment is steroid induction therapy followed by maintenance therapy with azathioprine, which is effective in most cases. […] For patients who do not respond to standard treatment, second-line treatment with other immunosuppressants can be effective. […] Treatment should be aimed at biochemical remission of the disease, which is defined as normalisation of transaminases and immunoglobulin G. […] Patients should be monitored intensively during the first months of treatment in order to monitor side-effects, assess symptoms and individualise treatment. […] Liver transplantation is indicated for terminal phases of autoimmune hepatitis. […] Therapeutic advances have reduced the need for transplantation for AIH. […] Recurrence of AIH may occur after liver transplantation.

• #40 Patient education: Autoimmune hepatitis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/autoimmune-hepatitis-beyond-the-basics/print

  Exercise — Exercise is good for overall health and is encouraged, but it has no specific benefit for people with AIH. […] Prescription and nonprescription drugs — Many drugs are broken down by the liver. Thus, it is always best to check with a health care provider or pharmacist before starting a new prescription. Unless the liver is already scarred, most drugs are safe. Some people with active liver disease will be advised to take a smaller dose of medication. […] An important exception is acetaminophen (sample brand name: Tylenol), commonly used for headaches, other aches and pains, and fever. In people with any type of liver disease, the maximum recommended dose of acetaminophen is no more than 2000 mg (in divided doses) per 24 hours. Thus, it is reasonable to take 500 mg every four to six hours, although this should not be repeated more than four times in one day.

• #41 Patient education: Autoimmune hepatitis (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/autoimmune-hepatitis-beyond-the-basics/print

  Herbal medications — There are a number of claims, particularly on the internet, that herbal medications can improve liver health. However, no single or combination of herbs has been proven to improve outcomes in people with AIH. Some herbs can cause serious liver damage, and some have been implicated in triggering AIH. For this reason, we do not currently recommend any herbal treatment for liver disease. […] Support — Do not underestimate the value of sharing your concerns with other people with AIH. Ask your health care provider about support groups or ways to connect with other people who may be willing to discuss their experiences with AIH.

• #42 FDA Clears Autoimmune Hepatitis Treatment, Zetomipzomib, As Investigational New Drug

  https://www.contagionlive.com/view/fda-clears-autoimmune-hepatitis-treatment-zetomipzomib-as-investigational-new-drug

  The FDA cleared zetomipzomib as an Investigational New Drug. A phase 2 clinical trial is the next step for this autoimmune hepatitis treatment. […] The therapy, zetomipzomib, is a first-in-class, selective immunoproteasome inhibitor, intended for the treatment of autoimmune hepatitis (AIH). […] While there are AIH treatments currently available, these chronic corticosteroids can increase the burden of morbidity and mortality. With zetomipzomib, Kezar Life Sciences seeks to provide a treatment regimen to reduces the need for chronic immunosuppression via corticosteroids. […] Patients with autoimmune hepatitis need new therapies that can better treat their disease, said Craig Lammert, MD, an assistant professor of medicine at Indiana University and executive director of the Autoimmune Hepatitis Association. Lifelong maintenance therapy is required for most patients with AIH and an alternative regimen that reduces or removes the need for immunosuppression with corticosteroids would be welcomed by patients and the medical community.

• #43 Stem cell therapies for autoimmune hepatitis | Stem Cell Research & Therapy | Full Text

  https://stemcellres.biomedcentral.com/articles/10.1186/s13287-021-02464-w

  Autoimmune hepatitis is a chronic inflammatory hepatic disorder which may cause liver fibrosis. Appropriate treatment of autoimmune hepatitis is therefore important. Adult stem cells have been investigated as therapies for a variety of disorders in latest years. […] For chronic hepatic disorder, stem cell therapy seems to be a safe and effective treatment choice. Mesenchymal stem cells (MSCs) therapy possess numerous advantageous properties such as multipotential for differentiation, anti-fibrosis properties, and immunomodulatory effects. […] Although the use of corticosteroids is the common treatment for AIH, some patients with disease react badly to such treatment, and some recovered patients may face severe adverse effects or recurrence following discontinuing steroid use. For these reasons, novel therapies are needed.

• #44

  https://xiahepublishing.com/m/2310-8819/JCTH-2024-00193

  Belimumab may be a promising option for treating AIH patients. […] LT is indicated for AIH-related liver failure or decompensated cirrhosis. […] The human gastrointestinal tract hosts a vast microbial ecosystem, comprising trillions of microorganisms, including bacteria, fungi, and viruses, collectively known as the gut microbiota. […] A study treating AIH mice with a mix of Bifidobacterium and Lactobacillus probiotics found that these probiotics inhibited inflammatory cell differentiation, promoted the differentiation of regulatory T cells (Tregs) for immune tolerance, improved intestinal barrier function, increased the abundance of intestinal flora in AIH mice, prevented the translocation of lipopolysaccharides to the liver, and reduced the production of inflammatory cytokines, thereby promoting AIH remission.

• #45 Stem cell therapies for autoimmune hepatitis | Stem Cell Research & Therapy | Full Text

  https://stemcellres.biomedcentral.com/articles/10.1186/s13287-021-02464-w

  Hematopoietic stem cell transplantation (HSCT) has become as a viable therapy. […] HSCT is used to eliminate autoreactive immune cells and recreate a naive, self-tolerant immune system in patients with autoimmune disorders. […] Multiple sclerosis, systemic sclerosis, and rheumatoid arthritis are among the autoimmune illnesses for which HSCT is a treatment option. […] Anti-thymocyte globulin, along with either high dosage Cyc or other chemotherapeutic drugs, is the most common conditioning protocol applied in people with AIH indicated for autologous HSCT. […] Despite the important role of stem cells in immune modulation, there are a few studies investigating the effects of stem cells on AIH. […] In summary, it has been shown that MSCs provide a therapeutic effect in AIH animal models. EAH had been alleviated by the administration of BMSCs in a dose-dependent way and this was explained that BMSCs had upregulated PD-L1 and inhibited IL-17. MSCs have been genetically modified with IL-35 to increase their efficacy by preventing the hepatocytes apoptosis by lowering the expression of FasL by mononuclear cells (MNC) and decreased IFN- expression level through the JAK1-STAT1/STAT4 signal pathway. Moreover, the BMSC-derived exosomes showed a promising therapeutic effect either containing additional miRs or none by inhibition of inflammatory cytokines.

• #46 Autoimmune Hepatitis: Causes, Symptoms, and Diagnosis

  https://www.healthline.com/health/autoimmune-hepatitis

  Autoimmune hepatitis is a chronic disease. It has no cure, but can be managed, and treated into remission. Remission means the disease is not active, or presenting symptoms. […] Treatment can slow down, stop, and sometimes reverse liver damage. Approximately 90 percent of treated AIH patients achieve remission. However if left untreated, the 5-year survival rate of AIH is around 50 percent. […] Multiple approaches are usually used together to treat AIH. […] These drugs can lessen or stop your immune systems attack on your body. Common immunosuppressants used for AIH include 6-mercaptopurine and azathioprine. […] Corticosteroid medications lower inflammation in the body, and can reduce immune system activity at high doses. […] The most common corticosteroid used for AIH is oral prednisone. In order to reduce liver inflammation, prednisone is often prescribed for a minimum of 18 to 24 months. Others may need to be on the drug indefinitely in order to prevent AIH from recurring. […] In very severe cases of AIH, a liver transplant is necessary. During this procedure, your entire liver will be removed and replaced with the liver of a donor (who may be alive or deceased). […] AIH can be treated with medication, and in severe instances, a liver transplant.

• #47 Living with AIH | AIHA – Autoimmune Hepatitis Association

  https://aihep.org/living-with-autoimmune-hepatitis/

  Patients with autoimmune hepatitis (AIH) typically lead long lives. With treatment, most patients dont need liver transplants and have normal life expectancies. […] Patients being treated for AIH do well long-term, and most will never need a liver transplant. In a diverse group of patients (including patients of advanced age), 91% are still living after 10 years, and 70% are still living after 20 years without liver transplants. […] After a flare, your doctor may prescribe steroid medications again or adjust your long-term immunosuppressant dose or frequency. […] In some AIH patients whose liver inflammation is well controlled, liver scarring (fibrosis) can be reversed. In small historical studies of AIH patients with follow-up liver biopsies, over 50% of treated patients may see some improvement (reduction) of liver fibrosis. […] Patients with early fibrosis whose liver enzymes return to normal with treatment will likely live long lives without needing a liver transplant or experiencing liver failure.

• #48 Autoimmune Hepatitis: Symptoms and Treatment

  https://patient.info/digestive-health/hepatitis/autoimmune-hepatitis

  Treatment works well in most cases. Usually, the inflammation settles and symptoms improve within a few months of starting treatment. […] Once the condition has completely settled, your doctor may advise a trial without treatment. […] In many cases, long-term maintenance treatment is needed. This means that the majority of people with autoimmune hepatitis will need to take low doses of immunosuppressant treatment for long periods of time, or even indefinitely. […] Side-effects from the treatment may be troublesome in some cases. […] For some people a liver transplant may be an option – for example: In the few people who do not respond to treatment with the medicines mentioned above; or in people diagnosed in the late stage of the disease with severe 'scarring’ of the liver (cirrhosis) or liver failure. […] With treatment, most people with autoimmune hepatitis have a normal life expectancy and feel well most of the time. The treatment used for autoimmune hepatitis has improved the outlook (prognosis) tremendously.

Zobacz więcej