Autoimmune hepatitis pdf 2012

Associated Data Supplementary Materials Supplemental material for Clinical management of autoimmune hepatitis. Abstract Autoimmune hepatitis is a rare and chronic liver disease that is characterised by increased serum transaminases and immunoglobulin G, inflammatory liver histology and presence of circulating autoantibodies.

Keywords: Autoimmune hepatitis, prednisone, prednisolone, induction therapy, European Association for Study of the Liver, clinical management. Introduction Autoimmune hepatitis AIH is a chronic inflammatory liver disease that predominantly affects women, but can occur in all ages and races. Simplified diagnostic criteria for the diagnosis of autoimmune hepatitis AIH.

Open in a separate window. Typical liver histology for AIH includes each of the following features: interface hepatitis, lymphocytic infiltrates in the portal tracts and extended into the lobule, emperipolesis active penetration by one cell into and through a larger cell and hepatic rosette formation. Compatible liver histology includes: chronic hepatitis with lymphocytic infiltration without the features considered typical. Atypical histology includes signs of other liver diseases such as steatohepatitis.

Why should we treat an AIH patient? Treatment for everyone? Is AIH treatment lifelong? How should we treat an AIH patient? Steroid induction therapy Steroid therapy is the mainstay for inducing remission in AIH: studies with azathioprine induction therapy alone showed low remission rates and high mortality. Maintenance therapy Azathioprine is the first drug of choice for maintenance therapy in AIH. Figure 1. Table 2. Key recommendations for treatment of an adult autoimmune hepatitis AIH patient.

Treatment is indicated in every patient and is generally life-long. Steroid induction therapy with predniso lo ne or budesonide is needed to induce remission. Azathioprine is the first drug of choice for maintenance of remission. Tapering of steroids should be response guided and tailored to the individual patient. Patients with side-effects on azathioprine might benefit from a switch to 6-MP. Patients with cirrhosis should undergo hepatocellular carcinoma surveillance.

What is a satisfactory response during treatment? Remission of disease Histological and biochemical remission of AIH should be the fundamental treatment goal in every patient. What problems do we encounter during AIH treatment? Side-effects Steroid therapy is accompanied by a variety of side-effects, including weight gain, diabetes mellitus, hypertension, emotional instability and even psychosis, that necessitate dose reduction or withdrawal of the drug.

Table 3. Management of medication and side-effects. Medication Side-effect How to manage? Steroids Diabetes mellitus Regular glucose measurements at start of steroid therapy HbA1c monitoring 6—12 monthly Osteoporosis Bone densitometry at start of steroid therapy and at 1—5 year intervals Supplementation of vitamin D and adequate calcium intake Bisphosphonates in patients with osteoporosis Cataract Ophthalmic assessment when on long-term steroids Hypertension Blood pressure assessment in patients with documented hypertension Azathioprine Cytopaenia Full blood count measurements every 2—4 weeks after start of treatment, followed by three-month intervals Non-melanoma skin cancer UV protective measures Dermatological monitoring when on long-term treatment.

Alternative treatment options There is no consensus on the best second-line treatment options in AIH. How to manage difficult-to-treat AIH patients? Acute presentation There are no validated definitions for AIH with acute presentation. Mood disorders Improvement in mood disorders should be an important treatment goal in AIH. When should we refer an AIH patient to a specialist centre? Table 4. Scenarios in which consultation with a specialist centre is recommended.

Uncertainties regarding diagnosis: seek help from an expert liver pathologist Uncertainties regarding treatment indication e. Future prospects Drugs under investigation Currently, a trial is ongoing with ianalumab, a monoclonal antibody against the B-cell activating factor receptor 48 Table 5. Ongoing trials with new drugs in autoimmune hepatitis AIH. Conclusion AIH treatment involves steroid induction therapy followed by maintenance therapy with azathioprine with biochemical remission of disease as primary treatment goal, which is achievable for the majority of patients.

Supplemental Material Supplemental material for Clinical management of autoimmune hepatitis: Click here for additional data file. Declaration of conflicting interests None declared. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

References 1. Autoimmune hepatitis. Nat Rev Dis Primers ; 4 : Suitability of the simplified autoimmune hepatitis score for the diagnosis of autoimmune hepatitis in a German Cohort. United European Gastroenterol J ; 6: — International Autoimmune Hepatitis Group Report: Review of criteria for diagnosis of autoimmune hepatitis.

J Hepatol ; 31 : — Late results of the Royal Free Hospital prospective controlled trial of prednisolone therapy in hepatitis B surface antigen negative chronic active hepatitis. Gut ; 21 : 78— Clinical, biochemical, and histological remission of severe chronic active liver disease: A controlled study of treatments and early prognosis. Gastroenterology ; 63 : — Treatment options for autoimmune hepatitis: A systematic review of randomized controlled trials.

J Hepatol ; 53 : — Usefulness of biochemical remission and transient elastography in monitoring disease course in autoimmune hepatitis. J Hepatol ; 68 : — Autoimmune hepatitis: Effect of symptoms and cirrhosis on natural history and outcome.

Hepatology ; 42 : 53— Relapse is almost universal after withdrawal of immunosuppressive medication in patients with autoimmune hepatitis in remission.

J Hepatol ; 58 : — Patient selection based on treatment duration and liver biochemistry increases success rates after treatment withdrawal in autoimmune hepatitis. J Hepatol ; 62 : — Formulation and application of a numerical scoring system for assessing histological activity in asymptomatic chronic active hepatitis. Hepatology ; 1 : — Azathioprine versus prednisone in non-alcoholic chronic liver disease CLD.

Relation to a serological classification. Liver ; 2 : 95— Diagnosis and management of autoimmune hepatitis. Hepatology ; 51 : — Treatment response in patients with autoimmune hepatitis. Hepatology ; 52 : — Berg, U. Hopf, and P. By OLT, planted because of initial nonfunction and required a second retransplantation 3 years later because of vanishing bile duct syndrome.

Further complications observed were normal liver function can be re-established; however, the recurrent cholangitis n 5 1 , asymptomatic thrombosis of abnormal immunologic background in these patients is not the hepatic artery n 5 1 , prolonged intensive care n 5 2, corrected.

Graft function might be impaired by recurrence due to necrotizing esophagitis, severe neurologic prob- of AIH. It has been n 5 4 , and sarcoidosis of the skin. This difference was statistically significant duced by autoimmune hepatitis.

On the latest available biopsy specimen 18 of 22 patients demonstrated mild-to-moderate graft hepatitis, METHODS suggestive of recurrent autoimmune hepatitis.

In addition, fibrotic changes were seen in 5 of these patients. Titers of Twenty-four 22 female, 2 male patients who were transplanted from to for decompensated AIH were included in the autoantibodies were elevated in 12 of 18 patients with graft study.

Mean age at time of OLT was Four recipients and 8 grafts of the 18 patients AIH and primary biliary cirrhosis.

Of the 4 patients with normal liver CsA -based immunosuppressive regime. Induction therapy with tissue, 2 grafts, but none of the recipients, were positive for the interleukin-2 antibody BT , antilymphocyte globulin, or HLA-B8 and -DR3. No correlation between the presence antithymocyte globulin was performed in 3 patients treated with or absence of the antigenic determinants HLA-B8 and FK and in 8 patients treated with CsA.

Autoimmune hepatitis in Iran: what we know, what we don't know and requirements for better management. Hepat Mon. DOI: AIH is a disease that needs to be managed Received: 29 Jan under the supervision of a team of specialists, including at least Revised: 05 Feb one hepatologist or a gastroenterologist with an interest in liver Accepted: 15 Feb disease, as well as a laboratory that can measure all serum autoan- Keywords: tibodies with accurate titres.

This article is highly recommended Hepatitis, Autoimmune to hepatologists, gastroenterologists, virologists, general practitio- Iran ners, and researchers who are interested in public health. All rights reserved. Autoimmune hepatitis AIH has been the subject of the disease. However, there has not been groups, there have been no such statistics or analysis any rigorous research conducted so far on the long-term available for Iranian patients with AIH until now.

The outcomes of AIH in Iranian patients. In this issue of Hepa- number of Iranian studies published so far about auto- titis Monthly, Malekzadeh et al.

We only have features and long term treatment outcomes of Ira- two studies concerning the clinical characteristics of nian patients diagnosed with AIH We also now know that Iranian DOI: AIH is a rare disease and there are only a few credible Consequently this leads to improper treatment of pa- data sources on the epidemiological aspects of the dis- tients with glucocorticoids. In most ref- agnosed with AIH 20 which is a much higher rate than erences it has been emphasized that treatment should what has been seen in the US and European countries.

These criteria delay in achieving remission and consequently postpone were later revised by an expanded panel in The the achievement of treatment endpoint criteria. It is also very important to ensure that there are no and after treatment with glucocorticoids.

Apart from the contraindications for treatment such as; severe cytope- human leukocyte antigen HLA typing test and tests for nia, morbid obesity or severe osteoporosis Nevertheless, in recent years these system are routinely available in Iranian laboratories. Another problem in Iran is the incorrect these criteria with prednisolone-based treatments The importance of this issue is that ASMA and the titres equal to or more than for ALKM-1, even mild, but consistent elevations in serum transami- are considered to be positive and have scoring values 24, nases, can be associated with; persistent hepatitis 30 , However, many Iranian medical centers only consid- relapse after treatment withdrawal 31 , progression to er the test positive if the titres are equal to or more than cirrhosis 32 , and poor prognosis Therefore a treat- Leber, Blutproteine und Nahrungseiweiss.

Epidemiological indices of 31 patients with autoimmune hepatitis and their response to in many studies 34, 35 including a survey by Malekza- treatment. Unfortunately, there have been no 3. Clinical and labo- ratory characteristics of Autoimmune Hepatitis.

Among other new possible treatments are; budesonide ;12 4 Coeliac disease in autoimmune liver disease: a study 36 , and the later seems to be a good alternative cross-sectional study and a systematic review. Dig Liver Dis. Ursodeoxycholic acid which is usu- 5.