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(AIH) Autoimmune hepatitis -causes and Symptoms

by Sarfraz Hussain 2 years ago in health
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Autoimmune Hepatitis (AIH): Definition, Types

Autoimmune Hepatitis (Lupoid hepatitis)

(AIH) is a chronic auto-immune disease that comes in two forms. Diagnosis is based on elevated aminotransferase and IgG levels and positive results in smooth muscle or nuclear antibody tests (type 1 AIH) or liver-kidney antibody tests (type 2 AIH). Infections or drugs can be triggers; the type 2 AIH auto antibody gene has been identified.

The incidence of autoimmune hepatitis is 10 17: The prognosis of the disease without treatment is poor. In Finland, about 6% of liver transplants are performed due to AIH. New alternatives have been found to treat the disease. Hepatic metabolized budesonide can be used for induction therapy, eliminating the systemic side effects of corticosteroids. The majority of patients require ongoing maintenance therapy, usually using thiopurines.

With standard care,% of patients achieve remission. The rest can be given cyclosporin and tacrolimus, and those who do not respond to all other treatments can receive rituximab. Treatment response is monitored by plasma aminotransferase levels, serum IgG levels, and liver biopsy (AIH), a chronic, progressive, and immune-mediated liver disease of unknown etiology, involving approximately 80% of women.

Diagnosis is based on a typical histological picture, hyperglycemia, and positive results in autoantibody tests. The disease is considered to be a T-cell-mediated autoimmune disease in which a virus, drug, or other environmental factor triggers an immune response against auto- or neoantigens of liver cells.

In AIH, the number of regulatory t cells (Treg cells) is reduced and their function is impaired. The immune response leads to the activation and differentiation of helper t cells into Th1 and Th2 cells and, together with cytokines that mediate inflammatory responses, the initiation of an inflammatory cascade, liver cell damage, and ultimately cirrhosis.

Liver cell damage results from either direct cell-mediated or antibody-mediated cytotoxicity, or a combination thereof. Measles and hepatitis viruses, as well as cytomegalovirus and Epstein Barr viruses, have been suspected to be triggers. Several different drugs have been described to trigger autoimmune hepatitis.

These include methyldopa, nitrofurantoin, diclofenac, interferon, minocycline, and atorvastatin, as well as TNF-alpha blockers (Czaja and Manns 2010).

AIH is divided into two main types based on clinical and serological criteria (TABLE 1). So far only type 2 AIH:n triggering autoantigen has been identified. Cytochrome P450 IID6 has a linear 33 amino acid chain against which LKM1 antibodies react. The antigen is found on the surface of liver cells. LKM1 antibodies are also sometimes detected in patients with hepatitis C.

Genetic factors predisposing to autoimmune hepatitis include phenotypes HLA-DR3 and HLA-DR4 (Czaja 2010) (TABLE 1). Patients with HLA-DR3-related disease are usually young and have severe and often recurrent disease. SLA antibody-positive disease is associated with severe scatological changes, the need for longer immunosuppressive therapy, and a more likely recurrence of the disease at the end of treatment.

The disease is diagnosed in people of all ages, but it is more common in women (3.6: 1). So far, there are no precise data on the prevalence of the disease in Finland, but according to a Norwegian study, it is 16.8: and the annual incidence is 1.9: (Boberg et al. 1998).

These estimates probably also correspond to the prevalence of the disease in the world.

TABLE 1. Autoimmune hepatitis subtypes and their clinical characteristics. Variable Type 1 Type 2 Typical antibodies Autoantigens ANA and SMA-va, atypical panca-va, asialoglycoprotein antibodies SLA-ag, LP-ag LKM-va (types 1 3), LC1-va ANA-va: chromatin, ribonucleoproteins LKM-va: CYP450 2A6 SMA-va: microfilaments (filamentous LKM1-va: CYP450 2D6 Actin) and intermediate-sized filaments (vimentin, LKM2-va: CYP450 2C9 desmin) SLA-va: trnp (ser) sec (transfer ribonucote panca-va: nuclear plate proteins LKM-3-va: UDP-glucuronosyltransferase (UGT1A) LC1-va: formiminotransferase-cyclodeaminase (FTCD) Genetic risk factors DRB1 * 0301 and * 0401 B14, DR3, C4A-Q0, DR7 Age of illness years,and over 50 years 2 14 years Women 78% 89% Progression to cirrhosis 45% 80% Common comorbidities Autoimmune thyroiditis (10 23%), ulcerative colitis (2 8%), rheumatism (2 5%), psoriasis (3%), LED ( 1 2%, celiac disease (1 2%) Type 1 DM (79%), autoimmune thyroiditis, vitiligo ANA-va = nuclear antibodies, SMA-va = smooth muscle antibodies, LKM-va = liver-kidney microsomal antibodies (types 1 3), LC1-va = liver-cytosolic antibodies, panca = neutrophil cytoplasmic antibodies, SLA-ag = soluble liver antigen, LP-ag = liver-pancreatic antigen Diagnosis Clinical picture of disease AIH usually progresses slowly, but% of cases start abruptly and resembles acute viral hepatitis or toxic liver damage, in which case the disease may require an early liver transplant.Type 1 AIH is most common in Finland.Usually, the patient is a young woman, by an accidental diagnosis of non-specific symptoms (eg fatigue, nausea, loss of appetite, weight loss, amenorrhea, abdominal pain, pruritus, or joint symptoms) or in the setting of elevated aminotransferases, anemia, or high blood counts. Surprisingly, the disease can be found to have progressed to the cirrhosis stage in studies of fatigue and jaundice.

It is often associated with other autoimmune diseases, the most common of which are joint symptoms (synovitis), autoimmune thyroiditis, rheumatism, ulcerative colitis, diabetes, celiac disease, and vitiligo. Laboratory findings.

In addition to high aminotransferase levels, typical findings include positive results in nuclear and smooth muscle antibody tests and marked hypergammaglobulinemia. Type 2 AIH is very rare in Finland.Most of the patients are children, 2 14 years old.

In terms of clinical course, the disease is more aggressive and progresses faster. International simplified diagnostic criteria for the disease (Hennes et al. 2008) are presented in TABLE 2. Histology. A liver biopsy is recommended for all AIH patients to confirm the diagnosis (Gleeson and Heneghan 2011).

Although liver histology is typical of AIH, it is not specific to it. Histological features include infiltration of lymphoplasmacytic cells in portal areas, the spread of inflammation to surrounding liver tissue as so-called interphase inflammation, and rosettes of liver cells surrounded by necrosis areas as a result of inflammatory activity (Fig. 1) (Hennes et al. 2008). 1765In terms of clinical course, the disease is more aggressive and progresses faster.

International simplified diagnostic criteria for the disease (Hennes et al. 2008) are presented in TABLE 2. Histology. A liver biopsy is recommended for all AIH patients to confirm the diagnosis (Gleeson and Heneghan 2011). Although liver histology is typical of AIH, it is not specific to it. Histological features include infiltration of lymphoplasmacytic cells in portal areas, the spread of inflammation to surrounding liver tissue as so-called interphase inflammation, and rosettes of liver cells surrounded by necrosis areas as a result of inflammatory activity (Fig. 1) (Hennes et al. 2008). 1765In terms of clinical course, the disease is more aggressive and progresses faster. International simplified diagnostic criteria for the disease (Hennes et al. 2008) are presented in TABLE 2.

A liver biopsy is recommended for all AIH patients to confirm the diagnosis (Gleeson and Heneghan 2011). Although liver histology is typical of AIH, it is not specific to it. Histological features include infiltration of lymphoplasmacytic cells in portal areas, the spread of inflammation to surrounding liver tissue as so-called interphase inflammation, and rosettes of liver cells surrounded by necrosis areas as a result of inflammatory activity (Fig. 1) (Hennes et al. 2008). 1765Liver biopsy is recommended for all AIH patients to confirm the diagnosis (Gleeson and Heneghan 2011). Although liver histology is typical of AIH, it is not specific to it. Histological features include infiltration of lymphoplasmacytic cells in portal areas, the spread of inflammation to surrounding liver tissue as so-called interphase inflammation, and rosettes of liver cells surrounded by necrosis areas as a result of inflammatory activity (Fig. 1) (Hennes et al. 2008). 1765Liver biopsy is recommended for all AIH patients to confirm the diagnosis (Gleeson and Heneghan 2011).

Although liver histology is typical of AIH, it is not specific to it. Histological features include infiltration of lymphoplasmacytic cells in portal areas, the spread of inflammation to surrounding liver tissue as so-called interphase inflammation, and rosettes of liver cells surrounded by necrosis areas as a result of inflammatory activity (Fig. 1) (Hennes et al. 2008). 1765the spread of inflammation to the surrounding liver tissue into so-called interphase inflammation; and rosettes of liver cells surrounded by necrosis areas as a result of inflammatory activity (Fig. 1) (Hennes et al. 2008). 1765the spread of inflammation to the surrounding liver tissue into so-called interphase inflammation; and rosettes of liver cells surrounded by necrosis areas as a result of inflammatory activity.

OVERVIEW Boundary zone activity and liver cell necrosis are signs of severe liver damage. Histological examination is necessary for the early stages of the disease for differential diagnosis and for determining the degree of inflammatory activity and fibrosis. About one-third already show cirrhosis changes at the diagnosis stage (Al-Chalabi et al. 2006, Werner et al. 2008, Gleeson and Heneghan 2011). Histological changes observed at biopsy also have a clear predictive value: if necrosis is already detected in the primary biopsy, the disease progresses to cirrhosis in up to 82% of patients and the five-year survival rate without transplantation is only 55% (Strasbourg and Manns 2011). Normal liver enzyme values ​​do not rule out histological changes, as they only% show a normal histological picture on liver biopsy, even if liver values ​​are within the reference range.A liver biopsy is needed to monitor the disease to determine inflammatory activity and to monitor the progression of fibrosis. Differential Diagnostics Histologically and clinically, AIH resembles, for example, other acute and chronic hepatitis.

Differential diagnostics must exclude, among other things, viral hepatitis and drug-induced liver damage, as well as copper accumulation or Wilson's disease (TABLE 3). AIH may occur simultaneously although AB FIGURE 1. Histological finding in autoimmune hepatitis. A) Abundant portal plasma cell-positive inflammation (arrow) and interphase activity at the edge of the portal area in hematoxylin-eosin staining. B) In severe autoimmune hepatitis, rapidly interconnected (conflating) areas of necrosis develop within which live hepatocytes remain. They form islands, called rosettes. Rosette formation of periportal hepatocytes (arrow) in Heroic staining. Photo: Johanna Arola. TABLE 2. Simplified criteria for clinical diagnosis of autoimmune hepatitis (AIH).

Variable Limit value Score S-ANA-VA, S-SMA-VA 1:40 1 S-ANA, S-SMA-VA 1:80 anti-number-VA 1:40 2 * SL antigen Positive test result S-IgG Above the upper limit of the reference range 1 1.10 x above the upper limit of the reference range 2 Suitable for AIH 1 Histological picture of the liver Typical of AIH 2 Hepatitis viral serology Negative test result 2 Score Probable AIH 6 Confident AIHSimplified criteria for clinical diagnosis of autoimmune hepatitis (AIH).

Variable Limit value Score S-ANA-VA, S-SMA-VA 1:40 1 S-ANA, S-SMA-VA 1:80 anti-number-VA 1:40 2 * SL antigen Positive test result S-IgG Above the upper limit of the reference range 1 1.10 x above the upper limit of the reference range 2 Suitable for AIH 1 Histological picture of the liver Typical of AIH 2 Hepatitis viral serology Negative test result 2 Score Probable AIH 6 Confident AIHSimplified criteria for clinical diagnosis of autoimmune hepatitis (AIH).

Variable Limit value Score S-ANA-VA, S-SMA-VA 1:40 1 S-ANA, S-SMA-VA 1:80 anti-number-VA 1:40 2 * SL antigen Positive test result S-IgG Above the upper limit of the reference range 1 1.10 x above the upper limit of the reference range 2 Suitable for AIH 1 Histological picture of the liver Typical of AIH 2 Hepatitis viral serology Negative test result 2 Score Probable AIH 6 Confident AIHtypical of Hepatitis virus serology Negative test result 2 Score Probable AIH 6 Confident AIH * Up to 2 points in total for autoantibodies Abbreviations in Table 1. M. Färkkilätypical of Hepatitis virus serology Negative test result 2 Score Probable AIH 6 Confident AIH * Up to 2 points in total for autoantibodies Abbreviations in Table 1. M. Färkkilä

TABLE 3. Diseases to be considered in the differential diagnosis of autoimmune hepatitis. may occur concomitantly with PBC or PSC (so-called overlapping syndromes). Disease Hepatitis viruses (AD) Other viral hepatitis Drug-induced or natural product-induced hepatitis Primary biliary cirrhosis (PBC) Primary sclerosing cholangitis (PSC) Autoimmune cholangitis Wilson's disease HCl disease HCaostasis HCl, Hypochromatosis HBV-DNA, HDV-VA EBV-NH, CMV-NH, HHV-6-VA, History of S-mitochondrial antibodies, S-IgG, S-IgM, liver histological examinations: granulomas ERCP typical, magnetic cholangiography Normal ERC, liver biopsy cholangitis, mitochondrial antibody test negative but nuclear antibody test positive, IgG increased S-ceruloplasmin, du-cu, liver biopsy S-ferritin, S-Fe, S-transferrin saturation, HFE gene test (C282Y), iron from liver biopsy specimen Alpha 1 -antitrypsin, genotyping if necessary: ​​PiZZ / PiSS / PiMZ / Pisz History of S-GT, B-MCV, S-CDT, S-ASAT, S-ALAT pa with primary biliary cirrhosis (PBC), autoimmune cholangitis and especially primary sclerosing cholangitis (PSC) (so-called overlapping syndromes). In children, AIH is a common finding associated with PSC. As many as 50% of children with PSC have concomitant AIH, compared with only about 78% in adults (Kaya et al. 2000, Gleeson and Heneghan 2011). AIH: a cholestatic form of the disease (markedly elevated alkaline phosphatase) or poor response to standard medication in adults with suspected sclerosing cholangitis.

The diagnosis of PSC is based on endoscopic retrograde cholangiography (ERC). A patient has an intermediate form of AIH and PBC if he is diagnosed with nuclear or smooth muscle antibodies and if the results of mitochondrial antibody tests are positive. Plasma IgG and IgM levels are also often elevated. Of the AIH-positive patients, 8 12% have mitochondrial antibodies and 8% have histological changes consistent with PBC (Gleeson and Heneghan 2011). Treatment Without treatment, the prognosis of the disease is very poor in the majority of patients and only 10% live more than ten years. Corticosteroid therapy has significantly altered the prognosis of the disease.The goal of induction therapy is to turn off the activity of the disease and the disease into remission.

Maintenance treatment aims to prevent the development of fibrosis and cirrhosis. Decreases in aminotransferase levels, normalization of serum IgG levels, and loss of interphase hepatitis changes are used as measures of response to treatment (Manns et al. 2010, Gleeson and Heneghan 2011). Within six months, in 80% of patients with standard corticosteroid therapy or a combination of corticosteroid and other immunosuppressive medication, aminotransferase levels are reduced to less than twice the upper limit of normal (Soloway 1972). When is treatment appropriate? A recent recommendation recently published by the British Society of Gastroenterology (BSG) (Gleeson and 1767Maintenance treatment aim to prevent the development of fibrosis and cirrhosis.

Decreases in aminotransferase levels, normalization of serum IgG levels, and loss of interphase hepatitis changes are used as measures of response to treatment (Manns et al. 2010, Gleeson and Heneghan 2011). Within six months, in 80% of patients with standard corticosteroid therapy or a combination of corticosteroid and other immunosuppressive medication, aminotransferase levels are reduced to less than twice the upper limit of normal (Soloway 1972). When is treatment appropriate? A recent recommendation recently published by the British Society of Gastroenterology (BSG) (Gleeson and 1767Maintenance treatment aim to prevent the development of fibrosis and cirrhosis. Decreases in aminotransferase levels, normalization of serum IgG levels, and loss of interphase hepatitis changes are used as measures of response to treatment (Manns et al. 2010, Gleeson and Heneghan 2011).

Within six months, in 80% of patients with standard corticosteroid therapy or a combination of corticosteroid and other immunosuppressive medication, aminotransferase levels are reduced to less than twice the upper limit of normal (Soloway 1972). When is treatment appropriate? A recent recommendation recently published by the British Society of Gastroenterology (BSG) (Gleeson and 1767normalization of serum IgG levels and disappearance of interphase hepatitis changes (Manns et al. 2010, Gleeson and Heneghan 2011).

OVERVIEW TABLE 4. Topics of corticosteroids or immunosuppressive therapy in autoimmune hepatitis. When treatment is appropriate Young patient Moderate or severe disease: AST above 5 x ULN IgG above 2 x ULN Confluent necrosis in liver tissue sample Cirrhosis with mild histological activity When required No serologically detectable activity (AST, ALT, IgG) or histologically advanced autoimmune hepatitis with cirrhosis without evidence of inflammatory activity (Heneghan 2011), treatment is appropriate for young patients, all patients with moderate to severe AIH, and patients with mild cirrhosis activity in a tissue sample (TABLE 4). AIH is defined as having moderate or severe activity if the plasma AST is more than five times and the IgG is more than twice the upper limit of the reference range and confluent necrosis is detected in the liver tissue sample. Medication. Because the etiopathogenesis of the disease is still poorly understood, drug treatment is based on quenching the aberrant immune response with medication (TABLE 5).

Figure 2 shows the course of AIH treatment. In induction therapy, especially in the acute phase, prednisolone (30 to 60 mg/day) or methylprednisolone is used either alone or in combination with azathioprine or 6-mercaptopurine (Manns et al. 2010a, Gleeson and Heneghan 2011). However, in the largest comparative study of AIH to date, budesonide (9 mg/day) is as effective an induction drug as prednisolone (40 mg/day).In addition, it was associated with fewer adverse events (Manns et al. 2010b).

The primary corticosteroid response is achieved in% of patients based on what is considered a measure of response to treatment. If the onset is acute, it is recommended to predate TABLE 5. Drugs and indications for the treatment of autoimmune hepatitis. Subject Medication Dose / day Remarks Induction Budesonide 3 9 mg Mild disease, no systemic symptoms Maintenance of remission Prednisolone mg Moderate to severe disease Budesonide 3 9 mg Maintenance of remission Prednisolone mg Maintenance of low dose (10 mg / day) Remission maintenance Thiopurines 1 2 mg / kg with corticosteroid or as monotherapy Mycophenolate 2 g Maintenance of remission in patients intolerant to thiopurines Relapse Budesonide 6 9 mg New induction Prednisolone mg Thiopurines 1 2 mg / kgif maintained with corticosteroid Mycophenolate 2 g Dose increase to pre-relapse tolerated dose 1768 Disease unresponsive to standard therapy Ciclosporin Tacrolimus Rituximab 5 6 mg / kg (concentration) 2 10 mg (concentration) mg IV on days 0 and Response 6 maintenance Maintenance of Remission in a disease unresponsive to thiopurine and corticosteroid therapy Maintenance of Remission in a disease unresponsive to thiopurine and corticosteroid therapy Chronically active disease unresponsive to other therapies with high IgG value as a sign of B cell activation M. Färkkiläon days 0 and Response assessment maintenance 6 Every 12 months Maintenance of remission in disease unresponsive to thiopurine and corticosteroid therapy Maintenance of remission in disease unresponsive to thiopurine and corticosteroid therapy Other unresponsive, chronically active disease IgGon days 0 and Response assessment maintenance 6 Every 12 months Maintenance of remission in disease unresponsive to thiopurine and corticosteroid therapy Maintenance of remission in disease unresponsive to thiopurine and corticosteroid therapy Other unresponsive, chronically active disease IgG.

Mild: S-ALT <5 x upper limit of normal S-IgG <2 x upper limit of normal Histology grade 1 Budesonide 6 9 mg Moderate/severe: S-ALT 5 upper limit of normal S-IgG 2 x upper limit of normal Histology grade 2 Prednisolone mg + Azathioprine 1 2 mg/kg Remission: * S-ASAT normal, S-IgG normal repeated Budesonide 6 3 mg Corticosteroid discontinuation in Remission * monotherapy 3 years G 1 Liver biopsy G 2 Discontinuation Permanent remission Follow-up Relapse Maintenance treatment FIGURE 2. Treatment of autoimmune hepatitis.

In asymptomatic patients with mild disease, budesonide monotherapy may be initiated as monotherapy. In moderate to severe disease, treatment is already initiated primarily with a combination of prednisolone and thiopurine. The response to treatment is monitored by serum ALT and IgG levels. If the disease is still in remission after 2 to 3 years of treatment, corticosteroids may be discontinued in combination therapy. If remission persists during monotherapy, treatment may be discontinued. Usually, however, this requires that histological activity (G), especially interphase inflammation, be extinguished. Remission = normal ALT and IgG, relapse = ALT> 3 x ULN, remission loss = ALT> upper limit of normal, IgG> 20 mg / l nisolone mg / day in combination with azathioprine 1 2 mg / kg / day.

Corticosteroid therapy is discontinued very slowly, within months following the response to treatment. In the case of asymptomatic, mild disease, treatment may be initiated with budesonide or a lower dose of corticosteroid (20 to 30 mg/kg).Some patients (20 to 35%) do not respond to standard care, and sometimes the patient does not tolerate a high dose of corticosteroids. Treatment of disease not responding to standard care. The lack of response to corticosteroid induction therapy is predicted by, among other things, acute or young-onset disease (less than 40 years), jaundice, or high plasma bilirubin at the time of diagnosis, and the HLA-DRB1 * 0310 haplotype (Selvarajah 2012).

Ciclosporin or tacrolimus may be administered to patients who do not respond to a corticosteroid. If the patient does not tolerate thiopurines, 1769jaundice or high plasma bilirubin at the time of diagnosis and HLA-DRB1 * 0310 haplotype (Selvarajah 2012). Ciclosporin or tacrolimus may be administered to patients who do not respond to a corticosteroid. If the patient does not tolerate thiopurines, 1769jaundice or high plasma bilirubin at the time of diagnosis and HLA-DRB1 * 0310 haplotype (Selvarajah 2012). Ciclosporin or tacrolimus may be administered to patients who do not respond to a corticosteroid. If the patient does not tolerate thiopurines, 1769.

REVIEW 1770 NUCLEAR MATTERS 88 is the most common chronic autoimmune disease found in young women. 88Diagnosed and untreated, its prognosis is very poor. In induction therapy, budesonide is similar to prednisolone without systemic adverse events. 88The majority of patients require continuous maintenance therapy, most commonly with azathioprine or 6-mercaptopurine. 88In patients who do not respond to standard care (approximately 20%), alternative medicines include ciclosporin, tacrolimus, and rituximab. it is possible to use mycophenolate instead (Sharzehi et al. 2010). If the disease has not responded to thiopurines, it is unlikely that mycophenolate will also elicit a therapeutic response (Selvarajah et al. 2012).

If the patient also does not respond to combination therapy (corticosteroid, thiopurine/mycophenolate, and cyclosporine), rituximab (Burak et al. 2008), a chimeric, CD20 monoclonal antibody against the B-lymphocyte cell surface transmembrane receptor can be used. It effectively reduces the number of these receptors through both cytotoxic mechanisms and induction of programmed cell death. Rituximab may be used in patients who do not respond to other therapies who show high serum IgG levels as a sign of strong B cell activation.

Initial treatment experience in individual patients has been excellent, and patients have achieved complete remission after 1 2 infusions as assessed by both liver enzyme and IgG response (Burak et al. 2008). Maintenance treatment. The problem with the treatment of autoimmune hepatitis is the recurrence of the disease when the medication is stopped or reduced, and indeed, it is evident that the majority of patients require continuous maintenance medication (van Gerven et al. 2013). It uses primarily azathioprine (1 2 mg/kg) or 6-mercaptopurine and the lowest possible corticosteroid dose to maintain remission. This is achieved with azathioprine treatment alone in up to 80% of patients with remission.

If the disease recurs during medication, relapses can be treated with corticosteroid inductions and it is also possible to increase the thiopurine dose. If the patient is intolerant to thiopurines or the drug is insufficient to maintain remission, maintenance therapy with ciclosporin or tacrolimus, in milder cases, budesonide, may be used.

Combination diseases, most commonly budesonide, corticosteroids, a combination of thiopurine and ursodeoxycholic acid (EASL Clinical Practice Guidelines 2009, Trivedi et al. 2012). In advanced cirrhosis, corticosteroid therapy should be discontinued due to, among other things, complications of infection. Medication may be attempted to be reduced or discontinued if the disease has been in remission for at least one year based on liver values ​​and immunoglobulin levels and no hepatic biopsy shows inflammatory changes or advanced connective tissue supplementation compared to the previous tissue sample. Pregnancy and AIH Because a significant proportion of those affected are young women, pregnancy and AIH are not a rare combination.

Patients have normal fertility if the disease has not reached the stage of cirrhosis. Before planned pregnancy, the disease should be brought into good remission to avoid exacerbations during pregnancy. A disease with a good balance of care does not increase miscarriages or congenital malformations. Initiated azathioprine therapy should be continued throughout pregnancy and lactation as usual to prevent post-pregnancy exacerbations (Gleeson and Heneghan 2011). Mycophenolate, on the other hand, should not be used during pregnancy. After delivery, the patient should be closely monitored for possible disease activation. Immunosuppressive medication is sensitively increased, usually by increasing the corticosteroid dose. M. FärkkiläAfter delivery, the patient should be closely monitored for possible disease activation. Immunosuppressive medication is sensitively increased, usually by increasing the corticosteroid dose. M. FärkkiläAfter delivery, the patient should be closely monitored for possible disease activation. Immunosuppressive medication is sensitively increased, usually by increasing the corticosteroid dose. M. Färkkilä.

8Follow-up The goal of drug therapy is remission. In this case, plasma or serum liver enzyme and IgG values ​​are normal and no evidence of inflammatory activity is observed on histological examination. The response to treatment is monitored by aminotransferase values: an increase in them indicates active liver cell destruction (TABLE 6). Normal concentrations do not exclude the inflammatory activity observed in histopathological examinations. Increasing immunoglobulin levels are a good predictor of disease activity, but instead, serum antibody levels (ANA, SMA) do not correlate with the degree of inflammation. To ensure a primary response, a follow-up liver biopsy is appropriate within 1 to 2 years of starting treatment. Inflammatory activity and fibrosis should be monitored with tissue samples every 3 to 5 years (Gleeson and Heneghan 2011).In advanced cirrhosis, echocardiography of the liver is required semi-annually due to the risk of hepatocellular carcinoma (Feld et al. 2005) (TABLE 6).

Due to the increased risk of osteoporosis, patients recommended calcium-vitamin D supplementation therapy. Bone density measurement should be done at least every couple of years after the diagnosis phase (Gleeson and Heneghan 2011). Prognosis The prognosis is quite good if the disease has not already progressed to cirrhosis at the diagnosis stage or if treatment for the acute form of the disease has been started in time. In the Canadian study, the ten-year survival rate was 94% if the patient had no cirrhosis. The proportion was the same in asymptomatic and symptomatic (Gautam et al. 2006). In contrast, cirrhosis already detected at the diagnosis stage weakens the prognosis, with a ten-year survival rate of only 62%. Disease increases mortality (Hoeroldt et al. 2011): The standardized mortality rate (SMR) for all causes of death is 1.63.

The overall prognosis of patients in treatment is good, with 90% of patients surviving ten years without a liver transplant. TABLE 6. Patient follow-up. Variable Inflammatory activity Fibrogenesis Hepatic function Portal venous pressure Liver cancer screening Poor response Liver transplantation Study ALT, AST, AFOS In case of significant hepatic enlargement or discontinuation of liver biopsy and if necessary S-C3, C4, IgG Hepatic biopsy, S-every B 5, AST-ALT ratio CT, prealbumin and bilirubin values ​​Gastroscopy to exclude varicose veins In cirrhotic disease: abdominal resonance every 6 months, CT or MK Overlapping syndrome if necessary: ERCP or magnetic cholangiography to exclude PSC Exclusion of Wilson's disease Transplantation is usually associated with advanced cirrhosis of the liver with complications (ascites, crow bleeding, hepatic encephalopathy, and spontaneous bacterial peritonitis or hepatorenal syndrome). The MELD score, which describes the liver function, is more than 15 or the Child-Pugh score is more than 10.3. In the European data (www. Eltr.org), 2.6% of liver transplants are due to AIH. 32 patients with chronic and five patients with acute autoimmune hepatitis, accounting for 6% of all transplants, and an average of 20% of patients relapse to transplantation, mostly with mild immunosuppressive medication (Gautam et al. 2006).as the five- and ten-year survival rate in the European data is 75% (Schramm et al. 2010), in world.

9LITERATURE REVIEW Al-Chalabi T, Boccato S, Portmann BC, et al. Autoimmune hepatitis (AIH) in the elderly: a systematic retrospective analysis of a large group of consecutive patients with definite AIH followed at a tertiary referral center. J Hepatol 2006; 45: Boberg KM, Aadland E, Jahnsen J, Raknerud N, Stiris M, Bell H. Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population. Scand J Gastroenterol 1998; 33: Burak KW, Williamson-Coffey S, Swain MG, et al. Rituximab for refractory autoimmune hepatitis: interim results of a phase 1 study. Hepatology 2008; 48: S1093. Czaja AJ. Autoimmune hepatitis.

In: Feldman M, Friedman LS, Brandt LJ, ed. Sleisenger and Fordtran s gastrointestinal and liver disease pathophysiology, diagnosis, management. 9th edition. 2010,s Czaja AJ, Manns M P. Advances in the diagnosis, pathogenesis, and management of autoimmune hepatitis. Gastroenterology 2010; 139: EASL Clinical Practice Guidelines: Management of cholestatic liver diseases. J Hepatol 2009; 51: Feld JJ, Dinh H, Arenovich T, etc. Autoimmune hepatitis: Effect of symptoms and cirrhosis on natural history and outcome. Hepatology 2005; 42: Gautam M, Cheruvattath R, Balan V. Recurrence of autoimmune liver disease after liver transplantation: a systematic review. Liver Transpl 2006; 12: Gleeson D, Heneghan MA. British Society of Gastroenterology (BSG) guidelines for the management of autoimmune hepatitis. Gut 2011; 60: Hennes EM, Zeniya M Czaja AJ, et al., Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology 2008; 48: Hoeroldt B, McFarlane E, Dube A, et al.Long-term outcomes of patients with autoimmune hepatitis managed at a nontransplant center.

Gastroenterology 2011; 140: Kaya M, Angulo P, Lindor KD. Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an evaluation of a modified scoring system. J Hepatol 2000; 33: Manns M, Czaja AJ, Gorham JD, et al., AASLD Practice guidelines. Diagnosis and management of autoimmune hepatitis. Hepatology 2010 (a); 51: Manns MP, WoynarowskiI M, Kreisel W, et al. Budesonide induces remission more effectively than prednisone in a controlled trial of patients with autoimmune hepatitis. Gastroenterology 2010 (b); 139: Montano-Loza AJ, Carpenter HA, Czaja AJ. Predictive factors for hepatocellular carcinoma in type 1 autoimmune hepatitis. Am J Gastroenterol 2008; 103: Schramm C, Bubenheim M, Adam R, etc.

European Liver Intestine Transplant Association: primary liver Transplantation for autoimmune hepatitis: a comparative analysis of the European Liver Transplant Registry. Liver Transpl 2010; 16: Selvarajah V, Montano-Loza AJ, Czaja AJ. Systematic review: managing suboptimal treatment Responses in autoimmune hepatitis with conventional and nonstandard drugs. Aliment Pharmacol Ther 2012; 36: Sharzehi K, Huang MA, Schreibman IR, Brown KA. Mycophenolate mofetil for the treatment of autoimmune hepatitis in patients refractory or intolerant to conventional therapy. Can J Gastroenterol 2010; 24: Soloway RD, Summerskill WH, Baggenstoss AH, et al., Clinical, Biochemical, and histological remission of severe chronic active liver disease: a controlled study of treatments and early prognosis.

Gastroenterology 1972; 63: 820e33. Strasbourg CP, Manns MP. Therapy of autoimmune hepatitis.Best Practice Res Clin Gastroenterol 2011; 25: Trivedi PJ, Hirschfield GM. Review article: overlap syndromes and autoimmune liver disease. Aliment Pharmacol Ther 2012; 36: van Gerven NM, Verwer BJ, Witte BI, etc. Relapse is almost universal after withdrawal of immunosuppressive medication in patients with autoimmune hepatitis in remission. J Hepatol 2013; 58: Werner M, Prytz H, Ohlsson B, et al., Epidemiology and the initial presentation of autoimmune hepatitis in Sweden: a nationwide study. \

Scand J Gastroenterol 2008, 43: MARTTI FÄRKKILÄ, Professor, Chief Physician University of Helsinki, Department of Clinical Medicine and HUCH, Department of Medical Sciences, Department of Gastroenterology ATTENTIONS,osakeomistus (Orion Corporation) Summary Autoimmune hepatitis is a chronic liver disease with two subtypes, type 1 with anti-nuclear or smooth muscle antibodies and type 2 with LKM1 or LC1 antibodies, and both with hyper gamma globulinemia and typical histology.

The prevalence of AIH is between 10 to 17 per in Europe. Up to% of cases present with acute hepatitis. Budesonide can be used as first-line induction therapy in non-cirrhotic patients, and thiopurines, Mercaptopurine, or mycophenolic acid as maintenance Therapies. Patients not responding to conventional therapy can be treated with Ciclosporin, Tacrolimus, or rituximab or finally with liver Transplantation M. Färkkiläand both with hyper gamma globulinemia and typical histology. The prevalence of AIH is between 10 to 17 per in Europe. Up to% of cases present with acute hepatitis. Budesonide can be used as first-line induction therapy in non-cirrhotic patients, and thiopurines, Mercaptopurine, or mycophenolic acid as maintenance Therapies. Patients not responding to conventional therapy can be treated with Ciclosporin, Tacrolimus, or rituximab or finally with liver Transplantation M. Färkkiläand both with hyper gamma globulinemia and typical histology.

The prevalence of AIH is between 10 to 17 per in Europe. Up to% of cases present with acute hepatitis. Budesonide can be used as first-line induction therapy in non-cirrhotic patients, and thiopurines, Mercaptopurine, or mycophenolic acid as maintenance Therapies. Patients not responding to conventional therapy can be treated with Ciclosporin, Tacrolimus or rituximab or finally with liver Transplantation M. FärkkiläTacrolimus or rituximab or finally with liver Transplantation M. FärkkiläTacrolimus or rituximab or finally with liver Transplantation M. Färkkilä.

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I am a professional journalist and I work as a writer and reporter in a national newspaper. The purpose of my life is to help people. Useful Tips on Health Care to Improve the Lives of an Ordinary Man.

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