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At least 19 recordsLinked to original sources
PubMedDate not supplied

Autoimmune hepatitis, autoimmune gastritis, and gastric carcinoid in a type 1 diabetic patient: a case report.

The history of a 45-year-old male type 1 diabetic patient is presented. At the age of 29 years, he was diagnosed to have an autoimmune hepatitis with incipient liver cirrhosis. Five years later, a successful liver/pancreas transplantation was performed. Eighteen months later, however, pancreatic insufficiency occurred due to thrombosis of the pancreatic graft. Besides these conditions, iron deficiency, pernicious anemia, and autoimmune gastritis were also diagnosed. Serum parietal cell antibodies (PCA) and intrinsic factor antibodies (AIF) were positive. At 45, this patient was found to have a gastric carcinoid tumor. The clinical importance of PCA is discussed with regard to chronic atrophic gastritis and pernicious anemia, which both predispose toward gastric carcinoid tumors. Autoimmune type 1 diabetic patients who have a high prevalence of PCA should be screened for gastric autoimmune manifestations and tumors, as the history of this patient illustrates.

Autoantibodies↗
PubMed1995

[Autoimmune hepatitis, autoimmune gastritis, hypergastrinemia and stomach carcinoid].

A 48-year-old woman with type II diabetes developed fatigue, arthralgia and myalgia. A few weeks later she was found to have hepatomegaly. The erythrocyte sedimentation rate was raised (53/93 mm), as were liver enzyme activities (GOT 186 U/l; GPT 240 U/l; gamma-GT 199 U/l), the gamma-globulin levels (40.7%;IgG 4470 mg/dl, IgA 698 mg/dl, IgM 245 mg/dl), antinuclear antibodies and antibodies against double-strand DNA, smooth muscles and actin. Laparoscopy revealed small-nodular liver cirrhosis. The autoimmune hepatitis was treated with prednisolone (initially 60 mg daily, then reduced to 10 mg daily) and azathioprine (initially 100 mg daily, reduced to 50 mg daily). The symptoms markedly improved. But one year later, during follow-up examination, gastric polyps were found, excised and histologically found to be carcinoid. The gastrin level was raised to 765 pg/ml. Another year later the liver cirrhosis had advanced further and the type A gastritis was still present, but there was no sign of carcinoid recurrence.

Autoimmune Diseases↗
PubMed2006

[The role of liver biopsy in the diagnosis of autoimmune hepatitis].

Autoimmune hepatitis is a progressive inflammatory hepatitis of unknown etiology. The diagnosis of autoimmune hepatitis is guided by the descriptive criteria and score system implemented by the International Autoimmune Hepatitis Group in 1994. In addition to clinical and laboratory data and the presence of autoantibodies, liver biopsy plays an important role in the diagnostic regimen. Previous studies have emphasized that the histological picture of autoimmune hepatitis was characteristic but not pathognomic for the disease, therefore diagnosis was based on the exclusion of other chronic liver diseases. The goal of this study is to summarize the experiences based on the examination of 93 liver biopsies from 87 patients suffering from autoimmune hepatitis in the Department of Pathology St. László Hospital, Budapest, during the period of 2000-2006. The morphological picture was evaluated based on the criteria of Histological Activity Index as well as other histological features. In addition it was compared to the usual histological characteristics of viral hepatitis, most importantly hepatitis C. The morphological changes examined first were the ones that constitute the diagnostic criteria of autoimmune hepatitis: the severity of portal inflammation and interface hepatitis, the cell composition of the inflammation, rosette formation of periportal hepatocytes, and the presence and extent of centrilobular and bridging necrosis. Results indicated that in most cases the portal/periportal inflammation was very active and lead to the destruction of the original liver structure which was followed by regeneration process (periportal rosettes). Among the inflammatory cells, plasma cells were predominant. In the majority of the cases severe centrilobular and/or bridging necrosis was also present, which strongly elevated the index number, approaching the maximum value. In most patients, only mild fibrosis was observed and merely a handful had progressed to cirrhosis. In conclusion the morphological picture of autoimmune hepatitis is not only characteristic but also pathognomic for the disease. Of course, additional data on clinical and biochemical findings, circulating autoantibodies, and abnormal levels of serum globulins are also necessary for correct diagnosis of autoimmune hepatitis.

Adolescent↗
PubMed2003

[Autoimmune hepatitis].

Autoimmune hepatitis is an unresolving, hepatocellular inflammation of unknown cause that is characterized by the presence of periportal hepatitis on histologic examination, tissue autoantibodies in serum, and hypergammaglobulinemia. By international consensus, the designation autoimmune hepatitis has replaced alternative terms for the condition. Three types of autoimmune hepatitis have been proposed based on immunoserologic findings. Type 1 autoimmune hepatitis is characterized by the presence of antinuclear antibodies (ANA) or smooth muscle antibodies (SMA) (or both) in serum. Seventy percent of patients with type 1 of autoimmune hepatitis are women. This type is the most common form and accounts for at least 80% of cases. Type 2 is characterized by the presence of antibodies to liver-kidney microsome type 1 (anti-LKM1) in serum. Patients with this type of autoimmune hepatitis are predominantly children. Type 3 autoimmune hepatitis is characterized by the presence of antibodies to soluble liver antigen (anti-SLA) in serum. There are no individual features that are pathognomonic of autoimmune hepatitis, and its diagnosis requires the confident exclusion of other conditions. The large majority of patients show satisfactory response to corticosteroid (usually prednisone or prednisolone) therapy. For the past 30 years it has been customary to add azathioprine as a "steroid sparing" agent to allow lower doses of steroids to be used and remission, once achieved, can be sustained in many patients with azathioprine alone after steroid withdrawal. Patients with autoimmune hepatitis who have decompensated during or after corticosteroid therapy are candidates for liver transplantation.

Autoantibodies↗
PubMed2004

Autoimmune hepatitis.

Autoimmune hepatitis affects all ages with a peak incidence in preadolescent girls. The pathogenesis of autoimmune hepatitis has not been defined. Susceptibility, clinical manifestations, and treatment outcomes are affected by environmental factors, individual immunoregulatory responses, genetic factors, age and gender. An international panel has developed diagnostic criteria for autoimmune hepatitis, and a scoring system to assess the strength of the diagnosis has also been proposed. This article discusses the diagnosis and treatment of the three types of autoimmune hepatitis proposed based on characteristic autoantibody profiles, as well as de novo autoimmune hepatitis, a new type of autoimmune hepatitis that has been recently described in liver transplant recipients without previous autoimmune disease.

Adolescent↗
PubMedDate not supplied

Autoimmune hepatitis.

Autoimmune hepatitis can present as either acute or chronic disease in children. Clinical and laboratory features, including association with extrahepatic autoimmune syndromes and prompt response to immunosuppressive treatment, circulating autoantibodies and hypergammaglobulinemia, suggest an immune etiology. However, the disease mechanism remains uncertain. Different types of autoimmune hepatitis are defined on the basis of which autoantibodies are present: anti-smooth muscle (type 1), anti-liver/kidney microsomal (type 2), or anti-soluble liver antigen (type 3). Diseases which may be clinically similar to autoimmune hepatitis must be excluded before the diagnosis of autoimmune hepatitis is established: Wilson's disease, primary sclerosing cholangitis, chronic hepatitis B or C, and drug-induced liver disease are among the most important entities. Corticosteroids alone or with azathioprine constitute the usual treatment for autoimmune hepatitis. Although some children achieve a complete remission, or even recovery, and can stop immunosuppressive treatment, others required low-dose prednisone treatment indefinitely.

Adrenal Cortex Hormones↗
PubMedDate not supplied

Current concepts in autoimmune hepatitis.

Autoimmune hepatitis has a global occurrence, diverse clinical phenotype, and evolving treatment options. The goals of this report are to review the codified diagnostic criteria, spectrum of clinical presentations, proposed pathogenic mechanisms, conventional treatment strategies, and promising interventions. The literature published in English from 1980-2005 was reviewed and an updated current perspective provided. Autoimmune hepatitis affects all ages, may be asymptomatic, frequently has an acute onset, and can present as fulminant hepatitis. Perivenular (zone 3) necrosis is within the histological spectrum. Autoimmune hepatitis can recur or develop de novo after liver transplantation. CD4+ T-helper cells and natural killer T cells have been implicated in the pathogenesis, and molecular mimicry may break self-tolerance. DRB1*0301 and DRB1*0401 are the susceptibility alleles among white North Americans and northern Europeans, whereas diverse alleles of HLA DR4 have been associated with the disease in Japan, mainland China, and Mexico. DRB1*1301 is associated with autoimmune hepatitis in South American children, and it may predispose to an indigenous etiologic agent. Antibodies to soluble liver antigen/liver pancreas may have prognostic importance, and cyclosporine and mycophenolate mofetil must be assessed by clinical trial before incorporation into management algorithms. Site-specific interventions are feasible, and they require a confident experimental animal model for evaluation. Variant syndromes lack diagnostic and therapeutic guidelines. In conclusion, autoimmune hepatitis must be considered in all patients with acute and chronic liver disease and those with allograft dysfunction after transplantation. New immunosuppressive agents and site-specific interventions promise to improve care.

Anti-Inflammatory Agents↗
PubMedDate not supplied

Autoimmune hepatitis.

Autoimmune hepatitis is an inflammatory liver disease in which the immune system is believed to orchestrate an immune attack onto the liver cell. Current knowledge suggests that both T helper 1 (TH1) and TH2 programmes are involved in the generation of the liver damage. Release of TH2 cytokines leads to the production of autoantibodies to the hepatocyte membrane that recruit killer cells. TH1 cytokines induce macrophage activation which contributes to hepatocyte destruction. Patients commonly possess the "autoimmune" HLA A1/B8/DR3 haplotype and a silent gene at the C4A locus with consequent partial deficiency of the complement component C4. Two main types of autoimmune hepatitis are recognised according to the presence of circulating non-organ specific autoantibodies. Patients with smooth muscle antibody and/or antinuclear antibody may be adults or children, while patients with antiliver kidney microsomal type 1 (LKM1) antibody are usually children or very young adults. In both types there is a preponderance of females. LKM1 antibody is also present in a proportion of adult patients, mainly male, with chronic hepatitis C virus infection. This observation originally led to the suggestion that hepatitis C virus may be the cause of this form of autoimmune hepatitis, but several studies have shown that the epitopes target of the LKM1 antibody in autoimmune hepatitis and chronic hepatitis C virus infection differ. Although autoimmune hepatitis responds satisfactorily to immunosuppression in the short term, progression to cirrhosis is frequent. It is hoped that ongoing research will provide a better understanding of the pathogenic mechanisms of liver damage leading to a more effective and specific mode of treatment.

Autoantibodies↗
PubMed1999

Cyclosporine therapy in patients with steroid resistant autoimmune hepatitis.

Autoimmune hepatitis is a form of chronic liver disease characterized by progressive hepatocellular inflammation, which usually responds to treatment with corticosteroids. However, 10% of patients with autoimmune hepatitis are refractory to corticosteroids and develop progressive liver disease and cirrhosis. We describe five patients with autoimmune hepatitis who did not respond to conventional corticosteroids and azathioprine therapy who were then treated with cyclosporine A. Cyclosporine A was started at 2-3 mg/kg/day and induced biochemical remission in four of five patients within 3 months. One of the four responders relapsed within 1 month of discontinuing cyclosporine on two occasions. Each time, liver tests promptly normalized after reinitiation of cyclosporine. Two responders were managed with cyclosporine alone. The single patient who did not respond to cyclosporine developed progressive liver failure, underwent orthotopic liver transplantation, and subsequently died of disseminated cytomegalovirus infection. Cyclosporine was generally well tolerated and none of the patients developed renal insufficiency. These data and review of 11 cases in the literature show that cyclosporine can induce remission of liver disease in patients with autoimmune hepatitis who are refractory to corticosteroids.

Adult↗
PubMed2005

Antiphospholipid syndrome in combination with autoimmune hepatitis.

Autoimmune hepatitis is rarely described in combination with antiphospholipid syndrome. So far, only cases have been presented where the secondary antiphospholipid syndrome occurred as an effect of autoimmune hepatitis. We report on a 56-year-old Caucasian female with a history of thrombosis, thrombocytopenia and the detection of anti-cardiolipin antibodies. Interestingly, a few years later the patient developed liver failure with highly elevated liver enzymes. The immunological antibody pattern verified the diagnosis of type I autoimmune hepatitis, and immunosuppressive therapy led to the recovery of the patient. Thus, we present the first case of autoimmune hepatitis as a consequence of primary antiphospholipid syndrome.

Antiphospholipid Syndrome↗
PubMed1994

Autoimmune cholangiopathy: the result of consecutive primary biliary cirrhosis and autoimmune hepatitis?

Autoimmune cholangiopathy is a recently proposed entity that describes a specific group of patients presenting overlapping features of primary biliary cirrhosis and autoimmune hepatitis, i.e., clinical and/or biochemical cholestasis, high titer antinuclear antibody, negative antimitochondrial antibody, and elevated immunoglobulin G. Liver histology shows primary biliary cirrhosis coexisting with varying degrees of parenchymal inflammation. In addition, these patients achieve remission on corticosteroid therapy. The patient in this report fulfilled the above criteria. However, preceding the autoimmune cholangitis stage, a typical antimitochondrial antibody-positive primary biliary cirrhosis was documented with favorable response to ursodeoxycholic acid treatment. Twenty months later, the patient developed autoimmune hepatitis with elevated aspartate aminotransferase and immunoglobulin G and high titer antinuclear antibody as well as corticosteroid dependency, whereas the antimitochondrial antibody disappeared. The patient's sera initially showed reactivity to three mitochondrial proteins, the 74-, 64-, and 56-kilodalton autoantigens of the 2-oxo acid dehydrogenase complexes, which was characteristic of primary biliary cirrhosis. After developing autoimmune hepatitis, reactivity to the 74- and 64-kilodalton antigens disappeared, whereas reactivity to the 56-kilodalton antigen decreased to low levels. Autoimmune cholangitis and probably other forms of the overlap syndrome may result from the association of two diseases: primary biliary cirrhosis and autoimmune hepatitis.

Antibodies, Antinuclear↗
PubMedDate not supplied

Pregnancy and autoimmune hepatitis.

Autoimmune hepatitis is a rare condition that is more common among women than men. An association between pregnancy and autoimmune hepatitis is rare. This clinical scenario requires the gastroenterologist and hepatologist to have a profound knowledge of clinical course counseling and medical management. The prognosis in well-controlled and closely monitored patients is good. In this review, we discuss the most important aspects of autoimmune hepatitis and pregnancy as part of the Symposium on Liver and Pregnancy, co-sponsored by the Mexican Association of Hepatology and the Mexican Association of Gynecologists and Obstetrics.

Female↗
PubMed2006

[Analysis of prognostic factors in patients with autoimmune hepatitis].

Autoimmune hepatitis (AIH) is the chronic inflammatory liver disease of unknown etiology, demonstrating progressive injury of liver, finally leading to insufficiency of this organ. Possible association between clinical forms, course and prognosis of AIH has been considered recently. Aim of this study is to evaluate autoimmune hepatitis exacerbation risk in respect to epidemiological, clinical and laboratory signs demonstrated in patients with freshly diagnosed AIH. Retrospective study included 42 patients hospitalized in Liver Unit of Department of Infectious Diseases Medical University of Bialystok between 1999 and 2003, suspected for autoimmune hepatitis. In majority of patients onset of the disease was sudden and associated with significant increase of ALT activity. The most frequent, first sign of the disease was jaundice, that was observed in over 50% among patients, pruritus was reported by 1/4 patients. In 12 patients (41.4%) beginning of the disease was oligosymptomatic, i.e. without jaundice and pruritus. These patients demonstrated significantly lower ALT activity, bilirubin and gamma-globulin concentrations. Moreover prevalence of antinuclear antibodies in this group was less frequent, and frequency of hospitalization because of AIH exacerbations was also lower. Concluding, oligosynptomatic course of the disease accompanied by lower ALT activity can be related to better prognosis of AIH, and indicate mild course associated with low risk of exacerbations. On the c:her hand faster progression of the disease can be expected in younger patients demonstrating significant increase of ALT activity at the moment of initial diagnosis.

Adult↗
PubMed1997

A 12-year-old girl with antimitochondrial antibody-positive autoimmune hepatitis.

Autoimmune hepatitis is rare and, in childhood, is typically associated with either the nuclear and/or smooth muscle antibody or with the liver kidney microsomal type 1 antibody. Antimitochondrial antibodies, which are considered diagnostic of primary biliary cirrhosis, have not been described in the paediatric age. We report for the first time a 12-year-old girl with antimitochondrial-antibody-positive autoimmune hepatitis. Antimitochondrial antibody positivity was determined by immunofluorescence and immunoblot. The patient's age, clinical, biochemical and histological features and response to immunosuppressive treatment support the diagnosis of autoimmune hepatitis.

Aspartate Aminotransferases↗
PubMed2001

Autoimmune hepatitis.

Autoimmune hepatitis is one of the causes of chronic progressive liver disease in childhood. Here we report 14 cases with clinical findings, therapeutic management and prognosis, in order to define the course of the disease. Diagnosis of autoimmune hepatitis was done with the presence of at least one of these autoantibodies; antinuclear antibody, smooth muscle antibody, liver-kidney microsomal type 1 antibody, and perinuclear antineutrophilic cytoplasmic antibody. Patients were seen every 3 to 6 months. After doing a complete physical examination, biochemical parameters and autoantibodies determined at each visit. Mean age at diagnosis was 10.9 +/- 2.6 years (range, 7-15.5 years) and female to male ratio was 1:3. Thirteen patients had jaundice and all had high levels of ALT, AST and gammaglobulin. Hepatomegaly was found in 71.4% and splenomegaly in 64.3% of the patients. All patients were classified as type 1 autoimmune hepatitis. Liver biopsies revealed severe active hepatitis with mononuclear cell infiltration in portal areas, piecemeal necrosis. Drug therapy consisted of prednisone (2 mg/kg/day) per oral at the beginning, and addition of azathioprine (1.5 mg/kg/day) per oral at the 3rd-6th month with slow tapering of prednisone in 12 children. Both drugs were started together to two patients. Follow-up period was 30.7 +/- 15.6 months (range, 12-72 months). Sustained normalization of ALT could not be obtained with tapering doses of prednisone alone. Decrease in ALT levels did not correlate with disappearance of serum autoantibodies. None of the patients showed decompensation of liver disease. Azathioprine administration is necessary to decrease prednisone dose and to maintain a sustained normal transaminase values.

Adolescent↗
PubMed2001

Glutathione-S-Transferase M1 null genotype in autoimmune hepatitis.

Autoimmune hepatitis is associated with genes located in the major histocompatibility complex. The search for genes at other loci that may play a role in disease susceptibility and/or severity is an area of active investigation in autoimmune liver diseases. Genes for glutathione-S-transferases, enzymes that are widely distributed and collectively metabolize carcinogens, pollutants, drugs, and a broad spectrum of harmful, foreign compounds have been associated with liver disease. The objective of this study was to search for a relationship between the glutathione-S-transferase Ml null genotype and autoimmune hepatitis using polymerase chain reaction analysis. The findings indicate that the frequency of the null genotype is not increased in patients with autoimmune hepatitis when compared to control subjects. These results coupled with similar ones in primary biliary cirrhosis do not support a role for this mutation in autoimmune liver disease.

Adolescent↗
PubMed1995

Evidence for spontaneous immunosuppression in autoimmune hepatitis.

Autoimmune hepatitis (AIH) runs a variable clinical course. Slow disease progression or even spontaneous remissions can be observed and suggest that the autoimmune process can, at least to a certain extent, be controlled by regulatory elements of the patient's own immune system. In experimental autoimmune hepatitis (EAH) spontaneous recovery is regularly observed and associated with antigen-specific and antigen-nonspecific suppression. The aim of the current study was to search for similar immunoregulatory phenomena in patients with AIH. We examined T-cell reactivity to soluble human liver antigens in 11 patients with active autoimmune hepatitis and 30 patients with other liver diseases (chronic viral hepatitis, primary biliary cirrhosis, fatty liver). T-cell reactivity to liver antigens was almost exclusively confined to the AIH patients. In 9 of these 11 patients we were able to compare the T-cell response in active untreated disease, directly after initiation of immunosuppressive therapy and in remission, at which point T-cell reactivity was found to be markedly reduced. Addition of irradiated peripheral blood cells from the active disease phase or the remission phase to the responding cells taken before treatment showed that in eight of the nine patients there was marked suppression of the liver-specific T-cell response by cells from the remission phase. Study of the in vivo immune responsiveness by giving a tetanus toxoid booster immunization to nine patients with active untreated AIH as well as healthy controls and patients with other liver diseases showed that none of the patients with AIH showed a response to the tetanus immunization, whereas almost all the other patients showed marked responses.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2000

[Massive hemorrhage due to esophageal varices as a form of presentation of autoimmune hepatitis].

Autoimmune hepatitis is a necro-inflammatory disease of unknown etiology. An acute or fulminant presentation is rare. We report a 18-year-old man presented with acute variceal hemorrhage. The patient was treated with endoscopic sclerotherapy, somatostatin, balloon tamponade and TIPS. The patient died within a week of massive bleeding and hepatic failure. This patient's history strongly suggests that autoimmune hepatitis can be presented as fulminant hemorrhage. The importance of diagnosis and treatment is emphasized to improve prognosis.

Acute Disease↗