1095 DEMONSTRATION OF AN HEPATOCYTE CANALICULAR LOCALIZATION OF CYP2D6, THE TARGET OF LIVER KIDNEY MICROSOME TYPE 1 AUTOANTIBODIES, MARKERS OF TYPE 2 AUTOIMMUNE HEPATITIS

2010 ◽  
Vol 52 ◽  
pp. S423
Author(s):  
E. Beleoken ◽  
F. El Rabaa ◽  
B. Grosse ◽  
M.Z. Mustafa ◽  
D. Samuel ◽  
...  
Keyword(s):  
Autoimmune Hepatitis ◽  
Kidney Microsome

Chronic active hepatitis associated with antiliver/kidney microsome antibody type 1: A second type of “autoimmune” hepatitis

Hepatology ◽  
1987 ◽  
Vol 7 (6) ◽  
pp. 1333-1339 ◽  
Author(s):  
Jean-Claude Homberg ◽  
Nisen Abuaf ◽  
Olivier Bernard ◽  
Shamsul Islam ◽  
Fernando Alvarez ◽  
...  
Keyword(s):  
Autoimmune Hepatitis ◽  
Chronic Active Hepatitis ◽  
Kidney Microsome

scholarly journals Autoimmune Hepatitis: Serum Autoantibodies in Clinical Practice

2021 ◽  
Author(s):  
Benedetta Terziroli Beretta-Piccoli ◽  
Giorgina Mieli-Vergani ◽  
Diego Vergani
Keyword(s):  
Autoimmune Hepatitis ◽  
Current Knowledge ◽  
Reference Method ◽  
Smooth Muscle Antibody ◽  
Autoimmune Sclerosing Cholangitis ◽  
Specific Autoantibody ◽  
Circulating Autoantibodies ◽  
International Autoimmune Hepatitis Group

AbstractCirculating autoantibodies are a key diagnostic tool in autoimmune hepatitis (AIH), being positive in 95% of the cases if tested according to dedicated guidelines issued by the International Autoimmune Hepatitis Group. They also allow the distinction between type 1 AIH, characterized by positive anti-nuclear and/or anti-smooth muscle antibody, and type 2 AIH, characterized by positive anti-liver kidney microsomal type 1 and/or anti-liver cytosol type 1 antibody. Anti-soluble liver antigen is the only AIH-specific autoantibody, and is found in 20–30% of both type 1 and type 2 AIH. Anti-neutrophil cytoplasmic antibody is frequently positive in type 1 AIH, being associated also with inflammatory bowel disease and with primary/autoimmune sclerosing cholangitis. The reference method for autoantibody testing remains indirect immunofluorescence on triple tissue (rodent liver, kidney and stomach), allowing both the detection of the majority of liver-relevant reactivities, including those autoantibodies whose molecular target antigens are unknown. Of note, the current knowledge of the clinical significance of autoantibodies relies on studies based on this technique. However, immunofluorescence requires trained laboratory personnel, is observer-dependent, and lacks standardization, leading to ongoing attempts at replacing this method with automated assays, the sensitivity, and specificity of which, however, require further studies before they can be used as a reliable alternative to immunofluorescence; currently, they may be used as complementary to immunofluorescence.

Different immunogenetic background in autoimmune hepatitis type 1, type 2 and autoimmune sclerosing cholangitis

2002 ◽  
Vol 36 ◽  
pp. 156 ◽  
Author(s):  
James A. Underhill ◽  
Yun Ma ◽  
Dimitrios P. Bogdanos ◽  
Paul Cheeseman ◽  
Giorgina Mieli-Vergani ◽  
...  
Keyword(s):  
Autoimmune Hepatitis ◽  
Sclerosing Cholangitis ◽  
Autoimmune Sclerosing Cholangitis ◽  
Autoimmune Hepatitis Type 1

Autoimmune Hepatitis

2015 ◽  
Author(s):  
Jennifer Y Chen ◽  
Karin L. Andersson
Keyword(s):  
Autoimmune Hepatitis ◽  
Scoring System ◽  
Elevated Serum ◽  
Smooth Muscle Antibody ◽  
Treatment Regimens ◽  
Recommended Treatment ◽  
Serum Transaminases ◽  
Disease Subtypes

Autoimmune hepatitis (AIH) is defined by elevated serum transaminases along with the presence of one or more characteristic serum autoantibodies, including antinuclear antibody (ANA), anti–smooth muscle antibody (ASMA), and anti–liver-kidney microsomal type 1 antibody (anti-LKM-1); elevated levels of serum immunoglobulin G (IgG); interface hepatitis on histology; and responsiveness to immunosuppressive therapy. AIH has been classified into two disease subtypes based on serologic markers: type 1, which is characterized by the presence of either ANA or ASMA, and type 2, which is characterized by the presence of either anti-LKM-1 or anti–liver-cytosol antibody type 1 (anti-LC-1). This review addresses the epidemiology, natural history, pathogenesis, and management of AIH, as well as ongoing challenges. Several recent advances are highlighted, including the creation of a simplified diagnostic scoring system and the use of budesonide for AIH treatment. Figures show the pathology of AIH, treatment approach of moderate to severe AIH with combination therapy, azathioprine metabolism, and management of treatment outcomes. Tables list a comparison of type 1 and type 2 AIH, antibodies in AIH, the revised diagnostic scoring system (including a simplified version), indications for treatment in AIH, recommended treatment regimens for AIH by the American Association for the Study of Liver Diseases, and adverse effects associated with therapy for AIH. This review contains 4 highly rendered figures, 7 tables, and 146 references.

Type 1 and type 2 autoimmune hepatitis in adults share the same clinical phenotype

2015 ◽  
Vol 41 (12) ◽  
pp. 1281-1287 ◽  
Author(s):  
P. Muratori ◽  
C. Lalanne ◽  
A. Fabbri ◽  
F. Cassani ◽  
M. Lenzi ◽  
...  
Keyword(s):  
Autoimmune Hepatitis ◽  
Clinical Phenotype

scholarly journals Liver/kidney microsomal antibody type 1 and liver cytosol antibody type 1 concentrations in type 2 autoimmune hepatitis

Gut ◽  
1998 ◽  
Vol 42 (5) ◽  
pp. 721-726 ◽  
Author(s):  
L Muratori ◽  
M Cataleta ◽  
P Muratori ◽  
M Lenzi ◽  
F B Bianchi
Keyword(s):  
Liver Disease ◽  
Disease Activity ◽  
Autoimmune Hepatitis ◽  
Immunosuppressive Treatment ◽  
Serum Samples ◽  
Liver Cytosol ◽  
Enhanced Chemiluminescence ◽  
Microsomal Antibody

Background—Liver/kidney microsomal antibody type 1 (LKM1) and liver cytosol antibody type 1 (LC1) are the serological markers of type 2 autoimmune hepatitis (AIH).Aims—Since LKM1 and LC1 react against two distinct liver specific autoantigens (cytochrome P450IID6 (CYP2D6) and a 58 kDa cytosolic polypeptide respectively), the aim was to see whether LKM1 and LC1 concentrations correlate with liver disease activity.Patients—Twenty one patients with type 2 AIH were studied.Methods—All sera were tested by indirect immunofluorescence, counterimmunoelectrophoresis, and immunoblotting visualised by enhanced chemiluminescence. To evaluate LKM1 and LC1 levels, the 50 kDa microsomal reactivity (corresponding to CYP2D6) and the 58 kDa cytosolic reactivity were quantified by densitometric analysis.Results—Seven patients were positive for LKM1, nine for LC1, and five for both. Serial serum samples at onset and during immunosuppressive treatment were analysed in 13 patients (four positive for LKM1, six positive for LC1 and three positive for both). During remission, LKM1 concentration remained essentially unchanged in six of seven patients, and decreased in only one. Conversely, in two of nine patients, LC1 was completely lost, and, in the remaining seven, LC1 concentration was reduced by more than 50%. After immunosuppression tapering or withdrawal, flare ups of liver necrosis ensued with increasing LC1 concentration, but not LKM1.Conclusions—LC1 concentration, at variance with that of LKM1, parallels liver disease activity, and its participation in the pathogenic mechanisms of liver injury can be hypothesised.

scholarly journals Decompensated Cirrhosis as Presentation of LKM1/LC1 Positive Type 2 Autoimmune Hepatitis in Adulthood. A Rare Clinical Entity of Difficult Management

2021 ◽  
Vol 12 (1) ◽  
pp. 67-75
Author(s):  
Alessandro Granito ◽  
Simona Pascolini ◽  
Chiara Ricci ◽  
Marco Ferronato ◽  
Luigi Muratori ◽  
...  
Keyword(s):  
Liver Disease ◽  
Autoimmune Hepatitis ◽  
Immunosuppressive Therapy ◽  
Clinical Signs ◽  
Decompensated Cirrhosis ◽  
Positive Type ◽  
Curative Therapy ◽  
End Stage

Background: Autoimmune hepatitis (AIH) is a chronic and aggressive liver disease that rapidly evolves into cirrhosis and end-stage liver disease if not timely diagnosed and treated with immunosuppressive therapy. AIH is classified into type 1 and type 2 according to the autoantibody pattern, with smooth muscle antibodies and/or antinuclear antibodies as serological markers of AIH-1, while antiliver cytosol antibody type 1 and/or antiliver/kidney microsomal antibody type 1 characterize type 2 AIH, which mainly affects children, including infants, and adolescents. Case Summary: We describe a case of type 2 AIH, clinically onset in a 34-year-old woman with decompensated cirrhosis. Only a thorough analysis of the autoantibody profile allowed for a diagnosis of an AIH-2 evolved into cirrhosis. The patient received a moderate corticosteroid therapy without achieving optimal disease control. We discuss the controversial decision of whether or not to treat the patient with immunosuppressive therapy, which should be balanced with the potential risk of infectious and other complications. A review of the literature on the management of patients with autoimmune cirrhosis is also presented. Conclusions: AIH-2 can be clinically onset in adult patients with cirrhosis and its complications, without being preceded by major clinical signs. Due to the difficult management of cirrhosis with immunosuppressive treatments, a patient-tailored strategy with a case-by-case approach is needed to prevent major complications such as infections, potentially precluding liver transplantation the only curative therapy.

Presentation and Outcomes of Autoimmune Hepatitis Type 1 and Type 2 in Children

2020 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Isaiah G. Roepe ◽  
John M. Vierling ◽  
John A. Goss ◽  
Tamir Miloh
Keyword(s):  
Autoimmune Hepatitis ◽  
Autoimmune Hepatitis Type 1

scholarly journals Clinical and biochemical features of autoimmune hepatitis in 36 pediatric patients

2006 ◽  
Vol 43 (1) ◽  
pp. 45-49 ◽  
Author(s):  
Maria Angela Bellomo-Brandão ◽  
Elizete Aparecida Lomazi da Costa-Pinto ◽  
Adriana Maria Alves De Tommaso ◽  
Gabriel Hessel
Keyword(s):  
Autoimmune Hepatitis ◽  
Pediatric Patients ◽  
Clinical Manifestations ◽  
Clinical History ◽  
First Year ◽  
Duration Of Symptoms ◽  
Similar Disease ◽  
Biochemical Features

BACKGROUND: Few studies on autoimmune hepatitis have enrolled non-Caucasian groups. AIMS: To evaluate Brazilian children with type 1 and 2 autoimmune hepatitis regarding outcome and clinical and biochemical parameters. PATIENTS AND METHODS: Thirty-six patients were submitted to a protocol that evaluated the clinical history, physical and biochemical data, and the course of the disease. Twenty-four children had type 1 autoimmune hepatitis, seven had type 2 and five had unclassified autoimmune hepatitis. Most patients were females (77%), with a median age at diagnosis of 11 years, and the median duration of symptoms was 5.5 and 8 months for types 1 and 2, respectively. Jaundice and choluria were the most common clinical manifestations. RESULTS: Treatment with azathioprine and prednisone was successful in patients with type 1 and 2 autoimmune hepatitis. AST and ALT decreased after 4 to 8 weeks of treatment compared to pretreatment levels in type 1 autoimmune hepatitis. Increased GGT values returned to pretreatment levels after 1 year in the two types. Three patients died and three other patients underwent liver transplantation. CONCLUSIONS: Non-Caucasian children had a similar disease when compared to Caucasian ones with autoimmune hepatitis. Increased levels of GGT during the first year of treatment should not be the only parameter for the indication of cholangiopathy.

Sign in / Sign up

Export Citation Format

Share Document