Autoimmune Hepatitis

2015 ◽  
Author(s):  
Jennifer Y Chen ◽  
Karin L. Andersson

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.

Author(s):  
Benedetta Terziroli Beretta-Piccoli ◽  
Giorgina Mieli-Vergani ◽  
Diego Vergani

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.


2020 ◽  
Author(s):  
Amutha Anandakumar ◽  
Pradeep A Praveen ◽  
Christine W. Hockett ◽  
Toan C Ong ◽  
Elizabeth T Jensen ◽  
...  

QJM ◽  
2006 ◽  
Vol 99 (12) ◽  
pp. 871-876 ◽  
Author(s):  
J. West ◽  
J. Brousil ◽  
A. Gazis ◽  
L. Jackson ◽  
P. Mansell ◽  
...  

PLoS ONE ◽  
2018 ◽  
Vol 13 (12) ◽  
pp. e0208905 ◽  
Author(s):  
Noel Pabalan ◽  
Raphael Enrique Tiongco ◽  
Jefferyl Kae Pandac ◽  
Noemi Anne Paragas ◽  
Shamar Lo Lasta ◽  
...  

2014 ◽  
Vol 24 (8) ◽  
pp. 1455-1460 ◽  
Author(s):  
Keisuke Ashihara ◽  
Tomohito Tanaka ◽  
Risa Maruoka ◽  
Yoshihiro J. Ono ◽  
Akiko Tanabe ◽  
...  

ObjectiveType 1 endometrial cancer (EC) is typically sex hormone sensitive; however, most women diagnosed with EC have already gone through menopause. Several studies have reported that the postmenopausal ovary is hormonally active, and estradiol (E2) production from the ovaries persists for as much as 10 years beyond menopause. The aim of this study was to evaluate whether sex steroid production from the ovaries contributes to the pathogenesis of type 1 EC.Materials and MethodsThis was a prospective study of 53 women treated for EC (28 cases of type 1 disease and 25 cases of type 2 disease). Serum specimens were collected from the peripheral and ovarian veins of participants undergoing bilateral oophorectomy. The sex steroid hormone levels and hormonal milieu on cervical cytology were evaluated as maturation value (MV). In addition, the degree of stromal hyperplasia of the ovary was evaluated histologically.ResultsAlthough the E2 levels of the peripheral veins did not show any significant differences [8.2 (5.1–12.4) vs 7.4 (5.1–11.7) pg/mL, respectively; P < 0.05], the patients with type 1 EC had a higher E2 level in the ovarian vein than those with type 2 EC [25 (13.8–42.5) vs 15 (10.0–23.0) pg/mL, respectively; P < 0.05]. There were also no significant differences in the rate of moderate to marked hyperplasia of the ovarian stroma between the groups; however, the thickness of the ovarian cortex demonstrated a correlation with the ovarian E2 level. In addition, the MV displayed a strong correlation with the ovarian E2 level, but not the peripheral E2 level.ConclusionsThe postmenopausal ovary is hormonally active, especially in patients with type 1 EC. The degree of ovarian stromal hyperplasia may (at least in part) contribute to the progression of type 1 EC, and MV may predict the level of E2 production from the ovaries in postmenopausal women.


2002 ◽  
Vol 36 ◽  
pp. 156 ◽  
Author(s):  
James A. Underhill ◽  
Yun Ma ◽  
Dimitrios P. Bogdanos ◽  
Paul Cheeseman ◽  
Giorgina Mieli-Vergani ◽  
...  

2020 ◽  
Vol 22 (5) ◽  
pp. 473-480
Author(s):  
Alla A. Tarasova ◽  
Michail P. Kostinov ◽  
Julia A. Paramonova

This review highlights the problems of vaccine prophylaxis of pneumococcal infection and influenza in patients with type 1 and type 2 diabetes mellitus (CD1 and CD2). The features of pneumococcal infection and influenza in conditions of unsatisfactory compensation of the disease are described. The necessity of protection against pneumococcal infection and influenza in the modern epidemiological situation is justified. Results of efficacy and safety of vaccination, including those in children with type 1 diabetes mellitus, are established. The existing foreign and domestic regulatory documents on vaccine prevention of actual infections are shown.Despite the available documents on vaccination of patients with diabetes, there are problems with inadequate immunization coverage of patients with CD1 and CD2 due to the lack of precise clinical recommendations for the management of these categories both inpatient and outpatient as these recommendations do not cover the questions of vaccine prophylaxis. In Russian studies only the treatment regimens are evaluated without taking into account the fact that vaccines are related to medicines and that the prevention of respiratory infections is a curative measure and can inhibit the development of complications of diabetes.It is necessary to coordinate the work of general practitioners, pediatricians, therapists and the efforts of endocrinologists to increase the coverage of immunization of patients with various forms of diabetes mellitus.


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

2001 ◽  
Vol 2 (2) ◽  
pp. 56
Author(s):  
H.M. Nawawi ◽  
M. Muhajir ◽  
C.K. Yeo ◽  
W.M. Wan Nazaimoon ◽  
K. Yusoff ◽  
...  

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