PMID- 25466500
OWN - NLM
STAT- MEDLINE
DCOM- 20150831
LR  - 20141216
IS  - 1538-3199 (Electronic)
IS  - 1538-3199 (Linking)
VI  - 44
IP  - 11
DP  - 2014 Dec
TI  - Autoimmune hepatitis: a classic autoimmune liver disease.
PG  - 341-6
LID - S1538-5442(14)00111-4 [pii]
LID - 10.1016/j.cppeds.2014.10.005 [doi]
AB  - AIH is characterized by chronic inflammation of the liver, interface hepatitis, 
      hypergammaglobulinemia, and production of autoantibodies. Based on the nature of 
      the serum autoantibodies, two types of AIH are recognized: type 1 (AIH-1), 
      positive for ANA and/or anti-smooth muscle antibody, and type 2 (AIH-2), defined 
      by the positivity for anti-liver kidney microsomal type 1 antibody or for 
      anti-liver cytosol type 1 antibody. AIH demonstrates a female preponderance with 
      the female-to-male ratio of 4:1 in AIH-1 and 10:1 in AIH-2. Several genes confer 
      susceptibility to AIH and influence clinical manifestation, response to 
      treatment, and overall prognosis. Most are located within the human leukocyte 
      antigen (HLA) region, which is involved in the presentation of antigenic peptides 
      to T cells and thus in the initiation of adaptive immune responses. The strongest 
      associations are found within the HLA-DRB1 locus. In patients with increased 
      genetic susceptibility to AIH, immune responses to liver autoantigens could be 
      triggered by molecular mimicry. Because of molecular mimicry, different 
      environmental agents, drugs, and viruses might produce AIH. In AIH, T cells are 
      numerically and functionally impaired, permitting the perpetuation of effector 
      immune responses with ensuing persistent liver destruction. AIH is rare but 
      highly treatable inflammatory condition of the liver. Subclinical and 
      asymptomatic disease is common. AIH therefore needs to be considered in the 
      differential diagnosis of all patients with elevated liver enzymes. Clinical 
      response to immunosuppressive therapy is characteristic and supports the 
      diagnosis.
CI  - Copyright (c) 2014 Mosby, Inc. All rights reserved.
FAU - Moy, Libia
AU  - Moy L
AD  - Division of Pediatric Gastroenterology, New York University School of Medicine, 
      New York, NY.
FAU - Levine, Jeremiah
AU  - Levine J
AD  - Division of Pediatric Gastroenterology, New York University School of Medicine, 
      New York, NY. Electronic address: jeremiah.levine@nyumc.org.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20141126
PL  - United States
TA  - Curr Probl Pediatr Adolesc Health Care
JT  - Current problems in pediatric and adolescent health care
JID - 101134613
RN  - 0 (Adrenal Cortex Hormones)
RN  - 0 (Anti-Inflammatory Agents)
RN  - 0 (Autoantibodies)
RN  - 0 (Immunosuppressive Agents)
SB  - IM
MH  - Adrenal Cortex Hormones/*therapeutic use
MH  - Anti-Inflammatory Agents/*therapeutic use
MH  - Autoantibodies/immunology
MH  - Child
MH  - Diagnosis, Differential
MH  - Hepatitis, Autoimmune/*immunology/physiopathology/therapy
MH  - Humans
MH  - Immunosuppressive Agents/*therapeutic use
MH  - Liver/*immunology/pathology
MH  - Liver Function Tests/methods
MH  - Prognosis
MH  - Randomized Controlled Trials as Topic
MH  - T-Lymphocytes/immunology
EDAT- 2014/12/04 06:00
MHDA- 2015/09/01 06:00
CRDT- 2014/12/04 06:00
PHST- 2014/12/04 06:00 [entrez]
PHST- 2014/12/04 06:00 [pubmed]
PHST- 2015/09/01 06:00 [medline]
AID - S1538-5442(14)00111-4 [pii]
AID - 10.1016/j.cppeds.2014.10.005 [doi]
PST - ppublish
SO  - Curr Probl Pediatr Adolesc Health Care. 2014 Dec;44(11):341-6. doi: 
      10.1016/j.cppeds.2014.10.005. Epub 2014 Nov 26.