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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
Published on: December 15, 2011
Hepatic autoantigens in patients with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy.
P Obermayer-Straub1, J Perheentupa, S Braun
1Department of Gastroenterology and Hepatology, Hanover Medical School, Germany.
Gastroenterology
|August 28, 2001
Summary
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) hepatitis is linked to specific autoantibodies, particularly anti-CYP1A2, which is highly specific but not sensitive. Autoimmune hepatitis and APECED hepatitis have distinct autoantibody targets.
Area of Science:
- Immunology
- Hepatology
- Genetics
Background:
- Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) results from AIRE gene mutations, causing candidiasis and autoimmune conditions.
- Hepatitis is a significant manifestation of APECED syndrome.
Purpose of the Study:
- To identify diagnostic autoantibodies for APECED-associated hepatitis.
- To screen for autoantibodies against known idiopathic autoimmune hepatitis markers and cytochrome P450 enzymes.
Main Methods:
- Sera from 64 APECED patients were analyzed.
- Screening involved indirect immunofluorescence, Western blot, Ouchterlony assay, ELISA, and immunoprecipitation.
- Autoantibodies against 10 cytochrome P450s were investigated.
Main Results:
- Anti-liver microsomal antibodies were found in 50% of APECED hepatitis cases.
- CYP1A2 and CYP2A6 were identified as autoantigens; anti-CYP1A2 was highly specific but insensitive for APECED hepatitis.
- Anti-CYP1A2 and anti-CYP2A6 were absent in autoimmune hepatitis and control groups.
Conclusions:
- Anti-CYP1A2 autoantibodies are specific markers for APECED hepatitis.
- Autoimmune hepatitis and APECED hepatitis target different autoantibodies, indicating distinct disease mechanisms.
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