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Otoimmün hepatit

Year 2019, Volume: 11 Issue: 2, 55 - 67, 29.08.2019

Abstract

ÖZET
Otoimmün hepatit, kadın cinsiyet dominansı, yükselmiş
transaminaz ve immünglobulin G düzeyleri, otoantikor
seropozitifliği ve histolojik düzeyde “interface” hepatit
bulgularıyla karakterize, immün mekanizmalarla ortaya
çıkan kronik bir karaciğer hastalığıdır. Asemptomatik
transaminaz yüksekliğinden siroz ve portal
hipertansiyon bulguları ve fulminan hepatite varan
farklı klinik tablolarla kendini gösterebilir. Hastalığın
erken tanısı konulursa başlanacak immünosüpresif
tedavi ile progresyonun durduğu, histolojik
düzeyde fibrozisin gerilediği ve siroza ilerlemesinin
önlenebileceği gösterilmiştir. Karaciğerdeki otoimmün
inflamasyonu başlatan ve sürdüren mekanizmalar
ve hepatik toleransın neden kaybolduğu hala ortaya
konulamamıştır. Ootoimmün hepatit için spesifik ve
hedeflenmiş tedaviler yoktur ve tedavi hala non-selektif
immünosüpresyona dayanmaktadır. Bu derlemede
otoimmün hepatititin patogenez, tanı ve tedavisindeki
yenilikler ele alınmakta, uluslarası otoimmün hepatit
grubunun konsensus raporları irdelenmekte ve
otoimmün hepatitle örtüşen primer sklerozan kolanjit,
primer biliyer kolanjit sendromlarına ilişkin Paris kriterleri
vurgulanmaktadır.
Otoimmün hepatitin tedavisinde Hamburg 2016
konsensus raporları şu şekildedir. Steroidler remisyonun
indüksiyonunda (normal transaminaz ve IgG) seçilecek
ilaçlardır, ancak optimal doz şemaları tartışmalıdır.
Azatiyopurin remisyonun sürdürülmesinde (idamesi)
seçilecek ilaçtır. Azatiyopurin metabolit düzeyleri dozun
ayarlanmasında ve ilaç alımına uyumsuzluğunun ortaya
konulmasında yardımcı olur. İki yıldan uzun süren stabil
biyokimyasal remisyon ilacın kesilme denemesi için ön
koşuldur, çoğu yaşam boyu tedavi gerektirir. 

SUMMARY
Autoimmune hepatitis is a chronic liver disease caused by
immune mechanisms, characterized by signs of female gender
dominance, elevated transaminase and immunoglobulin
G levels, autoantibody seropositivity and exist of interface
hepatitis at histological level. Asymptomatic transaminase
elevation may present with different clinical manifestations
ranging from cirrhosis and portal hypertension to fulminant
hepatitis. If the disease is diagnosed early, progression is
stopped with the immunosuppressive treatment, fibrosis
regresses at histological level and cirrhosis can be prevented.
Mechanisms that initiate and maintain autoimmune
inflammation in the liver and the absence of hepatic
tolerance have yet to be revealed. There are no specific
and targeted therapies for Autoimmune hepatitis and the
treatment is still based on non-selective immunosuppression.
In this review, the pathogenesis, diagnosis and treatment of
autoimmune hepatitis is discussed, consensus reports of the
international autoimmune hepatitis group are examined and
primary sclerosing cholangitis overlapping with autoimmune
hepatitis and the Paris criteria for primary biliary cholangitis
syndromes are emphasized. Hamburg 2016 consensus
reports on the treatment of autoimmune hepatitis are as
follows. Steroids are drugs to be selected in the induction
of remission (normal transaminase and IgG), but optimal
dosing schemes are controversial. Azathioprine is the drug
to be chosen for the maintenance of remission. Metabolite
levels of Azathioprine help in the adjustment of the dose and
to demonstrate its incompatibility with drug intake. Stable
biochemical remission, which lasts more than two years, is a
prerequisite for drug discontinuation, most of which require
lifelong treatment.

References

  • 1. Manns MP, Lohse AW, Vergani D. Autoimmune hepatitis--Update . J Hepatol 2015;62(1 Suppl):S100-11.
  • 2. Liberal R, Grant CR, Mieli-Vergani G, Vergani D. Autoimmune hepatitis: a comprehensive review. J Autoimmun 2013;41:126-39.
  • 3. Yang F, Wang Q, Bian Z, Ren LL, Jia J, Ma X. Autoimmune hepatitis: East meets west. J Gastroenterol Hepatol 2015;30:1230-6.
  • 4. Czaja AJ. Review article: permanent drug withdrawal is desirable andachievable for autoimmune hepatitis. Aliment Pharmacol Ther 2014;39:1043-58.
  • 5. Heneghan MA (2018). Pathogenesis of autoimmune hepatitis. In: S. Chopra (Ed), UpToDate. Retrieved from http://www.uptodate.com/home
  • 6. Zachou K, Muratori P, Koukoulis GK, Granito A, Gatselis N, Fabbri A, et al. Review article: autoimmune hepatitis -- current management and challenges. Aliment Pharmacol Ther 2013; 38:887- 913.
  • 7. Heneghan MA (2018). Clinical manifestations and diagnosis of autoimmune hepatitis. In: S. Chopra (Ed), UpToDate. Retrieved from http://www.uptodate. com/home
  • 8. Heneghan MA (2018). Extrahepatic manifestations of autoimmune hepatitis. In: S. Chopra (Ed), UpToDate. Retrieved from http://www.uptodate. com/home
  • 9. Czaja AJ. Review article: the management of autoimmune hepatitis beyond consensus guidelines. Aliment Pharmacol Ther 2013;38:343-64.
  • 10. Heneghan MA (2018). Definition and treatment of variants of autoimmune hepatitis. In: Lindor KD. (Ed), UpToDate. Retrieved from http://www. uptodate.com/home
  • 11. Sebode M, Hartl J, Vergani D, Lohse AW & International Autoimmune Hepatitis Group (IAIHG), Autoimmune hepatitis: From current knowledge and clinical practice to future research agenda. Liver International 2018; 38: 15-22
Year 2019, Volume: 11 Issue: 2, 55 - 67, 29.08.2019

Abstract

References

  • 1. Manns MP, Lohse AW, Vergani D. Autoimmune hepatitis--Update . J Hepatol 2015;62(1 Suppl):S100-11.
  • 2. Liberal R, Grant CR, Mieli-Vergani G, Vergani D. Autoimmune hepatitis: a comprehensive review. J Autoimmun 2013;41:126-39.
  • 3. Yang F, Wang Q, Bian Z, Ren LL, Jia J, Ma X. Autoimmune hepatitis: East meets west. J Gastroenterol Hepatol 2015;30:1230-6.
  • 4. Czaja AJ. Review article: permanent drug withdrawal is desirable andachievable for autoimmune hepatitis. Aliment Pharmacol Ther 2014;39:1043-58.
  • 5. Heneghan MA (2018). Pathogenesis of autoimmune hepatitis. In: S. Chopra (Ed), UpToDate. Retrieved from http://www.uptodate.com/home
  • 6. Zachou K, Muratori P, Koukoulis GK, Granito A, Gatselis N, Fabbri A, et al. Review article: autoimmune hepatitis -- current management and challenges. Aliment Pharmacol Ther 2013; 38:887- 913.
  • 7. Heneghan MA (2018). Clinical manifestations and diagnosis of autoimmune hepatitis. In: S. Chopra (Ed), UpToDate. Retrieved from http://www.uptodate. com/home
  • 8. Heneghan MA (2018). Extrahepatic manifestations of autoimmune hepatitis. In: S. Chopra (Ed), UpToDate. Retrieved from http://www.uptodate. com/home
  • 9. Czaja AJ. Review article: the management of autoimmune hepatitis beyond consensus guidelines. Aliment Pharmacol Ther 2013;38:343-64.
  • 10. Heneghan MA (2018). Definition and treatment of variants of autoimmune hepatitis. In: Lindor KD. (Ed), UpToDate. Retrieved from http://www. uptodate.com/home
  • 11. Sebode M, Hartl J, Vergani D, Lohse AW & International Autoimmune Hepatitis Group (IAIHG), Autoimmune hepatitis: From current knowledge and clinical practice to future research agenda. Liver International 2018; 38: 15-22
There are 11 citations in total.

Details

Primary Language Turkish
Subjects Clinical Sciences
Journal Section Review Article
Authors

Nihat Akbayır This is me

Publication Date August 29, 2019
Submission Date March 13, 2019
Published in Issue Year 2019 Volume: 11 Issue: 2

Cite

Vancouver Akbayır N. Otoimmün hepatit. Maltepe tıp derg. 2019;11(2):55-67.