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Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.

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Autoimmune hepatitis classification

Introduction

Overview

Overview

According to European Association for the Study of the Liver, There are three types of autoimmune hepatitis based on the types of antibodies present- AIH type 1, AIH type 2, AIH type 3. Overlap syndrome is a type of syndrome found in a patient who presents with the features of AIH, PBC-AIH or PSC-AIH.

Classification

Classification

According to European Association for the Study of the Liver, there are three types of autoimmune hepatitis based upon the types of antibodies present:[1][2][3][4][5]

Autoimmune hepatitis Type-1:

  • Common type of AIH (accounts almost for 90% of AIH cases) is type one.
  • Presents at any age and prevalent worldwide.
  • Antibodies like ANA (antinuclear antibodies), SMA (smooth muscle antibodies) or anti-SLA/LP (soluble liver antigen/liver pancreas antibodies) are characteristics.
  • Antiactin antibodies are more specific.
  • Association with HLA (human leukocyte antigens) DR3, DR4, and DR13 is present.
  • Treatment failure is rare but if relapse occurs after withdrawal of treatment and long-term maintenance therapy is given occasionally.

Autoimmune hepatitis Type 2:

  • Accounts for 10% of AIH cases.
  • Presents usually in childhood and young adulthood and prevalent worldwide but rare in North America.
  • Antibodies like anti-LKM1(liver/kidney microsomal antibody type 1), anti-LC1(antibodies against liver cytosol type 1 antigen) and rarely anti-LKM3(liver/kidney microsomal antibody type 3) are characteristics.
  • Anti–LKM-1 and anti–LC-1 are more specific.
  • Association with HLA DR3 and DR7.
  • Treatment failure is common and frequent relapse rates after withdrawal of treatment, and long-term maintenance therapy commonly is given.

Autoimmune hepatitis Type 3:

  • AIH type 3 is similar to AIH type 1 but presents more severe.
  • SLA/LP (soluble liver antigen/liver pancreas antibodies) is characteristics.
  • Ro52-antibody positive

Overlap syndrome:

Patients who present with the features of primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC) along with the features of AIH, PBC-AIH or PSC-AIH.[6][7]

Classification of overlap syndrome
Name of syndrome Dominant disease Recessive disease
PBC-AIH overlap PBC AIH
PSC-AIH overlap PSC AIH
AIH-PBC overlap AIH PBC
AIH-PSC overlap AIH PSC
AIH, autoimmune hepatitis; PBC, primary biliary cholangitis; PSC, primary sclerosing cholangitis.
References

References

  1. ↑ Sener AG (2015). “Autoantibodies in autoimmune liver diseases”. APMIS. 123 (11): 915–9. doi:10.1111/apm.12442. PMID 26359647.
  2. ↑ “EASL Clinical Practice Guidelines: Autoimmune hepatitis”. J. Hepatol. 63 (4): 971–1004. 2015. doi:10.1016/j.jhep.2015.06.030. PMID 26341719.
  3. ↑ Floreani A, Niro G, Rosa Rizzotto E, Antoniazzi S, Ferrara F, Carderi I, Baldo V, Premoli A, Olivero F, Morello E, Durazzo M (2006). “Type I autoimmune hepatitis: clinical course and outcome in an Italian multicentre study”. Aliment. Pharmacol. Ther. 24 (7): 1051–7. doi:10.1111/j.1365-2036.2006.03104.x. PMID 16984499.
  4. ↑ Liberal R, Mieli-Vergani G, Vergani D (2013). “Clinical significance of autoantibodies in autoimmune hepatitis”. J. Autoimmun. 46: 17–24. doi:10.1016/j.jaut.2013.08.001. PMID 24016388.
  5. ↑ Krawitt EL (2006). “Autoimmune hepatitis”. N. Engl. J. Med. 354 (1): 54–66. doi:10.1056/NEJMra050408. PMID 16394302.
  6. ↑ Kuiper EM, Zondervan PE, van Buuren HR (2010). “Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome”. Clin. Gastroenterol. Hepatol. 8 (6): 530–4. doi:10.1016/j.cgh.2010.03.004. PMID 20304098.
  7. ↑ van Buuren HR, van Hoogstraten H, Terkivatan T, Schalm SW, Vleggaar FP (2000). “High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis”. J. Hepatol. 33 (4): 543–8. PMID 11059858. Vancouver style error: initials (help)

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Manpreet Kaur, MD [2]