Autoimmune hepatitis (AIH) is a rare disease, characterized by female predominance, hypergammaglobulinemia, autoantibodies, association with HLA DR3 and HLA DR4 and a good response to immunosuppression.
Autoimmune hepatitis (AIH) is usually a chronic portal-based hepatitis with prominent plasma cells. Although necroinflammatory activity throughout the lobule is described, centrilobular necrosis (CN) is only rarely the predominant pattern of injury.
Types
autoimmune hepatitis type 1 is characterized by anti-nuclear (ANA) and/or anti-smooth muscular (SMA) autoantibodies.
autoimmune hepatitis type 2 is characterized by liver/kidney microsomal autoantibodies (LKM).
autoimmune hepatitis type 3 may be distinguished by autoantibodies to soluble liver proteins (SLA) or the liver pancreas antigen (LP).
The autoimmune hepatitis type 2 (AIH-2) affects predominantly pediatric patients and is characterized by a more severe clinical course, a higher frequency of relapse under immunosuppressive treatment and a more frequent progression to cirrhosis.
The autoimmune hepatitis type 1 and autoimmune hepatitis type 3 show a higher age of onset and a better long-term response to immunosuppressive treatment.
Synopsis
hypergammaglobulinemia
multifocal hepatic necrosis with bridging in the acute stage
aggressive hepatitis with mononuclear cell infiltration or macronodular cirrhosis in the late stages
chronic portal-based hepatitis with prominent plasma cells
lobular necroinflammatory activity
centrilobular necrosis
Lesional patterns
autoimmune active chronic hepatitis
autoimmune panlobular hepatitis
autoimmune bridging mecrosis
autoimmune massive hepatic necrosis
Types
autoimmune hepatitis type 1 (lupoid hepatitis) (anti-smooth muscle antibody, antinuclear antibodies)
autoimmune hepatitis type 2 (LKM1-associated autoimmune hepatitis or anti-LKM1 chronic active hepatitis)
autoimmune hepatitis type 3 (soluble liver antigen, liver/pancreas antigen)
Associations
autoimmune disease
- celiac disease
- primary sclerosing cholangitis
- inflammatory bowel diseases (IBDs) (9123955, 8447289)
- familial autoimmune enteropathy (7996356)
- cryoglobulinaemia
- primary biliary cirrhosis (16416210)
- autoimmune enteric leiomyositis (15948126)
miscellaneous
Overlaps
primary biliary cirrhosis (PBC)
primary sclerosing cholangitis
chronic hepatitis C-immune predominant
chronic hepatitis C-immune predominant
autoimmune cholangitis
cryptogenic chronic hepatitis
References
Histology of autoimmune hepatitis and its variants. Guindi M. Clin Liver Dis. 2010 Nov;14(4):577-90. PMID: 21055683
Krawitt EL. Autoimmune hepatitis. N Engl J Med. 2006 Jan 5;354(1):54-66. PMID: 16394302
Brunt EM, Di Bisceglie AM. Histological changes after the use of mycophenolate mofetil in autoimmune hepatitis. Hum Pathol. 2004 Apr;35(4):509-12. PMID: 15116334
Misdraji J, Thiim M, Graeme-Cook FM. Autoimmune hepatitis with centrilobular necrosis. Am J Surg Pathol. 2004 Apr;28(4):471-8. PMID: 15087666
Obermayer-Straub P, Strassburg CP, Manns MP. Autoimmune hepatitis. J Hepatol. 2000;32(1 Suppl):181-97. PMID: 10728804
Alvarez F, Berg PA, Bianchi FB, Bianchi L, Burroughs AK, Cancado EL, Chapman RW, Cooksley WG, Czaja AJ, Desmet VJ, Donaldson PT, Eddleston AL, Fainboim L, Heathcote J, Homberg JC, Hoofnagle JH, Kakumu S, Krawitt EL, Mackay IR, MacSween RN, Maddrey WC, Manns MP, McFarlane IG, Meyer zum Buschenfelde KH, Zeniya M, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol. 1999 Nov;31(5):929-38. PMID: 10580593
Burgart LJ, Batts KP, Ludwig J, Nikias GA, Czaja AJ. Recent-onset autoimmune hepatitis. Biopsy findings and clinical correlations. Am J Surg Pathol. 1995 Jun;19(6):699-708. PMID: 7755156
Homberg JC, Abuaf N, Bernard O, Islam S, Alvarez F, Khalil SH, Poupon R, Darnis F, Levy VG, Grippon P, et al. Chronic active hepatitis associated with antiliver/kidney microsome antibody type 1: a second type of "autoimmune" hepatitis. Hepatology. 1987 Nov-Dec;7(6):1333-9. PMID: 3679093