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Liver and intrahepatic bile ducts-nontumor

Hepatitis - noninfectious

Autoimmune hepatitis

Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 2 May 2012, last major update May 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.


● Formerly called lupoid hepatitis
● Indolent or severe
● 70% women, usually ages 20-45 years
● A diagnosis of exclusion
● Associated with HLA-B8 or HLA-DRw3
● 60% have other autoimmune disorders (rheumatoid arthritis, thyroiditis, Sjogren’s syndrome, ulcerative colitis)
Symptoms: amenorrhea, arthralgias, symptoms of chronic hepatitis (eMedicine)


● Viral markers negative, elevated IgG, high ALT and AST (300-500 mg/dl)

Antinuclear antibody: 80% have ANA > 1:80, but ANAs also present in 60% with primary biliary cirrhosis, 50% with alcohol related liver disease, 40% with hepatitis B

Anti-smooth muscle actin antibody: 50% sensitive, not specific, often occurs with antinuclear antibodies
● Directed against F-act, part of hepatocyte cytoskeleton, usually > 1:80 vs. low titers in normals
● Indirect fluorescence shows uniform staining of rat stomach muscularis propria, muscularis mucosa and walls of blood vessels but faint staining of glomerular mesangial zones
● Cable-like staining of Hep 2 cells

Anti-liver-kidney-microsomal antibody: 25% sensitive, usually younger patients
● Antibodies to LKMA and CK 8/18
● Rat distal tubules are negative, but hepatocytes and proximal convoluted tubules are positive
● If positive, anti-mitochondrial antibodies are negative
● Confirm LKMA with Western blot
● LKMA is cytochrome P450 component for LKMA-1; LKMA-2 in patients taking ticrynafen (off market), LKMA-3 in patients with delta hepatitis

Anti-mitochondrial antibody: 15%, may be part of overlap syndrome with primary biliary cirrhosis
Type 1: anti-smooth muscle antibody and antinuclear antibody positive; typically teenager girls or ages 45-70 years
● 10% have other autoimmune disorders such as arthralgias and thyroid disease
Type 2: anti-liver-kidney-microsomal antibody positive
● More common in children
● Adult cases may represent chronic hepatitis C
● Often presents with acute or fulminant hepatitis
● 17% have other autoimmune disorders
Type 3: 25% have only antisoluble liver protein antibody
● Negative for ANA and anti-liver-kidney-microsomal antibody
● 75% have anti-smooth muscle antibody or anti-liver membrane antibody
● Usually women, mean age 37 years
● Usually no systemic autoimmune manifestations


● Steroids (dramatic response); failure to respond suggests diagnosis is wrong
● Liver transplantation (Ann Hepatol 2012;11:222)
● Overall, excellent long term prognosis (Hepat Res Treat 2011;2011:390916)

Gross images

HIV+ woman after HAART treatment

Micro description

● Dense uniform portal infiltrate with abundance of plasma cells, central vein to portal tract bridging necrosis, extensive piecemeal necrosis, central necrosis with plasma cells, variable eosinophils

Micro images

Interface hepatitis

HIV+ woman after HAART treatment

Differential diagnosis

● Hepatitis A, chronic hepatitis C (less severe and more focal interface hepatitis), primary biliary cirrhosis, primary sclerosing cholangitis, overlap syndromes (Ulster Med J 2011;80:15), drug/toxin reaction

Additional references

Best Pract Res Clin Gastroenterol 2011;25:689, Ann Hepatol 2005;4:6, Minerva Gastroenterol Dietol 2007;53:43

End of Liver and intrahepatic bile ducts-nontumor > Hepatitis - noninfectious > Autoimmune hepatitis

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