Conditions · Gastroenterology and hepatology

Autoimmune hepatitis !

A long-term liver inflammation in which the immune system attacks liver cells. It responds to immunosuppressive treatment but can lead to cirrhosis.

Updated 2026-11-01

Structures involved

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Why it happens

What it is

A chronic, immune-mediated inflammatory disease of the liver characterised by raised aminotransferases, autoantibodies, high immunoglobulin G and interface hepatitis on biopsy, which usually responds to immunosuppression.

Who gets it

Affects mainly women (about three out of four patients), at any age, with peaks in adolescence and middle age. It occurs worldwide; in Latin America, including Colombia, it is a recognised cause of chronic hepatitis and of acute hepatitis in young women once viral causes are excluded.

Causes

Loss of immune tolerance to liver antigens in a genetically susceptible person (HLA-DR3 and DR4)
Possible triggers: viral infections, drugs (minocycline, nitrofurantoin, statins and others) and herbal products
Frequently associated with other autoimmune diseases

Risk factors

Female sex
Other autoimmune disease: autoimmune thyroiditis, type 1 diabetes, coeliac disease, rheumatoid arthritis, ulcerative colitis
Family history of autoimmune disease
Exposure to drugs known to trigger autoimmune-like hepatitis

Pathophysiology

T lymphocytes recognise liver antigens and, with insufficient regulatory T-cell control, attack hepatocytes. Plasma cells produce autoantibodies and excess immunoglobulin G. Inflammation starts at the boundary between portal tracts and the lobule (interface hepatitis), and persistent necroinflammation drives fibrosis that can progress to cirrhosis.

Classification

Type 1: antinuclear (ANA) and/or anti-smooth muscle (ASMA, anti-actin) antibodies; most common, mainly adults
Type 2: anti-liver-kidney microsomal type 1 (anti-LKM1) or anti-liver cytosol type 1 antibodies; mainly children, often more severe
Overlap syndromes with primary biliary cholangitis or primary sclerosing cholangitis

Symptoms and signs

Fatigue (most common), malaise and loss of appetite
Right upper quadrant discomfort
Jaundice, dark urine and pale stools
Joint pains and amenorrhoea
About a third have no symptoms and are found by abnormal liver tests; some present with cirrhosis or acute liver failure

Diagnosis

Clinical: Consider it in any unexplained acute or chronic hepatitis, especially in a woman with other autoimmune disease, after excluding viral, drug, alcohol, metabolic (Wilson disease) and fatty liver causes.
Tests: Raised ALT and AST, raised immunoglobulin G, autoantibodies (ANA, ASMA, anti-LKM1, anti-SLA). Liver biopsy is needed: interface hepatitis with lymphoplasmacytic infiltrate and rosettes. Simplified scoring systems combine autoantibodies, IgG, histology and absence of viral hepatitis.
Imaging: Ultrasound excludes biliary obstruction and assesses signs of cirrhosis; elastography estimates fibrosis; cholangiography if overlap with sclerosing cholangitis is suspected.

Differential diagnosis

Viral hepatitis (A, B, C, E) and other viral infections
Drug-induced liver injury
Wilson disease (always in young patients)
Metabolic dysfunction-associated steatotic liver disease and alcohol-related liver disease
Primary biliary cholangitis and primary sclerosing cholangitis

Goal of treatment

Achieve complete biochemical remission (normal aminotransferases and IgG), prevent progression to cirrhosis and liver failure, and limit the adverse effects of long-term immunosuppression.

Treatment

Medical: Induction with corticosteroids (prednisone or prednisolone; budesonide in non-cirrhotic patients) usually combined with azathioprine as a steroid-sparing agent.
Medical: Maintenance with azathioprine, with slow steroid tapering; mycophenolate mofetil, calcineurin inhibitors or other agents for intolerance or non-response.
Follow-up: Regular liver tests and IgG; blood counts and TPMT assessment with azathioprine; bone protection, glucose and blood pressure checks with corticosteroids; liver cancer and varices surveillance if cirrhosis.
Surgical: Liver transplantation for acute liver failure or decompensated cirrhosis not responding to treatment.

Complications

Cirrhosis and portal hypertension
Acute liver failure
Hepatocellular carcinoma in patients with cirrhosis
Side effects of immunosuppression: infections, osteoporosis, diabetes, cytopenias

Prognosis

Untreated severe disease has high mortality, but most patients reach remission with immunosuppression and have a near-normal life expectancy. Relapse after stopping treatment is common, so many need lifelong maintenance; outcome is worse when cirrhosis is present at diagnosis.

Test yourself

Think of the answer, then tap the question to see it.

Which autoantibodies define type 1 autoimmune hepatitis?

Antinuclear antibodies (ANA) and anti-smooth muscle antibodies (ASMA).

Which autoantibody defines type 2 and in whom is it typical?

Anti-LKM1, typical in children and adolescents.

What is the characteristic biopsy finding?

Interface hepatitis with a lymphoplasmacytic infiltrate.

What is the first-line treatment?

Corticosteroids, usually combined with azathioprine for maintenance.

Which serum protein is typically raised?

Immunoglobulin G (polyclonal hypergammaglobulinaemia).

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Educational information, not medical advice. For any symptom, see a health professional.

Sources: Autoimmune hepatitis: MedlinePlus Medical Encyclopedia (MedlinePlus (U.S. National Library of Medicine), 2024); Autoimmune Hepatitis - NIDDK (National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), 2024).

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Representative model, for educational purposes only.

Liver highlighted on the Durgical 3D model