Autoimmune hepatitis

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Autoimmune hepatitis
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Fatigue (medical), jaundice, abdominal pain, arthralgia
Complications	Cirrhosis, liver failure, hepatocellular carcinoma
Onset	Any age, but most common in young women
Duration	Chronic
Types	N/A
Causes	Autoimmunity
Risks	Genetic predisposition, environmental factors
Diagnosis	Liver function tests, autoantibody tests, liver biopsy
Differential diagnosis	Viral hepatitis, primary biliary cholangitis, primary sclerosing cholangitis
Prevention	N/A
Treatment	Corticosteroids, immunosuppressive drugs
Medication	Prednisone, azathioprine
Prognosis	Variable; good with treatment
Frequency	Rare
Deaths	N/A

Chronic, autoimmune disease of the liver

Autoimmune hepatitis
Synonyms	Autoimmune chronic hepatitis; AIH
Pronounce
Field	Hepatology, Rheumatology, Immunology
Symptoms	Often asymptomatic, fatigue, right upper abdominal pain, anorexia, nausea, jaundice, joint pain, rash
Complications	Chronic liver disease, cirrhosis, hepatocellular carcinoma, liver failure
Onset	Bimodal presentation: 10-20 years of age, 40-50 years of age
Duration	Lifelong
Types	Type 1, Type 2, seronegative
Causes	Genetic predisposition with environmental triggers (such as medications or viral infections)
Risks	Female gender, additional autoimmune diseases
Diagnosis	Liver enzyme levels, autoantibody panels, liver biopsy
Differential diagnosis	Primary biliary cholangitis, Primary sclerosing cholangitis, Viral hepatitis
Prevention
Treatment	Prednisone, Azathioprine, Mycophenolate mofetil, Liver transplant
Medication	Corticosteroids, Immunosuppressants
Prognosis	<50% survival if untreated, >90% survival if treated with long-term therapy
Frequency	Incidence 1-2 per 100,000 per year; Prevalence 10-25 per 100,000
Deaths

Autoimmune hepatitis (AIH) is a chronic autoimmune disorder of the liver, where the body's immune system mistakenly attacks liver cells, causing inflammation and damage. If left untreated, AIH can progress to cirrhosis and liver failure. In some cases, hepatocellular carcinoma may develop due to prolonged liver damage.

Risk Factors

AIH occurs more frequently in women, particularly in those with other autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, and thyroid disorders. The presence of a family history of autoimmune diseases or autoimmune hepatitis can increase the likelihood of developing the condition. Certain environmental factors, including viral infections or the use of certain medications, may trigger the onset of AIH in genetically predisposed individuals.

Cause

The cause of autoimmune hepatitis is not fully understood. It is thought to result from a combination of genetic predisposition and environmental triggers. Genetic mutations may predispose individuals to develop AIH, and environmental factors, such as infections or exposure to drugs, might trigger the immune system to attack liver cells. Research is ongoing into the specific mechanisms by which the immune system targets liver cells in autoimmune hepatitis. Evidence suggests that specific autoantibodies are produced, which target liver-specific proteins, such as liver cytosolic antigen type 1 and smooth muscle actin.

Signs and Symptoms

The signs and symptoms of autoimmune hepatitis can vary significantly. While some individuals may be asymptomatic or only mildly affected, others may experience severe symptoms. Common symptoms include:

• Fatigue (the most common symptom)
• Anorexia
• Nausea
• Right upper abdominal pain
• Jaundice (yellowing of the skin and whites of the eyes)
• Pruritus (itchy skin)
• Arthralgia (joint pain)
• Rash

In severe cases, individuals may experience complications such as encephalopathy, ascites, and splenomegaly. Chronic inflammation of the liver can also lead to cirrhosis, where scar tissue replaces healthy liver tissue. Some individuals may develop additional autoimmune disorders, such as vitiligo, autoimmune thyroiditis, or ulcerative colitis.

Diagnosis

Diagnosing autoimmune hepatitis typically involves a combination of:

• Blood tests to assess liver enzyme levels, including ALT (alanine aminotransferase), AST (aspartate aminotransferase), and bilirubin levels.
• The presence of autoantibodies, including:
  • Antinuclear antibodies (ANA)
  • Smooth muscle antibodies (SMA)
  • Anti-liver cytosolic antigen type 1 antibodies
  • Anti-mitochondrial antibodies (in some cases of overlap with other liver diseases)
• A liver biopsy is often performed to confirm the diagnosis, allowing for the examination of liver tissue and identification of characteristic findings, such as interface hepatitis.

Additionally, viral hepatitis must be ruled out through serological tests for hepatitis B and hepatitis C.

Treatment

Treatment for autoimmune hepatitis aims to suppress the overactive immune response and prevent liver damage. The primary medications used in the management of AIH include:

• Corticosteroids (such as prednisone) ‚Äì used to reduce inflammation in the liver.
• Azathioprine ‚Äì an immunosuppressant often used in combination with corticosteroids to maintain remission and reduce steroid-related side effects.
• Mycophenolate mofetil ‚Äì an alternative immunosuppressant used in cases where azathioprine is ineffective or not tolerated.

In some cases, liver transplant may be necessary for individuals who progress to cirrhosis or liver failure. Patients with AIH may require lifelong treatment to maintain remission and prevent relapse, with periodic monitoring of liver function and autoimmune markers.

Prognosis

The prognosis of autoimmune hepatitis varies depending on the severity of the disease and the effectiveness of treatment. If left untreated, AIH can lead to progressive cirrhosis and liver failure, with a significantly reduced life expectancy. However, with early diagnosis and appropriate treatment, the prognosis improves dramatically, with more than 90% survival in treated patients.

Complications

Autoimmune hepatitis can lead to several serious complications if not adequately managed:

• Cirrhosis ‚Äì scar tissue replaces healthy liver tissue, leading to impaired liver function.
• Hepatocellular carcinoma ‚Äì liver cancer that can develop as a result of chronic inflammation.
• Liver failure ‚Äì a life-threatening condition where the liver no longer functions adequately.
• Side effects from long-term immunosuppressive therapy, including increased risk of infections and osteoporosis.

Prognosis

If treated early with corticosteroids and azathioprine, the outlook is significantly better. Around 80-90% of patients go into remission within the first 1-2 years of treatment. However, long-term management is usually required to prevent relapse, and about 10-20% of patients eventually require a liver transplant due to cirrhosis or liver failure.

Diet and Lifestyle

While diet and lifestyle factors are not known to directly cause or prevent autoimmune hepatitis, it is important for individuals with cirrhosis to follow a healthy diet to support overall liver health. Avoiding alcohol and managing medications carefully is crucial for preventing further liver damage.

See Also

• Cirrhosis
• Liver transplant
• Hepatitis
• Autoimmune disease
• Liver disease
• Primary biliary cholangitis

External Links

• Autoimmune Hepatitis Guidelines - VA
• American Liver Foundation - Autoimmune Hepatitis
• Research Article on Autoimmune Hepatitis

NIH genetic and rare disease info

• NIH info on Autoimmune hepatitis

Autoimmune hepatitis is a rare disease.

This article is a medical stub. You can help WikiMD by expanding it!
Most recent articles Ongoing trials	Wikipedia