Autoimmune liver diseases are chronic, inflammatory diseases that usually progress slowly. The three main categories of autoimmune liver disease are autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC).

Autoimmune Hepatitis (AIH)

Autoimmune hepatitis is a liver disease with no known cause but is considered an “autoimmune” disorder because the immune system attacks the body’s own hepatocytes (liver cells).

  • Autoimmune Hepatitis is more common in women than in men.
  • It is often associated with other autoimmune disorders, such as Sjogren’s syndrome (dry eyes, dry mouth), Raynaud’s phenomenon (impaired circulation in hands and feet in response to cold), hyperthyroidism (Graves’ disease or Hashimoto’s thyroiditis) and rheumatoid arthritis.
  • Most people with AIH do not experience symptoms until their disease is advanced, but early symptoms may include fatigue. AIH sometimes begins with a “flare” with jaundice, fatigue, itching, fever, and right upper quadrant discomfort.
  • Untreated AIH can lead to progressive scarring of the liver (including cirrhosis). When diagnosed and treated early, AIH often can be controlled with drugs that suppress the immune system, including steroids and azathioprine.
  • A liver transplant may be an option if autoimmune hepatitis progresses to advanced liver disease.
  • Diagnosis of AIH may be made by blood tests (Anti-nuclear Antibody, Smooth Muscle Antibody), but often a liver biopsy is required.

Primary Biliary Cholangitis (PBC)

Primary biliary cholangitis (PBC) is also a liver disease with no known cause but is believed to be related to other autoimmune disorders. Primary biliary cholangitis begins with inflammation of the small bile ducts. The inflammation blocks the flow of bile out of the liver and liver cells, causing inflammation. As inflammation spreads, scar tissue (fibrosis) develops throughout the liver, replacing more and more liver tissue with fibrosis (scarring), leading to cirrhosis in some cases.

  • PBC occurs most commonly in middle-aged women.
  • People with PBC may experience fatigue and itching, and sometimes dry mouth.
  • PBC progresses slowly over many years as the bile ducts within the liver become more inflamed and scarred, eventually resulting in cirrhosis if untreated.
  • Diagnosis is usually made by blood tests (alkaline phosphatase, anti-mitochondrial antibody), and a liver biopsy is not always necessary.
  • Treatment for PBC with oral medications like Ursodeoxycholic acid (UDCA) and in many cases Obeticholic Acid (OCA) will often slow the progression and delay or prevent the development of cirrhosis.
  • Formerly referred to as Primary Biliary Cirrhosis, the name was recently changed because if carefully managed, most patients will never progress to cirrhosis or liver transplant.

Primary Sclerosing Cholangitis (PSC)

Primary Sclerosing Cholangitis (PSC) is a disease of the large bile ducts in which they become inflamed and heavily scarred. Its cause is unknown and at this time there is no treatment known to stop the disease from progressing.

  • PSC occurs mostly in men, half of whom have a history of either ulcerative colitis or Crohn’s disease.
  • PSC progresses slowly over many years, and as the bile ducts become more scarred, the liver becomes damaged, leading to cirrhosis in many cases.
  • Symptoms of PSC include itching, fatigue, abdominal pain and jaundice.
  • Cancer of the bile ducts (cholangiocarcinoma) is a complication which occurs in about 10% of people with PSC.
  • PSC is often diagnosed by MRI (MRCP) or endoscopy of the bile ducts (ERCP), and sometimes with a liver biopsy and blood tests.
  • Treatment is currently focused on managing symptoms, however clinical trials are underway for new medical therapies.
  • In advanced cases of PSC, liver transplantation may be a treatment option.

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