Autoimmune Liver Disease

Autoimmune Liver Disease

(Autoimmune Hepatitis, Primary Biliary Cirrhosis, Primary Sclerosing Cholangitis)

What is autoimmune liver disease ?

Autoimmune liver disease is a disease in which the body’s own immune system attacks the liver and causes it to become inflamed. The disease is chronic, meaning it lasts many years. If untreated, it can lead to cirrhosis and liver failure.

There are various types of autoimmune liver disease. The most common one is known as Autoimmune Hepatitis. This is the form that mostly affects young women and children. Primary Biliary Cirrhosis is another autoimmune liver disease affecting predominantly women. Primary sclerosing cholangitis affects men and sometimes children.

What causes autoimmune liver disease ?

Your immune system normally attacks bacteria, viruses and other invading organisms. It is not supposed to attack your own cells; if it does, the response is called autoimmunity. In autoimmune hepatitis, your immune system attacks your liver cells, causing long-term inflammation and liver damage. We don’t know why the body attacks itself in this way, although heredity and prior infections may play a role.

What are the symptoms and complications of autoimmune liver disease ?

Often, the symptoms of autoimmune liver disease are minor. When symptoms do occur, the most common are fatigue, abdominal discomfort, joint pain, itching, jaundice (yellowing of the skin and eyes), dark urine, loss of appetite and nausea. Some patients may develop signs of liver failure such as include ascites (fluid in the abdomen) and mental confusion. In 10 to 20% of cases, autoimmune hepatitis may present with symptoms like an acute hepatitis. Itching is an important and sometimes the only symptom of primary biliary cirrhosis. Patients with primary sclerosing cholangitis may present with recurrent episodes of fever and jaundice.

How is autoimmune liver disease diagnosed ?

To confirm a diagnosis of autoimmune liver disease you will need to undergo a panel of special blood tests to detect presence of one or more antibodies in your blood that may be attacking the liver. These are known as auto-antibody tests. Some patients with autoimmune liver disease may not have detectable auto-antibodies in their blood. Often a liver biopsy is required for confirming the diagnosis and assessing the severity of the disease. Please see the section on liver biopsy procedure to understand how a liver biopsy is done.

How is autoimmune liver disease treated ?

The goal of treatment is to stop the body’s attack on itself by suppressing the immune system. Autoimmune hepatitis is typically treated with a medicine called prednisolone, a type of steroid that acts on the immune system. Other drugs such as azathioprine may also be used. Treatment starts with a high dose of prednisolone. When symptoms improve, the dosage is lowered. In most cases, autoimmune hepatitis can be controlled but not cured. Patients will need to stay on the medicine for years, and sometimes for life. The medicines (prednisolone) can cause side effects including diabetes, weakness of bones, high blood pressure, glaucoma, weight gain and decreased resistance to infection. Close medical follow-up is therefore necessary. Primary biliary cirrhosis is a slowly progressive disease and if detected early can be effectively treated with a medicine known as ursodeoxycholic acid. Patients with primary sclerosing cholangitis can develop narrowing (strictures) of their bile ducts that may require endoscopic or surgical treatment.