Cholengitis

Your doctor will ask you about your health history and your family's health history, and perform a physical exam.

The following tests and procedures may be used to diagnose primary biliary cholangitis.

Blood tests:

Liver tests. These blood tests check the levels of enzymes that may signal liver disease and bile duct injury.

Antibody tests for signs of autoimmune disease. Blood tests may be done to check for anti-mitochondrial antibodies (AMAs). These substances almost never occur in people without the disease, even if they have other liver disorders. Therefore, a positive AMA test is considered a very reliable sign of the disease. However, a small number of people with primary biliary cirrhosis don't have AMAs.

Cholesterol test. More than half the people with primary biliary cholangitis have extreme increases in blood fats (lipids), including total cholesterol level.

Imaging tests may not be needed. However, they may help your doctor confirm a diagnosis or rule out other conditions with similar signs and symptoms. Imaging tests looking at the liver and bile ducts may include:

Ultrasound. Ultrasound uses high-frequency sound waves to produce images of structures inside your body.

FibroScan. Using an ultrasound-like probe, this test can detect scarring of the liver.

Magnetic resonance cholangiopancreatography (MRCP). This special magnetic resonance imaging (MRI) exam creates detailed images of your organs and bile ducts.

Magnetic resonance elastography (MRE). MRI is combined with sound waves to create a visual map (elastogram) of internal organs. The test is used to detect hardening of your liver (fibrosis) that might be a sign of cirrhosis.

There's no cure for primary biliary cholangitis, but medications are available to help slow the progression of the disease and prevent complications. Options include:

Ursodeoxycholic acid (UDCA). This medication, also known as ursodiol (Actigall, Urso), is commonly used first. It helps move bile through your liver. UDCA doesn't cure primary biliary cholangitis, but it seems to improve liver function and reduce liver scarring. It's less likely to help with itching and fatigue. Side effects may include weight gain, hair loss and diarrhea.

Obeticholic acid (Ocaliva). In 2016, the U.S. Food and Drug Administration approved this medication for primary biliary cholangitis. Studies show that when given alone or combined with ursodiol for 12 months it can help improve liver function and slow liver fibrosis. However, its use is often limited because it can cause increased itching.

Fibrates (Tricor). Researchers aren't exactly sure how this medicine works to help ease primary biliary cholangitis symptoms. But, when taken with UDCA, it has reduced liver inflammation and itching in some people. More studies are needed to determine long-term benefits.

Budesonide. When combined with UDCA, the corticosteroid budesonide may be of potential benefit for primary biliary cholangitis. However, this drug is associated with steroid-related side effects for people with more advanced disease. More long-term trials are necessary before budesonide can be recommended for treating this condition.

Liver transplant. When medications no longer control primary biliary cholangitis and the liver begins to fail, a liver transplant may help prolong life. A liver transplant replaces your diseased liver with a healthy one from a donor. Liver transplantation is associated with very good long-term outcomes for people with primary biliary cholangitis. However, sometimes the disease comes back several years later in the transplanted liver.