Primary biliary cholangitis

Other Names: PBC; Familial primary biliary cirrhosis; Primary Biliary Cirrhosis

Primary biliary cholangitis (PBC) is a chronic, progressive liver disease in which the bile ducts become inflamed and damaged.This leads to the buildup of bile and causes liver problems such as scarring, cirrhosis (scarring and poor liver function), and eventual liver failure. PBC is more common in women.

Cause

PBC is considered an autoimmune disease in which the immune system malfunctions and mistakenly attacks a person’s healthy bile duct cells, causing the inflammation and damage. It is thought to be caused by a combination of genetic susceptibility and environmental triggers (multifactorial inheritance).

Risk factors

You are more likely to get primary biliary cholangitis if you

• are a woman
• are older than age 35
• have a parent or sibling—particularly an identical twin—with the disease
• are of Northern European descent

Possible environmental triggers include:

• infections
• cigarette smoking

Symptoms

Many people do not have symptoms when they are first diagnosed and may not develop symptoms for several years. Early symptoms may include fatigue (the most common symptom), itchy skin (pruritus), and abdominal pain. As the disease progresses, people with PBC may develop weakness, nausea, diarrhea, swelling in the legs and feet (edema), bone and joint pain, jaundice, dark urine, and xanthomas. The symptoms of PBC can significantly impair quality of life. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

80%-99% of people have these symptoms

• Biliary cirrhosis
• Cirrhosis(Scar tissue replaces healthy tissue in the liver)
• Conjugated hyperbilirubinemia
• Dermatographic urticaria
• Hyperpigmentation of the skin(Patchy darkened skin)

30%-79% of people have these symptoms

• Abnormal circulating lipid concentration
• Abnormality of the intrahepatic bile duct
• Abnormality of the thyroid gland(Thyroid abnormality)
• Antinuclear antibody positivity
• Elevated alkaline phosphatase(Greatly elevated alkaline phosphatase)
• Hepatic failure(Liver failure)
• Hepatic fibrosis
• Hepatocellular carcinoma
• Increased circulating IgM level
• Jaundice(Yellow skin)
• Onychomycosis
• Orthostatic hypotension(Decrease in blood pressure upon standing up)
• Portal hypertension
• Pruritus(Itching)

5%-29% of people have these symptoms

• Abdominal distention(Abdominal bloating)
• Ascites(Accumulation of fluid in the abdomen)
• Celiac disease
• Excessive daytime somnolence(More than typical sleepiness during day)
• Fatigue(Tired)
• Hepatitis(Liver inflammation)
• Hypoalbuminemia(Low blood albumin)
• Increased circulating IgA level
• Osteoporosis

1%-4% of people have these symptoms

• Gastrointestinal inflammation

Diagnosis

Blood tests

Your doctor may recommend the following blood tests: Anti-mitochondrial antibodies (AMA). Anti-mitochondrial antibodies NIH external link are found in the blood of about 95 percent of people with primary biliary cholangitis.3

Liver tests

Liver tests NIH external link can show abnormal liver enzyme levels, which may be a sign of damage in your liver or biliary tract. Higher-than-normal levels of the liver enzyme alkaline phosphatase occur in people with diseases that destroy or block the bile ducts, such as primary biliary cholangitis.

Your doctor may diagnose primary biliary cholangitis if you have anti-mitochondrial antibodies and higher-than-normal levels of alkaline phosphatase in your blood, even if you have no other signs or symptoms of the disease.

Cholesterol

People with primary biliary cholangitis may have higher-than-normal cholesterol levels, which may be a sign that their liver is not working properly.

Imaging tests

Your doctor may use imaging tests such as x-rays and ultrasound to help diagnose primary biliary cholangitis by ruling out other causes of bile duct damage, such as gallstones, bile duct strictures, and tumors.

Liver biopsy

Your doctor may perform a liver biopsy to

• rule out other diseases that may be causing your symptoms
• confirm the diagnosis of primary biliary cholangitis
• determine whether the disease is advanced—as shown by the amount of liver scarring or cirrhosis—or very active

Treatment

Doctors treat primary biliary cholangitis with medicines. Your doctor may prescribe ursodiol (Actigall, Urso). Although ursodiol does not cure primary biliary cholangitis, it can slow the progression of liver damage. People who respond to ursodiol early in the course of primary biliary cholangitis can live longer without needing a liver transplant. If you do not respond to ursodiol, your doctor may prescribe obeticholic acid (Ocaliva). However obeticholic acid does not improve symptoms, and further research is needed to show whether it slows liver disease progression.

Your doctor may recommend over-the-counter medicines or prescribe medicines to treat symptoms of primary biliary cholangitis.

For mild itchy skin, your doctor may prescribe hydroxyzine (Vistaril). For more severe itchy skin, your doctor may prescribe cholestyramine (Locholest, Questran).

Doctors treat the complications of primary biliary cholangitis with medicines and dietary supplements . Your doctor may recommend changes in your diet and lifestyle.

High blood cholesterol levels

If you have higher-than-normal blood cholesterol levels, your doctor may prescribe medicines called statins  and recommend lifestyle changes.

Osteoporosis For osteoporosis k, your doctor may prescribe medicines that slow or stop bone loss and improve bone density. Your doctor may recommend dietary supplements of calcium and vitamin D.

Fat-soluble vitamin deficiencies For fat-soluble vitamin deficiencies, your doctor may recommend dietary supplements of vitamins A, D, E, and K. Follow your doctor’s instructions on the type and amount of vitamins you should take.

Cirrhosis

If primary biliary cholangitis leads to cirrhosis, doctors can treat the health problems related to cirrhosis with medicines, surgery, and other medical procedures. If cirrhosis leads to liver failure, you may need a liver transplant.

Your doctor will consider a liver transplant when your primary biliary cholangitis leads to liver failure.

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

• Ursodiol (Brand name: Actigall)Treatment of patients withprimary biliary cholangitis (formerly known as primary biliary cirrhosis).
• obeticholic acid (Brand name: Ocaliva)Treatment of primary biliary cholangitis (PBC) in combination with ursodeoxycholic acid (UDCA) in adults with an inadequate response to UDCA, or as monotherapy in adults unable to tolerate UDCA.
• Ursodiol (Brand name: Urso 250)Treatment of patients with primary biliary cirrhosis

NIH genetic and rare disease info

• NIH info on Primary biliary cholangitis

Primary biliary cholangitis is a rare disease.

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