Although there is no cure for primary biliary cholangitis (PBC), medications can manage symptoms và slow the progression of this liver disease.

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Overview

What is primary biliary cholangitis (PBC)?

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a disease that harms the liver’s ability khổng lồ function. It is chronic, which means it lasts for a long time or regularly comes bachồng.

In people with PBC, the bile ducts become injured, then inflamed, and eventually permanently damaged. The bile ducts are small tubes in the liver that carry bile (a substance needed khổng lồ digest food) from the liver khổng lồ other parts of the digestive system.

When the bile ducts don’t work, bile builds up in the liver. This buildup can cause scarring in the liver (cirrhosis). Cirrhosis makes it hard for the liver to lớn function properly. PBC is progressive sầu, meaning it gets worse over time. If it is not treated, cirrhosis can cause liver failure & even death.

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Who is affected by primary biliary cholangitis (PBC)?

Doctors typically diagnose PBC in people between 35 và 60 years of age. Over 90 percent of patients who develop PBC are women. It affects people of all races. PBC is estimated khổng lồ occur in about 65 of every 100,000 women and in 12 of every 100,000 men.


Symptoms and Causes

What causes primary biliary cholangitis (PBC)?

Doctors do not know for certain what causes PBC. Some experts believe sầu it may be related khổng lồ a problem with the immune system, where the immune system attacks healthy cells in the body toàn thân. In PBC, doctors believe sầu the immune system damages the bile ducts.

While there is not a known cause, some people with PBC also have other related autoimmune diseases. These conditions include autoimmune hepatitis, thyroid diseases, scleroderma, Raynaud’s diseases, Sjogren’s syndrome & celiac disease. Frequent urinary tract infections are also often seen in patients with PBC. In addition, infections, smoking và exposure khổng lồ certain chemicals may play a role in triggering PBC.

PBC also tends to lớn occur more commonly aao ước family members. If one thành viên of a family has PBC, the other family members are at an increased risk.

What are the symptoms of primary biliary cholangitis (PBC)?

Many people with PBC have no symptoms in the early stages. Some people discover they have sầu the condition when a doctor tests them for another issue. As the condition progresses, affected people may first experience symptoms including:

Tiredness (fatigue)Itchy skin (pruritus)Abdominal painDarkening of the skinSmall yellow or White bumps under the skin or around the eyesDry eyes và mouthMuscle và joint pain

In later stages of PBC, additional symptoms include:

Yellowing of the skin & whites of the eyes (jaundice)Internal bleeding in the upper stomach/lower esophagus from enlarged veins (varices)NauseaWeight lossDark-colored urine

Diagnosis và Tests

How bởi vì doctors diagnose primary biliary cholangitis (PBC)?

To diagnose PBC, a doctor will ask about your medical & family history, do a physical exam, order blood tests và other medical tests. Doctors use a blood thử nghiệm to lớn look for a specific substance in the blood called anti-mitochondrial antibody toàn thân (AMA). The presence of this substance almost always confirms PBC. Other blood chạy thử results in patients with PBC may reveal higher than normal levels of liver enzymes & higher levels of cholesterol and triglycerides. Blood work, specific for liver disease, will also be checked.

If you bởi vì have PBC, your doctor may also use an ultrasound kiểm tra, which provides images of the liver & the bile ducts. Ultrasound enables doctors lớn see any other abnormal findings in the liver. Sometimes, a doctor will perform a liver biopsy khổng lồ confirm the diagnosis. In this test, your doctor removes a small piece of the liver using a small needle & looks at it under a microscope.

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Management and Treatment

What are the treatments for primary biliary cholangitis (PBC)?

There is no known cure for PBC. Doctors use medication to manage symptoms và slow the progression of PBC. Ursodiol (Actigall®) helps remove bile from the liver. This treatment improves liver function and prevents progression of the liver disease in many cases. Another drug, obeticholic acid (Ocaliva®) is used in combination with ursodeoxycholic acid (UDCA) or alone if UDCA is not effective sầu or cannot be tolerated. This therapy reduces the amount of bile produced & increases the flow of bile out of the liver.

Other drug options are available depending on each patient’s quality situation and presence of other medical conditions. A liver transplant may be needed if liver function continues khổng lồ worsen despite medical treatment.

To treat the common problem of intense itching, diphenhydramine (Benadryl®; available over-the-counter) or the prescription drugs hydroxyzine (Vistaril®) or cholestyramine (Locholest®, Questran®) may be prescribed.

What are common complications or side effects of primary biliary cholangitis (PBC)?

Some people with PBC develop osteoporosis (thinning of the bones), which can lead lớn bone breaks (bone fractures). This complication typically occurs when a person is in the later stages of the disease. Doctors often treat osteoporosis by recommending regular exercise & prescribing medicines such as alendronate (Fosamax®) or risedronic acid (Actonel®). Both of these treatments help strengthen bones.

Other complications include high cholesterol levels, fat soluble vitamin deficiency, & an increased risk of liver cancer.


Prevention

Can primary biliary cholangitis (PBC) be prevented?

Because doctors bởi not know the cause of PBC, it cannot be prevented. However, you can take steps khổng lồ lessen liver damage, including:

Quit smoking, stop drinking alcohol & stop using illegal drugsTake all medicines as directed by your doctor.Eat a healthy, well balanced diet.Get regular exercise, such as walking.

Healthy food choices could include eating foods rich in vitamins A, D, E and K and/or supplements of these vitamins. Also, foods high in vitamin D and calcium could help prsự kiện osteoporosis. Ask your doctor for specific foods high in these vitamins and minerals.

Foods to lớn avoid include raw shellfish (because of possible bacteria presence); plus food high in salternative text, fat, & carbohydrates – especially added sugars.


Outlook / Prognosis

What is the outlook for people with primary biliary cholangitis (PBC)?

In most cases, PBC progresses slowly. Early diagnosis and treatment are important khổng lồ managing PBC symptoms and avoiding progression of the disease. For many people with the disease, medications và lifestyle changes can control symptoms, so the condition does not interfere with daily life.

If PBC is not treated, and liver function gets worse, it can lead lớn more serious health problems. People with PBC that progresses khổng lồ the late stages may need a liver transplant. The prognosis for these people after a transplant is excellent.


Living With

When should I liên hệ a doctor?

call your doctor if you develop symptoms of PBC. It is important to get an accurate diagnosis of liver problems so that you can treat them as soon as possible.