Primary biliary cholangitis: Get informed

Primary biliary cholangitis (PBC) is a chronic liver disease that mainly affects women over 40.

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Primary Biliary Cholangitis (PBC)

What is Primary Biliary Cholangitis?

Primary biliary cholangitis (PBC) is a chronic liver disease that chiefly affects women over 40. The most serious complication of this disease is biliary cirrhosis which in the terminal phase causes scarring lesions on the liver. PBC is not curable, but proper treatment can limit symptoms and prevent complications. In Europe, 2 out of every 100.000 people are likely to develop PBC in their lifetimes and it is estimated that between 1.9 and 40.2 people out of 100.000 already have PBC.

Diagnosis

PBC is usually diagnosed when a physician discovers reduction or obstruction (blockage) of bile secretions (cholestasis), or that the patient’s blood contains a specific type of antibody (antimitochondrial antibodies or AMA), or inflammation of biliary ducts in the liver. Beyond a clinical examination and a survey of previous medical conditions and medications, several other examinations may be necessary in order to properly diagnose PBC: an echography, lab work (alkaline phosphatase and gamma-glutamyl transferase levels (Gamma-GT)), ultrasounds, a liver biopsy or potentially genetic tests. Urinary tract infections and alcohol and tobacco use are risk factors you should be aware of.

Symptoms

Itchy skin rash (Pruritus), fatigue, Gougerot-Sjögren Syndrome (dry mouth and/or eyes) and abdominal pain are the principal symptoms of PBC. Some sufferers may additionally experience Restless Leg Syndrome and mental fog which may be mistaken for symptoms of depression.

Medication and other remedies exist to combat these symptoms, such as lotions and cold baths to deal with skin rashes. An allergen test will also help to verify if the skin rashes are linked to allergies rather than PBC. Talk to your physician about your symptoms to ensure a correct diagnosis.

Treatments

Ursodeoxycholic acid (UDCA) is the primary treatment prescribed for sufferers of PBC. It is a natural biliary acid, administered in pill form. Multiple dosages exist. The dose must be adjusted to the patient’s weight (13 – 15mg per kilo of body weight per day). Your physician should monitor your weight and adjust your treatment in accordance with any fluctuations. If the patient responds well to treatment, UDCA must continue to be taken for life, even if symptoms improve or disappear.

After treatment of six months to a year, a check-up should be conducted to determine if UDCA is effective. A secondary treatment may be prescribed in addition to UDCA

The age of the patient as well as the evolution of the condition will determine treatment. It is very important to get regular check-ups to adapt the treatment as necessary.

In the UK, you may be referred to a gastroenterologist (digestive disorders specialist) or a hepatologist (liver diseases specialist), in an office or practising at hospital. Referral to other specialists for certain symptoms or complications may be necessary.

PBC and Pregnancy

It is believed that UDCA presents no risks to the fetus during conception, gestation or birth. Still, you should be monitored closely by your physician if you are trying to conceive while being treated for PBC. Skin rashes may erupt and/or spread during pregnancy and certain complications such as cholestasis may require additional treatments to avoid deficiencies or risks to the baby. Portal hypertension should be closely watched for as it may have an impact on the blood vessels of the oesophagus and stomach.

Talking About Your Condition

Dealing with an incurable condition on an everyday basis can get complicated. Contact patient associations such as PBC Foundation in the UK can be a good way to meet other people affected by PBC. And if you are living with PBC or have a loved one who is, don’t hesitate to share and exchange on the Carenity Forum! You’ll be sure to find useful information and support among the members there. 

Information for this article comes from the NHS guide on PBC, and from the PBC Foundation.

Published 29 Oct 2018

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