Primary biliary cholangitis: Get informed

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 North America, 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 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

Ursodiol  (Ursodeoxycholic Acid in UK) 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 – 15 mg 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, Ursodiol generally 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 Ursodiol is effective. A secondary treatment may be prescribed in addition to Ursodiol.

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 USA, you may be referred to a gastroenterologist (digestive disorders specialist) or a hepatologist (liver diseases specialist). Referral to other specialists for certain symptoms or complications may be necessary.

PBC and Pregnancy

It is believed that Ursodiol presents no risks to the fetus during conception, gestation or birth. However, you should inform your doctor if you are pregnant or plan to become pregnant during treament. 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 the American Liver Foundation in the USA can be a good way to meet other people affected by PBC.

If you are living with PBC or have a loved one who is, don’t hesitate to share and discuss on the Carenity Forum! You’ll be sure to find useful information and support among other members.

Information from this article comes from the American Liver Foundation guide on PBC, and from the PBCers Organization

Published Oct 3, 2019