There is currently no cure for primary biliary cholangitis (PBC), but there are some options available for patients to delay the progression of the disease and avoid complications.
Self-care and lifestyle changes
The main aim of PBC treatment and care is to delay the progression of the disease as much as possible and to reduce its symptoms.
Taking certain self-care steps such as quitting smoking, not regularly drinking and maintaining a healthy weight can all help stop the disease from getting worse.
If you have been diagnosed with PBC, you should also avoid certain medications, including non-steroidal anti-inflammatory drugs like aspirin and ibuprofen.
Keeping a healthy daily routine of light exercise while also pacing yourself can also help reduce symptoms.
Medical treatments
The two primary medical treatments for PBC are ursodeoxycholic acid (UDCA) and obeticholic acid (OCA), which both work by increasing the flow of bile acids.
UDCA, which is a secondary bile acid itself, works by increasing bile acid flow and promoting the secretion of bile acids.
OCA is a newer drug that can help increase bile flow and reduce inflammation. It is usually used to treat people who cannot take UDCA or as an added therapy for those who do not adequately respond to UDCA.
Another option includes fibrates, which may reduce bile acid overload in liver cells. Fibrates may also increase phospholipid excretion into the bile and inhibit the effect of agents causing inflammation in the liver and biliary tree. The biliary tree is the system of vessels that directs secretions from the liver, gallbladder and pancreas. They work by binding to and activating the peroxisome proliferator-activated receptor (PPAR).
There are also newer therapeutic agents that more strongly bind to PPAR ligands, which are currently being investigated in late-stage clinical trials. These may offer a novel therapeutic option for patients with PBC in the future. However, more research is needed before they can reach the clinic.
Treatments for itching
Extreme itching, known as pruritus, can have a considerable negative effect on patients’ quality of life.
Fortunately, there are several treatments available that can help reduce itching associated with PBC. These include antihistamines, cholestyramine, the antibiotic rifampin, opioid antagonists containing naloxone and naltrexone and sertraline.
Regularly moisturizing your skin to ensure it does not become dry may also help reduce itching.
Liver transplant
For some patients with PBC, a liver transplant may be the only treatment option as the liver starts to fail. In this case, your liver will need to be replaced with that of a healthy donor.
Having a liver transplant means that you will need to take immunosuppressants for the rest of your life. This means that your immune system will be compromised and you will be more prone to catching infections. Even with immunosuppressants, there is still a risk that your body will reject the new liver.
Patients who have had a liver transplant may live up to 20 years after the transplant. In around 20% of cases, PBC recurs following a liver transplant.