What is portal hypertension in PBC?

As primary biliary cholangitis (PBC) progresses, your risk of developing portal hypertension — elevated pressure in the portal vein — increases as a result of liver scarring and damage. 

Although portal hypertension is a serious complication that must be monitored carefully, there are options available to help you manage the condition. 

What is portal hypertension?

The portal vein is a large blood vessel that sends nutrient-rich blood from the intestines, stomach, pancreas, spleen and gallbladder to the liver. There, the liver processes the blood, filtering out toxins and converting the nutrients into usable forms.

In patients with portal hypertension, blood pressure becomes too high in the portal vein. This high pressure can cause swollen, fragile veins known as varices to form throughout the gastrointestinal tract. These varices have a high risk of rupturing, which can lead to life-threatening internal bleeding if not treated right away. 

How PBC can lead to portal hypertension

PBC is characterized by progressive damage to the bile ducts of the liver, which leads to scarring over time. This can ultimately result in cirrhosis, or severe scarring of the liver. The scarring blocks blood from flowing properly through the portal vein, causing a backup that increases blood pressure.

Read more about PBC prognosis

Although portal hypertension is commonly associated with late-stage PBC, it can begin even during the early stages of PBC, before cirrhosis occurs. Some studies have shown that portal hypertension indicates poorer prognosis, regardless of the disease stage at which it’s detected. Therefore, early vigilance is critical to long-term health with PBC.

Symptoms of portal hypertension

Some symptoms of portal hypertension that you should be aware of include:

• Gastrointestinal bleeding: If you develop gastrointestinal bleeding, your stools may appear black or bloody, or you can begin vomiting blood due to a rupture. This is a medical emergency that requires immediate attention.
• Swelling: Portal hypertension can cause swelling in many areas of the body, including the abdomen, legs and feet.
• Behavioral changes: Some patients with portal hypertension may experience confusion, brain fog, changes in sleep or slurred speech.

Diagnosis of portal hypertension

In some cases, a diagnosis of portal hypertension can be made based on symptoms alone, especially in patients with advanced liver disease. However, your doctor may wish to perform diagnostic tests such as:

• Imaging studies including ultrasound, computed tomography (CT) or magnetic resonance imaging (MRI).
• Endoscopy, which uses a thin tube with a camera to detect varices in the gastrointestinal tract.
• Elastography, a noninvasive test that measures liver stiffness.

Your health care team may also administer a blood test to monitor overall liver function and check for markers of gastrointestinal bleeding.

Treating portal hypertension

When portal hypertension is caused by liver scarring, as is the case in PBC, it typically cannot be cured. However, there’s several treatment options to help manage symptoms and prevent severe complications.

Medication

Non-selective beta-blockers can help lower pressure within the portal vein, reducing the risk of variceal bleeding. Vasoconstrictors can also be used to lower blood pressure and flow, though they’re more often used to stop active bleeding.

Managing bleeding events

If you’re experiencing a gastrointestinal hemorrhage, your doctor may perform an endoscopy to correct the bleeding. The most common technique involves “banding” the varices with tiny elastic rings to stop blood flow to the area. You’ll likely need to undergo multiple sessions to completely destroy the varix. Another, more complicated, method involves inflating a small balloon in the stomach or esophagus to put pressure on the veins and stop the bleeding.

In some cases, you may need a shunt placed to maintain normal blood flow.

Liver transplantation

For patients with end-stage PBC, liver transplantation effectively removes the root cause of portal hypertension by replacing the diseased liver with a healthy one.

Learn more: “Liver transplants due to PBC: What you need to know”