Biliary Cirrhosis, Primary

Biliary Cirrhosis, Primary
Biliary Cirrhosis, Primary

Overview Of Biliary Cirrhosis, Primary

Primary biliary cirrhosis is now known as primary biliary cholangitis. The biliary tract is comprised of several bile ducts that serve to move bile, a bitter substance that aids in digestion, from the liver to the small intestines. If the ducts become swollen or inflamed, then the flow of bile is restricted. Due to the chronic inflammation, scarring of the ducts and the liver can occur. This scarring is known as cirrhosis, and if not managed, then it can lead to liver failure, an inevitably fatal condition without a transplant. The disease most often affects middle-aged women.

Causes Of Biliary Cirrhosis, Primary

What triggers biliary cholangitis is as yet unknown, but the condition itself is an autoimmune disorder. Therefore, your body’s own immune system attacks healthy, normal tissues. This disease may be linked to other autoimmune disorders such as:

Symptoms

 Symptoms often begin slowly, and the condition maybe quite advanced by the time of diagnosis.

Early symptoms may include:

  • Nausea and belly pain
  • Fatigue and loss of energy
  • Itching (pruritus) unrelieved by scratching
  • Fatty deposits under the skin
  • Fatty stools
  • Poor appetite and weight loss

As liver function worsens, the following symptoms may occur:

  • Fluid buildup in the legs (edema) and abdomen (ascites)
  • Confusion or brain fog
  • Redness on the palms of the hands
  • Yellowish tinge to the skin, mucous membranes, or eyes (jaundice)
  • In men, impotence, shrinking of the testicles, and breast swelling may be present
  • Easy bruising and abnormal bleeding, most often because of swollen veins in the digestive tract
  • Pale or clay-colored stools

Exams & Tests

Your doctor will take a medical history, and do a physical exam. They will order blood panels to check the following to see if the liver is functioning properly.

  • Cholesterol and lipoprotein blood tests
  • Prothrombin time (PT)
  • Albumin blood test
  • Immunoglobulin M level, and anti-mitochondrial antibody tests (results for the latter are positive in about 95% of cases)
  • Liver function tests (serum alkaline phosphatase is most important)

Depending on the blood test results, your doctor may order one or several of the below tests that can help measure how severe the liver disease is. 

  • FibroScan, a specialised ultrasound machine particularly for measuring fibrosis (liver scarring) and steatosis (fatty deposits). Other names include transient elastography or liver elastography. While not at all painful or invasive, unlike a regular sonogram, FibroScan will produce a thumping sensation against the skin. 
  • MRE, or magnetic resonance elastography, a test combining ultrasound and MRI.
  • Magnetic resonance cholangiopancreatography (MRCP), a specialised MRI that specifically targets the pancreatic and biliary systems.
  • Liver biopsy, either as a direct puncture to the abdomen, or trans-jugular. Trans-jugular liver biopsies involve threading a catheter down through the right jugular vein into the main hepatic vein to obtain the biopsy sample. The procedure is longer, but a safer choice for patients with clotting issues. 

Treatment Of Biliary Cirrhosis, Primary

As of yet, there is no cure for biliary cholangitis. The goal of treatment is to manage symptoms and monitor for liver failure. If the condition worsens, a liver transplant may be necessary.

Cholestyramine (or colestipol) and nalfurafine hydrochloride may help the itching. Likewise, for some, simple allergy medicines such as Zyrtec can soothe the itching. This is because hepatic pruritus is sometimes, though not always, the result of unprocessed toxins, and bile acids in the blood seeping through skin and triggering a histamine response. Yes, you are quite literally allergic to yourself. In most cases, the cause of hepatic pruritis is unknown, and difficult to fully treat.

Ursodeoxycholic acid can improve the removal of bile from the bloodstream. In addition, obeticholic acid (Ocaliva) can improve liver enzyme counts, and overall liver health in a number of liver conditions such as primary biliary cirrhosis and non-alcoholic steatohepatitis (NASH). Ursodiol (Actigall) can work in conjunction with Ocaliva. Vitamin replacement therapy restores vitamins A, K, E, and D, which are lost in fatty stools. Likewise, a calcium supplement or other bone medicines can prevent or treat weak or soft bones.