PBC (primary biliary cirrhosis) is a chronic ailment which mostly affects adults, especially women. Actually, it is 10 times more common in women than men. The PBC is characterized by destruction of small bile ducts located within the liver due to continuous inflammation. Nowadays, people diagnosed with PBC are increasing rapidly and at present 2.8 to 37 per million men and 25 to 335 per million women suffer from this disease.

What Is PBC?

PBC or primary biliary cirrhosis is a long-term illness which affects the liver and destroys the small bile ducts present within the liver at a slow rate. The liver produces the digestive liquid called bile which passes to the small intestine through the bile ducts and is required for digesting fatty vitamins and fats.

Inflammation is the reason for the destruction of bile ducts in PBC patients. As a result, the bile does not leave the liver. The inflammation keeps on damaging the liver cells, causing scarring of the liver which is also known as cirrhosis. Cirrhosis stops the return of the blood from the intestines to the heart. The progression of cirrhosis increases the scar tissue in the liver which hampers its ability to function properly.

Advancement of PBC is often gradual and slow. Even after the diagnosis, most of the patients can lead a productive and active life for another 10 to 15 years. Patients with normal liver tests during treatment can expect to live a normal life while those who don’t show any symptoms when being diagnosed seldom show those symptoms for a long time. PBC is a long-term ailment which can develop into a serious complication like cirrhosis which is often life-threatening.

What Are the PBC Symptoms?

The following are some of the early PBC symptoms that are commonly experienced by the patients.

• ŸFatigue (Tiredness). It is one of the first PBC symptoms. This tiredness is usually very debilitating in nature, but it is unclear why PBC causes fatigue. Since tiredness can be caused due to a lot of causes, therefore, it is not a specific symptom of PBC.
• ŸPruritus (itch). It is also one of the common symptoms that occur often. This liver disease itching can sometimes become very distressing if it spreads all over the skin. It is unclear as to why the itch occurs, but it is possible that it might be because of the buildup of a chemical from bile in the bloodstream.
• ŸPain felt on the upper right side of the abdomen (tummy) right over the liver. 1 in 5 patients of PBC experience this symptom.
• ŸDevelopment of pink or red blotches on the hands is another of the PBC symptoms.
• ŸSome patients develop feelings of nausea, diarrhea and bloating. They might have bulky and pale stools (feces) which are difficult to flush. Such stools are called fatty stools or steatorrhoea because your body is inability to digest fat if you are suffering from PBC.
• ŸDevelopment of distaste for alcohol in some of the patients and temporary quitting of smoking by patients who smoke.
• ŸSome PBC patients even suffer from other autoimmune diseases which have a different set of symptoms like dry eyes or dry mouth.  

During examination, the doctor might feel your enlarged liver. The development of jaundice might follow with the progression of the disease whose signs include yellowing of the skin and the whites of the eyes. The reason for this is a buildup of bilirubin, a chemical in bile, in the bloodstream. Other symptoms that are related to the liver might develop if cirrhosis or scarring of the liver occurs.

What Are the Complications of PBC?

If PBC is left untreated, it may have some serious complications that will become a real concern. These complications include:

• Cirrhosis.Scarring of the liver is known as cirrhosis which impedes the functioning of the liver. It usually occurs after primary biliary cirrhosis. Liver failure becomes a possibility if cirrhosis is not treated. In this case, the liver becomes unable to function normally.
• Portal hypertension (increased pressure in the portal vein). The portal vein is a large blood vessel which transports blood from the pancreas, intestine and spleen to the liver. Development of scar tissue interrupts the circulation of blood in and out of the liver, which causes blood get backed up and results in increased pressure in the vein. Moreover, due to the abnormal flow of blood through the liver, the filtering of the toxins and drugs from the bloodstream is affected too.
• Enlarged spleen (splenomegaly).Because of the portal hypertension, the enlargement of the spleen can occur due to the build-up of platelets and white blood cells.
• Gallstones and bile duct stones.The bile that is unable to flow out of the bile ducts turns into stones which cause both infection and pain.
• Enlarged veins (varices).Because of the blockage of blood circulation through the portal vein, backing up of the blood might take place in the veins that are present withinyour esophagus and stomach. Because these blood vessels have a very thin wall, the increased pressure in them can result in bleeding in your esophagus and upper stomach. You would need immediate medical care for this bleeding as it can be life-threatening.
• Liver cancer.Because the healthy liver tissues get destroyed in cirrhosis, the chances of liver cancer increase exponentially.
• Weak bones (osteoporosis).Patients of ursodeoxycholic acid primary biliary cirrhosis are at more risk of developing brittle and weaker bones.
• Vitamin deficiencies.The digestive system loses its ability to absorb fat-soluble vitamin A, D, E and K and fats due to the decrease of the bile production. This can result in a deficiency of important vitamins in the body. However, this happens only in advanced cases of PBC.
• Decreased mental function (hepatic encephalopathy). Patients who have suffered liver failure because of primary biliary cirrhosis might suffer from personality changes and start having problems with their concentration and memory.
• An increased risk of other diseases.Patients of primary biliary cirrhosis do not only suffer from liver and bile duct damage. They are at a greater risk of developing immune system and metabolic disorders like rheumatoid arthritis, CREST syndrome or limited scleroderma and thyroid problems.