Primary biliary cirrhosis is a disease in which bile ducts of the liver slowly fail. When bile ducts are destroyed toxins build up in the liver, which can lead to irreversible scarring of tissue. Cirrhosis refers to scar tissue of the liver. Bile is a fluid produced by the liver, which is important for fat digestion. Bile also helps the body to remove unwanted harmful substances – such as cholesterol, toxic metals, and old red blood cells. This is a chronic disease.
|Primary biliary cirrhosis
Patients with primary biliary cirrhosis can remain symptom-free for years –even after diagnosis. Symptoms experienced in the early stages of the disease are fatigue, itching, and dry eyes and mouth.
Fatigue comes in cycles, patients feel normal in the morning, but soon become exhausted in the afternoon. There is no association between the degree of tiredness and the progression or severity of the disease.
Pruritis or itching begins with the hands and feet and can move throughout the entire body. Itching may become more bothersome in the evening and less so in morning. Itching can be so severe that sleep is interrupted further exhausting patients with primary biliary cirrhosis.
Dry eyes and mouth are known as sicca syndrome. This symptom occurs mostly in patients with autoimmune diseases or disorders. Inflammation in the moisture secreting glands decreases saliva and tear production. This leads to swallowing problems, corneal ulcers and light sensitivity.
As the disease becomes more severe patients can experience jaundice, hyperpigmentation, edema, swollen or distended abdomen, cholesterol deposits, digestive difficulties, and urinary tract infections.
Jaundice causes the skin and whites of the eyes to turn yellow. It is the result of high levels of bilirubin in the blood stream. Normally bile ducts filter out bilirubin from the liver to the digestive tract to be expelled from the body. As the ducts are damaged more and more, their functioning decreases and the bilirubin builds up in the bloodstream.
Hyperpigmentation is due to poor bile flow – which increases melanin a skin pigment. Sometimes the deeper color is inconsistent and the skin appears blotchy.
Edema is swollen feet caused by the retention of salts and fluids that are normally controlled by the bile ducts. These fluids can also collect in the abdomen. Swelling of the abdomen is also referred to as ascites. Swelling occurs mostly later in the day in the initial appearance of this symptom.
Cholesterol deposits are caused by the body’s inability to filter excess cholesterol, as it does with healthy bile ducts. Cholesterol deposits appear on within the skin around the eyes on the eyelids, on the creases of the hands and soles of the feet. Not everyone with this disease will get cholesterol deposits. Cholesterol deposits are also known as xanthomas. The cholesterol is the ‘good cholesterol’ and does not increase the chances of heart disease.
Digestive problems appear as diarrhea and steatorrhea – or greasy, foul smelling fatty stools. These problems, like the other symptoms are due to the decrease of bile in the digestive tract.
Urinary tract infections are mostly seen in women with primary biliary cirrhosis.
Diagnosis of primary biliary cirrhosis is often made incidentally during blood tests for evaluating for other conditions, before there are any signs or symptoms of the disease.
Tests used to diagnose primary biliary cirrhosis are liver function tests, ultrasound imaging, anti-mitochondrial antibodies and a liver biopsy.
A liver function test will check the levels of enzymes such as alkaline phosphatase in the blood.
Ultrasound imaging uses high frequency sound waves to create a clear image of the liver and bile ducts, it can rule out other causes of bile flow obstruction such as gallstones and tumors.
Anti-mitochondrial antibodies are found in every cell and are used to destroy bacteria and pathogens. Patients with primary biliary cirrhosis can have an increased number of these antibodies, but a false positive test is possible.
A liver biopsy takes a small piece of liver tissue for microscopic examination by a pathologist. The tissue is removed using a small incision and a thin needle. Patients may be uncomfortable and in pain for a day or two after the biopsy. Biopsies can also be used over time to chart and monitor the progression of this disease.
There is no cure for primary biliary cirrhosis, treatment goals are to slow the progression of the disease, ease or relieve symptoms and preventing further complications.
Treatments used to slow the progression of the disease are medications. When treatment can no longer control the progression a liver transplant will be considered. Patients with primary biliary cirrhosis that receive a liver transplant often thrive, though there is a chance that the disease may recur.
There have been no therapies or medication discovered that improve the fatigue associated with primary biliary cirrhosis. The pruitis or severe itching can be controlled through the use of choletyramine, colestipol, rifampicin, and opioid antagonists
Regular check ups and working closely with a medical practitioner will greatly improve the quality of life of people with primary biliary cirrhosis.
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