Primary Biliary Cirrhosis

Primary biliary cirrhosis, sometimes called PBC, is an autoimmune disease marked by slow progressive destruction of bile ducts.

Bile helps your body absorb fat, fat soluble vitamins and eliminateworn-out red blood cells, cholesterol and toxins.

When bile ducts are damaged, as in primary biliary cirrhosis, toxic waste products can accumulate in your liver and sometimes lead to permanent scarring of liver tissue (cirrhosis).

A combination of genetic and environmental factors is thought to be responsible for the disease.

Medicationsare useful in slowing downthe disease progression, especially if treatment begins early.

Primary biliary cirrhosis may not be symptomatic in many people for years while other people experience signs and symptoms like:

• Fatigue
• Itchy skin 
• Dry eyes and mouth 

As the disease progresses, the signs and symptoms become more diverseand may include:

• Pain in the upper right portion of the abdomen
• Pain in bone, muscle or joint (musculoskeletal pain)
• Yellowing of the skin and eyes (jaundice) 
• Skin darkening unrelated to sun exposure (hyperpigmentation) 
• Swollen feet and ankles (edema) 
• Fluid accumulation in the abdomen due to liver failure (ascites) 
• Fatty deposits on the skin around your eyes, your eyelids, or in the creases in your palms, soles, elbows or knees (xanthomas) 
• Weak and brittle bones (osteoporosis), which can lead to fractures 
• Elevated cholesterol 
• Diarrhea that can be greasy (steatorrhea) 
• Decreased thyroid activity (hypothyroidism)

The cause of primary biliary cirrhosis is not clear. Many experts consider primary biliary cirrhosis an autoimmune disease in which the body assumes its own cells as foreign substances and invades them.

How primary biliary cirrhosis develops? 

The whole process starts when your body launches immune attack against the cells that line small bile ducts in your liver.

The T cells begin accumulating in your liver and cause inflammation. T cells are white blood cells that are part of your immune system. T cells fight against foreign bodies such as bacteria to keep your body healthy.

But in autoimmune diseases, they don’t recognize their own cells and turn hostile.

Inflammation in the smallest ducts spreads, in time, and destroys nearby liver cells.

As the disease progresses, more liver cells are destroyed and replaced by scar tissue (fibrosis) often leading to cirrhosis, a condition characterized by permanent scarring of liver tissue.

Making a diagnosis of primary biliary cirrhosis is done by performing several tests.

Visit your doctor if you have signs or symptoms that worry you.

Once diagnosed with PBC, you may be referred to a doctor who specializes in disorders of the digestive system (Gastroenterologist) or a doctor who specializes in liver diseases (Hepatologist).

The following tests and procedures may be used to diagnose primary biliary cirrhosis.

Laboratory tests

• Blood tests to check liver function: Liver function tests measure the enzyme levels, fluctuations in which can indicate liver disease and bile duct injury. 
• Blood tests to check for signs of autoimmune disease: Your blood can be tested for anti-mitochondrial antibodies (AMAs), the antibodies that exclusively occur in people with PBC. A positive AMA test confirms the presence of the disease. However, a few people with primary biliary cirrhosis don't have AMAs. 

Imaging tests

• Ultrasound: Ultrasound uses high-frequency sound waves to view the structures inside your body.
• Computerized tomography (CT scan): CT scan combines many X-ray images to create detailed, often three dimensional images of structures inside your body.
• Magnetic resonance imaging (MRI): An MRI scanner uses a magnetic field and radio waves to create detailed images of organs and tissues. It does not use radiation like X-ray.
• Magnetic resonance elastography (MRE):It is an advanced MRI based imaging technique that can assess hardening of your liver, indicative of cirrhosis.
• X-rays of your bile ducts: In addition to MRI, your doctor may recommend a type of bile duct X-ray called endoscopic retrograde cholangiopancreatography (ERCP). This is an invasive process and may lead to complications. It is seldom required for diagnosis.
• Liver biopsy: Biopsy provides a definitive and confirmatory diagnosis for the disease. It involves removal of a small amount of liver tissue which is then examined in a laboratory, either to confirm the diagnosis or to determine the stage of the disease.

Till now, no treatment has been proven to cure Primary Biliary Cirrhosis. The focus of all the available treatments is to slow down the disease progress, relieve the symptoms and prevent further complications.

The treatment approaches are:

Treating the disease

• Ursodeoxycholic acid (UDCA): Also calledursodiol, UDCA is considered the first line of therapy. When started early, it may prolong life but it doesn’t cure the disease.It improves symptoms and prevents development of advanced liver disease. Common side effects are weight gain, hair loss and diarrhea.
• Liver transplant: When no available treatment can halt the disease progression and the liver becomes prominently scarred, a liver transplant is considered.A healthy liver from donor is transplanted in the patient. Primary biliary cirrhosis can also develop in the transplanted liver but the process takes several years. Significantly higher survival rates are observed in patients who have had liver transplant.

New medications: Many drugs are being investigated for their probable activity in treating primary biliary cirrhosis.Immunosuppressants, especially methotrexateand colchicine, have been widely used, but their effectiveness has not been established. Antiviral drugs are under study. According to small studies, patients whose symptoms do not improve with UDCA could benefit from combination of fenofibrate or bezafibrate with UDCAbut larger studies are needed for generalizing the results.

Treating the symptoms

Your doctor may recommend treatments that alleviate symptoms and comfort you.

• Treatment for fatigue: Fatigue associated with primary biliary can be managed well by lolifestyle modifications, proper diet and adequate rest. A drug called modafinil, a central nervous system stimulant has shown promise in relieving fatigue symptoms.
• Treatment for itching: Anti-allergic medications like diphenhydramine (Benadryl) and loratadine (Alavert, Claritin,) are can be used to relieve itching.
• Cholestyramine (Prevalite) is an agent that reduces blood cholesterol. Cholestyramine has shown beneficial effects for most people, however it is unpalatable.
• Rifampin (Rifadin, Rimactane, others), an anti-tubercular agent is shown to reduce itching but the exact mechanism behind this is still not known.
• Opioid antagonists such as naloxone (Bunavail, Evzio) and naltrexone (Vivitrol) can also be used to reduce itching.
• Treatment for dry eyes and mouth: OTC (over- the- counter) artificial tears and saliva substitutes may help mitigate the problems. If they do not help, a drug called pilocarpine (IsoptoCarpine,Salagen) may be used to relieve dry eyes.

Preventing complications

Some common complications that occur primary biliary cirrhosis are:

• Increased pressure in the portal vein (portal hypertension): Regular screening for portal hypertension and enlarged veins is necessary and if confirmed to have portal hypertension, you may be prescribed drugs like nitrates, beta blockers or surgery.
• Weak bones (osteoporosis): Calcium and vitamin D supplements can be recommended for bone loss associated with the disease. Regular exercise can also help increase bone density. Consult with your doctor for selecting the right exercise regimen for you.
• Vitamin deficiencies: Primary biliary cirrhosis can cause deficiency of fat soluble vitamins. Your doctor may prescribe you the supplements.

Here are some suggestions that might make you feel better and cope better with the symptoms of primary biliary cirrhosis:

• Reduced sodium intake: Sodium cause water retentions and may contribute to tissue swelling or accumulation of fluid in your abdominal cavity (ascites). Chose low-sodium foods.
• Exercise: Indulge in some sort of physical activity that best suits your condition.Regular exercise may reduce your risk of bone loss. 
• Avoid alcohol: Liver is the organ that metabolizes alcohol. Alcohol stresses you liver to work harder. People with primary biliary cirrhosis should abstain from alcohol.
• Diets or supplements that stress your liver: Liver is the major site for metabolism of drugs and other supplements. Intake of a drug or supplement could possibly stress your already weakened liver. Talk to your doctor before starting any new medication or dietary supplement.

Living with chronic diseases including liver disease is frustrating and diseases with no cure add to the woes. You may feel as if you have been caught in the dead end. Such feelings can lower quality of your life.

To better cope with some distressing feeling, try these tips:

• Learn about your condition: Having an insight of what’s going on with your body could ease your symptoms and prepare you to accept the condition as it is.
• Learn more about the condition fromconversation with your doctor, local library and on websites affiliated with reputable organizations, such as the American Liver Foundation. 
• Eating well, exercising and getting enough rest can help you feel better.
• Spend time with your family and friends.

Primary biliary cirrhosis can be exhausting, so accept the help if someone wants to do your grocery shopping, wash a load of laundry or cook your dinner.

Join a support a support group and share what’s going on in your mind. Develop positive attitude towards life.

There are several risks and complications associated with primary biliary cirrhosis.

Risks

• Sex: Women are more prone to primary biliary cirrhosis are women.
• Age: Primary biliary cirrhosis is most likely to occur in people 30 to 60 years old.
• Genetic factors:Genetic factors combined with environmental factors like infection, smoking can put you at risk of the disease.

Complication

• Cirrhosis: Cirrhosis is permanent scarring of liver causing reduced or cessation of normal liver functions. Cirrhosis can progress to liver failure.
• Increased pressure in the portal vein (portal hypertension): Portal vein carries blood from intestine, spleen and pancreas to the liver.Cirrhosis can block normal blood flow through the portal vein and lead to increased pressure within the vein.
• Enlarged spleen (splenomegaly): Your spleen can become enlarged with white blood cells and platelets due to portal hypertension.
• Gallstones and bile duct stones: Stones can form in the gall bladder and bile duct due to reduced bile floe in the ducts, causing pain and infection.
• Enlarged veins (varices): Obstruction of blood flow in the portal vein can cause blood accumulation in the veins of your stomach and esophagus often leading to bleeding in your upper stomach or esophagus. This bleeding is a life-threatening emergency that requires immediate medical care.
• Liver cancer: Live cancer may be resultant to damages of healthy liver tissue.
• Weak bones (osteoporosis): People with primary biliary cirrhosis have an increased risk of brittle bones that are more prone to fracture.
• Vitamin deficiencies: Decrease absorption of fat soluble vitamins, owing to bile deficiency, can lead to deficient states.
• Decreased mental function (hepatic encephalopathy): Cognitive problems like those with memory, concentration and personality change have been observed in some people with PBC.

Other metabolic or immune system disorders such as thyroid problems, limited scleroderma (CREST syndrome) and rheumatoid arthritis may also be present.