Billary cirrhosis

Book an Appointment

Overview

Primary biliary cirrhosis (PBC) is an autoimmune, slowly progressive, cholestatic, liver disease characterized by a triad of chronic cholestasis, circulating anti-mitochondrial antibodies (AMA), and characteristic liver biopsy findings of nonsuppurative destructive cholangitis and interlobular bile duct destruction. About 10% of PBC patients, however, lack AMA. A variant, called PBC-autoimmune hepatitis (AIH) overlap, is characterized by the above findings of PBC together with findings of elevated serum alanine aminotransferase, elevated serum immunoglobulin G, and circulating anti-smooth muscle antibodies, with liver biopsy demonstrating periportal or periseptal, lymphocytic, piecemeal necrosis. PBC is hypothesized to be related to environmental exposure in genetically vulnerable individuals. It typically occurs in middle-aged females. Prominent clinical features include fatigue, pruritis, jaundice, xanthomas, osteoporosis, and dyslipidemia. The Mayo Risk score is the most widely used and best prognostic system. Ursodeoxycholic acid is the primary therapy. It works partly by reducing the concentration and injury from relatively toxic bile acids. PBC-AIH overlap syndrome is treated with ursodeoxycholic acid and corticosteroids, especially budesonide. Obeticholic acid and fibrate are promising new, but incompletely tested, therapies. Liver transplantation is the definitive therapy for advanced disease, with about 70% 10-year survival after transplantation. Management of pruritis includes local skin care, dermatologist referral, avoiding potential pruritogens, cholestyramine, and possibly opioid antagonists, sertraline, or rifaximin. Management of osteoporosis includes life-style modifications, administration of calcium and vitamin D, and alendronate. Statins are relatively safe to treat the osteopenia associated with PBC. Associated Sjogren’s syndrome is treated by artificial tears, cyclosporine ophthalmic emulsion to stimulate tear production; and saliva substitutes, cholinergic agents, and scrupulous oral and dental care. Complications of cirrhosis from advanced PBC include esophageal varices, ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, and hepatoma formation.


Symptoms

Initially patients may experience fatigue, weakness and weight loss. During later stages, patients may develop jaundice (yellowing of the skin), gastrointestinal bleeding, abdominal swelling and confusion.

People may experience:

Pain areas: in the abdomen

Gastrointestinal: bleeding, dark stool from digested blood, fluid in the abdomen, nausea, passing excessive amounts of gas, vomiting blood, or water retention

Whole body: fatigue, loss of appetite, or reduced hormone production

Skin: web of swollen blood vessels in the skin or yellow skin and eyes

Weight: weight gain or weight loss

Also common: bleeding, breast augmentation, bruising, dark urine, enlarged veins around belly button, itching, mental confusion, muscle weakness, shortness of breath, swelling, swelling in extremities, or swollen veins in the lower oesophagus


Causes

Bile is a liquid produced inside the liver that's used to help digest fats and remove waste products from the body. It passes out of the liver through small tubes called bile ducts.

In PBC, the immune system (the body's natural defence against infection and illness) mistakenly attacks the bile ducts.

It's not clear why this happens, but it's thought to be caused by a combination of subtle differences in how the immune system works.

The bile ducts become damaged and injured, causing bile to build up in the liver. This further damages the liver and may lead to scarring (cirrhosis).

Risk factors

Sex. Most people with primary biliary cholangitis are women.

Age. It's most likely to occur in people 30 to 60 years old.

Genetics. You're more likely to get the condition if you have a family member who has or had it.

Geography.

Chronic alcohol abuse.

Chronic viral hepatitis (hepatitis B, C and D)

Fat accumulating in the liver (nonalcoholic fatty liver disease)

Iron buildup in the body (hemochromatosis)

Cystic fibrosis.

Copper accumulated in the liver (Wilson's disease)

Poorly formed bile ducts (biliary atresia)

Calendar Schedule

Have a medical question?

We are available to help you with all your questions and concerns.

Complications

Ascites is the most common complication of cirrhosis[7]. It is also the most common complication that leads to hospital admission[29]. Approximately 15% of the patients with ascites will die in one year and 44% will die in five years

Liver scarring (cirrhosis). ...

Enlarged veins (varices). ...

Increased pressure in the portal vein (portal hypertension). ...

Enlarged spleen (splenomegaly). ...

Gallstones and bile duct stones. ...

Liver cancer. ...

Weak bones (osteoporosis). ...

Vitamin deficiencies..


Prevention

Stop drinking alcohol. Treat chronic hepatitis (if you have it). Avoid medications that stress the liver. Eat a healthy, well-balanced, low-fat diet, such as the Mediterranean diet.Reduce your risk of cirrhosis by taking these steps to care for your liver:

Do not drink alcohol if you have cirrhosis. If you have liver disease, you should avoid alcohol.

Eat a healthy diet. Choose a plant-based diet that's full of fruits and vegetables. Select whole grains and lean sources of protein. Reduce the amount of fatty and fried foods you eat.

Maintain a healthy weight. An excess amount of body fat can damage your liver. Talk to your doctor about a weight-loss plan if you are obese or overweight.

Reduce your risk of hepatitis. Sharing needles and having unprotected sex can increase your risk of hepatitis B and C. Ask your doctor about hepatitis vaccinations.

If you're concerned about your risk of liver cirrhosis, talk to your doctor about ways you can reduce your risk.