Summary about Disease
Biliary cirrhosis is a chronic liver disease characterized by the progressive destruction of the bile ducts within the liver. This leads to bile buildup (cholestasis), inflammation, scarring (fibrosis), and eventually cirrhosis, impairing liver function. The two main types are primary biliary cirrhosis (PBC), now often referred to as primary biliary cholangitis, and secondary biliary cirrhosis. PBC is an autoimmune disease, while secondary biliary cirrhosis is usually caused by a blockage of the bile ducts.
Symptoms
Symptoms vary depending on the stage of the disease. Early symptoms may be mild or absent. Common symptoms include:
Fatigue
Itching (pruritus)
Darkening of the skin (hyperpigmentation)
Jaundice (yellowing of the skin and eyes)
Pale stools
Dark urine
Swelling in the legs, ankles, or feet (edema)
Fluid buildup in the abdomen (ascites)
Enlarged spleen (splenomegaly)
Bone pain or fractures (due to osteomalacia or osteoporosis)
Xanthelasma (yellowish cholesterol deposits around the eyes)
Xanthomas (cholesterol deposits in the skin, tendons, or other tissues)
Causes
Primary Biliary Cholangitis (PBC): An autoimmune disease where the body's immune system mistakenly attacks the small bile ducts in the liver. The exact cause of PBC is unknown, but genetic predisposition and environmental factors are believed to play a role.
Secondary Biliary Cirrhosis: This occurs when the bile ducts become blocked for a prolonged period. Common causes include:
Gallstones in the bile ducts
Scarring or narrowing of the bile ducts after surgery
Tumors blocking the bile ducts
Congenital abnormalities of the bile ducts (e.g., biliary atresia)
Pancreatitis
Medicine Used
4. Medicine used
Ursodeoxycholic acid (UDCA): This medication helps improve bile flow from the liver and can slow the progression of PBC. It is the first-line treatment for PBC.
Obeticholic acid (OCA): Sometimes used in combination with UDCA for PBC patients who don't respond adequately to UDCA alone.
Fibrates: May be used off-label to help reduce itching and improve liver function in PBC patients.
Cholestyramine: A bile acid sequestrant used to relieve itching by binding bile acids in the intestine.
Calcium and Vitamin D supplements: To prevent or treat bone loss.
Medications to treat complications: Diuretics for fluid retention, beta-blockers for variceal bleeding, etc.
Liver transplant: A liver transplant may be necessary in advanced cases of biliary cirrhosis when the liver is failing.
Is Communicable
No, biliary cirrhosis (both primary and secondary) is not communicable. It cannot be spread from person to person through contact, air, or bodily fluids.
Precautions
Precautions focus on managing symptoms and slowing disease progression:
Follow doctor's instructions: Take medications as prescribed and attend regular check-ups.
Avoid alcohol: Alcohol can further damage the liver.
Maintain a healthy lifestyle: Eat a balanced diet, exercise regularly, and maintain a healthy weight.
Manage itching: Keep skin moisturized, avoid hot baths, and use prescribed medications.
Prevent infections: Get vaccinated against hepatitis A and B, influenza, and pneumococcal pneumonia.
Monitor for complications: Be aware of symptoms of liver failure (e.g., confusion, ascites) and seek medical attention promptly.
Protect bones: Take calcium and vitamin D supplements as prescribed and get regular bone density screenings.
Quit smoking: Smoking can worsen liver disease.
How long does an outbreak last?
Biliary cirrhosis is a chronic, progressive disease. There are no "outbreaks" in the traditional sense. The condition progresses over years or decades.
How is it diagnosed?
Diagnosis involves a combination of:
Blood tests: Liver function tests (ALT, AST, alkaline phosphatase, bilirubin), antibody tests (antimitochondrial antibodies - AMA), and other tests to assess liver function and rule out other conditions.
Imaging studies: Ultrasound, CT scan, or MRI to visualize the liver and bile ducts and look for blockages or other abnormalities.
Liver biopsy: A small sample of liver tissue is taken and examined under a microscope to confirm the diagnosis and assess the severity of the disease.
Timeline of Symptoms
9. Timeline of symptoms The timeline of symptoms can vary greatly from person to person.
Early stages: Many people have no symptoms or only mild symptoms such as fatigue or itching. Liver function tests may be abnormal.
Progression: As the disease progresses, symptoms become more noticeable. Jaundice, skin darkening, edema, ascites, and other complications may develop.
Late stages: In advanced cirrhosis, liver failure can occur, leading to serious complications such as hepatic encephalopathy, variceal bleeding, and liver cancer.
Important Considerations
Early diagnosis is crucial: Early diagnosis and treatment with UDCA can slow the progression of PBC and improve outcomes.
Regular monitoring is essential: People with biliary cirrhosis need regular monitoring by a hepatologist (liver specialist) to assess liver function and manage complications.
Liver transplant is a life-saving option: Liver transplant can be a successful treatment for advanced biliary cirrhosis when the liver is failing.
Support groups can be helpful: Joining a support group can provide emotional support and information for people with biliary cirrhosis and their families.
Consider genetic testing: Although not routine, genetic testing may be considered in some cases, particularly in those with a family history.