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Biliary Cirrhosis
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Fatigue, pruritus, jaundice
Complications	Liver failure, portal hypertension, osteoporosis
Onset
Duration
Types	N/A
Causes	Autoimmune destruction of bile ducts
Risks	Female gender, genetic predisposition
Diagnosis	Liver biopsy, antimitochondrial antibody test
Differential diagnosis	N/A
Prevention	N/A
Treatment	Ursodeoxycholic acid, liver transplant
Medication	N/A
Prognosis	Variable, depends on stage at diagnosis
Frequency
Deaths	N/A

Overview

Biliary Cirrhosis is a chronic liver disease characterized by progressive destruction of the small and medium-sized bile ducts within the liver. This leads to cholestasis, fibrosis, and eventually cirrhosis. The condition is primarily autoimmune in nature and is more prevalent in women.

Pathophysiology

The pathogenesis of biliary cirrhosis involves an autoimmune attack on the bile ducts. The immune system mistakenly targets the epithelial cells lining the bile ducts, leading to inflammation and destruction. Over time, this results in cholestasis, where bile cannot flow from the liver to the duodenum, causing liver damage and fibrosis.

Clinical Presentation

Patients with biliary cirrhosis often present with non-specific symptoms such as fatigue and pruritus. As the disease progresses, jaundice, hepatomegaly, and signs of portal hypertension may develop. Advanced disease can lead to complications such as liver failure and osteoporosis.

Diagnosis

Diagnosis of biliary cirrhosis is based on clinical, biochemical, and histological findings. Key diagnostic tests include:

• Liver function tests showing cholestatic pattern
• Presence of antimitochondrial antibodies (AMA) in the blood
• Liver biopsy demonstrating bile duct destruction and fibrosis

Management

The primary treatment for biliary cirrhosis is ursodeoxycholic acid, which can slow disease progression. In cases of advanced cirrhosis, liver transplantation may be necessary. Management also includes addressing symptoms such as pruritus and monitoring for complications like osteoporosis.

Prognosis

The prognosis of biliary cirrhosis varies depending on the stage at diagnosis and response to treatment. Early diagnosis and treatment can improve outcomes, but advanced disease may lead to liver failure and require transplantation.

Epidemiology

Biliary cirrhosis is more common in women, with a female-to-male ratio of approximately 9:1. It typically presents in middle-aged individuals, although it can occur at any age.

See Also

• Cirrhosis
• Autoimmune hepatitis
• Primary sclerosing cholangitis

External Links

• American Liver Foundation
• Hepatitis Foundation International