Primary biliary cirrhosis Flashcards
Another name
Cholangitis
Most common patient
Women aged 40-50 (90% of patients)
Far more common in females
Typical age of presentation is 50
Describe
A chronic disorder with progressive destruction of small bile ducts, leading to cirrhosis
Aetiology
Unknown
But immunological and serum anti-mitochondrial antibodies (AMA) are found in almost all patients
Risk factors
- Positive family history
- Many UTIs
- Smoking
- Past pregnancy
- Other autoimmune disease
- Use of nail polish/hair dye
Pathophysiology
- Interlobar bile ducts are damaged by CHRONIC AUTOIMMUNE GRANULOMATOUS INFLAMMATION resulting in cholestasis which may lead to fibrosis, cirrhosis and portal hypertension
- Serum anti-mitochondrial antibodies (AMA) found in almost all patients
- Likely that an environmental factor acts on genetically predisposed hosts
to trigger disease
Complications
- Cirrhosis
- Osteoporosis
- Malabsorption of fat-soluble vitamins (A,D,E,K) due to cholestasis
- Decreased bilirubin in the gut lumen results in osteomalacia and coagulopathy
Clinical presentation
Asymptomatic patients are discovered on routine examination or screening and may have hepatomegaly, a raised serum alkaline phosphate or anti- mitochondrial antibodies (AMA)
Pruritus (itching) and jaundice after
Lethargy and fatigue
Pigmented xanthelasma (yellow fat deposits under skin usually around eyelids) on eyelids deposits of cholesterol in the creases of the hands may be seen
What is earliest symptom
Pruritus (itching)
Differential diagnosis
- Autoimmune cholangitis
- Extrahepatic biliary obstruction should be exclude by ultrasound
Diagnosis
Blood
Ultrasound (can show alteration in liver architecture)
Liver failure shows characteristic histological features
What would you look for on blood test
- Increased alkaline phosphate
- Raised serum cholesterol
- Anti-mitochondrial antibodies (AMAs) - present in 95%, M2 antibody is 98% specific
- Raised serum IgM
Histological features of liver failure
- Portal tract infiltrate, mainly of lymphocytes and plasma cells
- Around 40% have granulomas
- Damage to and loss of small bile ducts and ductular proliferation
- Portal tract fibrosis and eventually cirrhosis is seen
Treatment
- URSODEOXYCHOLIC ACID:
Improves bilirubin and aminotransferase levels - Supplementation of Vitamin A,D,E,K (fat-soluble vitamins)
- BISPHOSPHONATES for osteoporosis
- Due to lack of effective medical therapy, primary biliary cirrhosis is a major indication for LIVER TRANSPLANTATION
Treatment of Pruritus
- COLESTYRAMINE works but unpalatable
* NALOXONE and NALTREXONE (opioid antagonists) shown to help