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Primary Biliary Cirrhosis (Cholangitis) (Risk factors (Smoking, Past…
Primary Biliary Cirrhosis (Cholangitis)
Epidemiology
Much more common in FEMALES than males
Typical age at presentation is 50 yrs
Women aged 40-50 yrs constitute 90% patients
Cause is unknown but its thought to be immunological and serum anti-mitochondrial antibodies (AMA) are found in almost all patients
A chronic disorder with progressive destruction of small bile ducts, leading to cirrhosis
Risk factors
Smoking
Past pregnancy
Many UTIs
Other autoimmune disease
Positive family history
Use of nail polish/hair dye
Pathophysiology
Serum anti-mitochondrial antibodies (AMA) found in almost all patients
Likely than an environmental factor acts on genetically predisposed hosts to trigger disease
Interlobular bile ducts are damaged by CHRONIC AUTOIMMUNE GRANULOMATOUS INFLAMMATION resulting in cholestasis which may lead to fibrosis, cirrhosis and portal hypertension
Clinical presentation
Lethargy and fatigue (which is frequently disabling) may accompany pruritus and precedes jaundice
When jaundice appears hepatomegaly is usually present
Pruritus (itching) is often the earliest symptom
Pigmented xanthelasma (yellow fat deposits under skin usually around eyelids)
Asymptomatic patients are discovered on routine examination or screening and may have hepatomegaly, a raised serum alkaline phosphate or anti-mitochondrial antibodies (AMA)
Complications
Osteoporosis
Malabsorption of fat-soluble vitamins (A, D, E, K) due to cholestasis
Cirrhosis
Decreased bilirubin in the gut lumen results in osteomalacia and coagulopathy
Differential Diagnosis
Autoimmune cholangitis
Extrahepatic biliary obstruction should be excluded by ultrasound
Diagnosis
Ultrasound
Can show a diffuse alteration in liver architecture and excludes extra hepatic cholestasis
Liver biopsy shows characteristic histological features
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
Blood
Anti-mitochondrial antibodies (AMAs) - present in 95%, M2 antibody is 98% specific
Raised serum IgM
Raised serum cholesterol
Increased alkaline phosphate
Treatment
Bisphosphonates for osteoporosis
Pruritus
Colestyramine works but unpalatable
Naloxone and naltrexone (opioid antagonists) shown to help
Supplementation of Vitamin A, D, E, K (fat soluble)
Ursodeoxycholic acid
Improves bilirubin and aminotransferase levels
Due to lack of effective medical therapy, primary biliary cirrhosis is a major indication for LIVER TRANSPLANTATION