Primary biliary cholangitis Flashcards

1
Q

What is PBC

A

autoimmune condition characterised by granulomatous destruction of the intrahepatic biliary ducts, leading to cholestasis and subsequent leakage of bile into the circulation.

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2
Q

PBC epidemiology

A
  • Rare disease with a prevalence of < 0.05%
  • Middle-aged:peak incidence between 45 and 60 years old
  • Female gender: ten times more common in females
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3
Q

Aetiology

A

immunological and serum anti-mitochondrial antibodies (AMA) - T cells target the cells in intrahepatic ducts and destroy them

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4
Q

RFs

A

F
Autoimmune conditions
FH
Smoking
Pregnancy

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5
Q

Pathophysiology of FBC

A

The inflammatory process coupled with trapping of bile acids in the liver leads to progressive fibrosis, cirrhosis, and eventually liver failure.

The presence ofanti-mitochondrial antibodies (AMAs) has been observed in almost all patients with PBC, further reinforcing the likely autoimmune nature of the condition.

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6
Q

50% of PBC patients have atleast one assosciated autoimmune condition such as

A
  • Sjögren’s syndrome (25%)
  • Raynaud’s phenomenon (25%)
  • Autoimmune thyroid disease (25%)
  • Rheumatoid arthritis (20%)
  • Systemic sclerosis(10%)
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7
Q

Key presentation

A

significant itching in a middle-aged female. Some patients may be asymptomatic and be simply diagnosed on a routine blood test demonstrating abnormal LFTs (e.g. raised ALP).

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8
Q

Signs

A
  • Skin hyperpigmentation: due to increased melanin
  • Clubbing
  • Mild hepatosplenomegaly
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9
Q

Symptoms

A
  • Pruritis (itchy skin) - leakage of bile salts
  • Fatigue and weight loss
  • Dry eyes and dry mouth - Sjögren’s syndrome
  • Obstructive jaundic
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10
Q

Primary investigations

A
  • LFTs
  • Antimitochondrial antibodies (AMA):present in 95% of patients (highly specific)
  • Antinuclear antibodies (ANA):present in 50% of patients
  • Raised serum cholesterol
  • Maybe MRCP + Liver biopsy
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11
Q

DDs

A
  • Primary sclerosing cholangitis - AMA would not found
  • Obstructive bile duct lesion
  • Drug induced cholestasis - can do liver biopsy
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12
Q

1st line management

A
  • Ursodeoxycholic acid
    • First-line agent in all patients, bile acid analgoue which dampen inflammatory response
  • Fat soluble vitamin supplementation
  • Codeine
  • Bisphosphonates
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13
Q

2nd line

A

Liver transplantation

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14
Q

Monitoring

A

Regular LFTs + US

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15
Q

Complications

A
  • Malabsorptionof fat-soluble vitamins A, D, E and K due to cholestasis; may result in coagulopathy due to decreased bilirubin in gut lumen
  • Hypercholesterolaemia:cholestasis is associated with hypercholesterolaemia
  • Liver cirrhosis:end-stage disease results in fibrosis and eventual cirrhosis, whilst portal hypertension may cause ascites and variceal bleeding
  • Hepatocellular carcinoma: 20-fold increased risk
  • Metabolic bone disease:osteoporosis and osteomalacia
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