PBC & PSC

Primary Biliary Cirrhosis (PBC)

PBC: introduction

Chronic granulomatous inflammation leading to progressive destruction of interlobular bile ducts.

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PBC: clinical features

Patient
- middle-aged women (8:1 = female:male)

Present
- incidental LFTs
- pruritus
- chronic liver disease

Complications
- malabsorption: osteomalacia, coagulopathy
- cirrhosis: portal HTN, hepatic encephalopathy, HCC

Associated conditions
- Sjogren’s, systemic sclerosis, CREST, RA, Hashimoto’s...

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PBC: investigations

Bloods
- LFTs: cholestatic
- autoAbs *: AMA M2 subtype in 98 %, others
- increased ­ IgM

Special
- USS: exclude extra-hepatic cholestasis
- ERCP
- biopsy: granulomas, copper accumulation

* AMA = anti-mitochondrial antibodies

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PBC: management

Symptoms
- pruritus: cholestyramine
- diarrhoea: codeine phosphate

Specific
- supplemental vitamins: ADEK
- ursodeoxycholic acid

End-stage
- liver transplant

Prognosis
- median survival: 7 – 10 years
- jaundiced: < 2 years
- post-transplant: > 5 years

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Primary Sclerosing Cholangitis (PSC)

PSC: introduction

Inflammation, fibrosis and stricturing of the intra- and extra- hepatic bile ducts.

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PSC: clinical features

Patient
- young
- male:female = 2:1

Presents
- incidental LFTs
- fluctuating symptoms: jaundice, pruritus, abdominal pain

Complications
- bacterial cholangitis
- cholangiocarcinoma (CA19-9 +ve)
- portal hypertension

Associated conditions
- UC in 80 %
- rarely Crohn’s / HIV

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PSC: investigation

Bloods
- LFTs
- auto-antibodies: SMA, ANCA

Special
- ERCP: multiple strictures of biliary tree, classical “beading”
- Biopsy: obliterative “onion-skinning” fibrosis

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PSC: management

Drugs
- cholestyramine (prevents reabsorption of bile acids)
- ursodeoxycholic acid

End-stage
- liver transplant

Prognosis
- median survival = 10 years

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