Friday, 1 October 2010

MRCP revision battle 24.1: Primary biliary cirrhosis

All this working and revising is leaving me with little internet-publishable material to put into my daily intros.  Looking for inspiration I ended up stumbling accross an utterly brilliant set of articles written back in the early 2000's by a (then) junior doctor called Michael Foxton in the Guardian.  Hopefully this link will take you to a list of his articles to soothe you after some revision.... but I warn you not to start looking before you've finished revising for the day because otherwise there is a real danger you will get no revision done!!

So today's battles are:

MRCP revision battle 24.1: Primary biliary cirrhosis
MRCP revision battle 24.2: Acute Myeloid Leukaemia
MRCP revision battle 24.3: Acute Promyelocytic Leukaemia
MRCP revision battle 24.4: Angioid retinal streaks
MRCP revision battle 24.5: SIADH
MRCP revision battle 24.6: Hyponatraemia
MRCP revision battle 24.7: Neutropenia and neutrophillia

MRCP revision battle 24.1: Primary biliary cirrhosis

I've never bonded well with this topic so this battle is a personal Waterloo for me...

Primary biliary cirrhosis is thought to be an autoimmune condition in which the interlobular bile ducts are damanged by chronic granulomatous inflammation. 

The result of this is:
  • progressive cholestasis
  • cirrhosis
  • portal hypertension

Females are more affected than males (9:1) and it tends to present in middle age.

It is associated with many other autoimmune conditions, including sjogrens, RA, systemic sclerosis, thyroid disease...

Primary biliary cirrhosis is often found incidentally by a raised alk phos on LFTs.Initially other LFTs may be normal but as the disease progresses bilirubin rises and PT may increase.

Associated signs (in late disease) include:
  • jaundice
  • xanthelasma
  • hepatosplenomegaly
  • clubbing

Complications include:
  • osteoporosis
  • portal hypertension
  • variceal haemorrhage

Diagnosis includes antibodies:
  • AMA M2 is highly specific and is positive in 98% of cases
  • SMA is positive in 30%
  • raised IgM

Treatment is:
  • cholestyramine for itching
  • osteoporosis prophylaxis
  • transplant

Once jaundice develops, without transplant the survival is < 2yrs.

On to AML...