Biliary Disease

09/05/2013 by admin | Gastroenterology
  1. Gallstone disease: ‘female, fat, fertile and forty!’
  2. Stones occur in 7% of men and 15% of women aged 18-65yo
  3. Prevalence is underestimated because 90% are asymptomatic
  4. Pathophysiology: gallstones form due to precipitation of cholesterol crystals in supersaturated bile. Increase in size by 2.5mm/y
  5. Clinical features:
    1. o Asymptomatic = GS is an incidental finding
    2. o Biliary colic = recurrent ‘colicky’ RUQ pain – precipitated by fatty foods
    3. o Cholecystitis = acute RUQ/right hypochondral pain and fever. If neck of GB gets obstructed = empyema of GB (=mortality 20-30%)
    1. o Cholestatic jaundice = jaundice with pale stools and dark urine = biliary obstruction (due to stone blocking common bile duct = ‘choledocolithiasis’), with evidence of infection on top of this, called = cholangitis
    2. o Ascending cholangitis = infection of bile duct (=cholangitis), caused by ascending infection from duodenum. Most commonly occurs when bile duct is already partially obstructed by a GS
    3. o Pancreatitis = GS migration along common bile duct – through ampulla of Vater.
    4. o Rare complications and presentations: biliary peritonitis (due to perforation), small bowel obstruction (‘gallstone ileus [blackage]’ = large GS being held up in ileocaecal valve), carcinoma of GB (90% of which are associated with GS), ‘porcelain gall bladder’ (=extensive calcification)
  1. Types of gall stone:
    1. o Cholesterol stones: vary in colour from light-yellow to dark-green or brown and are oval 2 to 3 cm in length, often having a tiny dark central spot. To be classified as such, they must be at least 80% cholesterol by weight.
    2. o Pigment stones: are small, dark stones made of bilirubin and calcium salts that are found in bile. They contain less than 20% of cholesterol
    3. o Mixed stones: typically contain 20–80% cholesterol. Other common constituents are calcium carbonate, palmitate phosphate, bilirubin, and other bile pigments. Because of their calcium content, they are often radiographically visible (~20% of all stones)
  2. Ix:
    1. o XR: ~ 20% (mixed stones) are visible
    2. o USS: most common Ix. Bile duct dilatation (intra- or extrahepatic) raises possibility of CBD stones, although transabdo USS can rarely confirm/exclude this
    3. o Liver transaminases: should be checked in any patient considered for cholecystectomy. If abnormal – an endoscopic retrograde cholangiopancreatography (ERCP) should be considered before surgery (may show stones in CBD)
    4. o ERCP: definitive imaging of biliary tree and provides opportunity to relieve biliary obstruction by endoscopic sphincterotomy and removal of CBD stones
    5. o Percutaneous transhepatic cholangiography: alternative approach to achieving direct visualisation of biliary tree. Transhepatic approach is potentially more traumatic to the patient and is reserved for when ERCP is not possible
    6. o Magnetic resonance cholangiopancreatography (MRCP): widely available alternative to ERCP
  3. Mx:
    1. o Watchful waiting: incidental finding requires no action
    2. o ERCP: both and Ix and Mx – used in patients with cholestatic jaundice in the presence of GS. In elderly or very unwell, ERCP may be sufficient to resolve problem.
    3. o Surgery: if patient is well enough to tolerate – definitive tx is cholecystectomy

 

Primary Biliary Cirrhosis (PBC):

  1. Rare, disease of mid-life women
  2. Pathology: non-suppurative granulomatous inflammation centred predominantly on the small interlobular bile ducts, resulting in progressive fibrosis and cirrhosis
  3. Possible cause = bacterial (E.coli) trigger
  4. p/c: with progressive chronic cholestasis, pruritis early on, may have vitamin deficiency, lethargy, fatigue, steatorrhoea, jaundice. May have established liver disease
  5. Asymptomatic individuals can be detected by abnormal LFTs (may also be mitochondrial antibody +ve), and may have associated a/i conditions: RA, thyroid, coeliac
  1. Ix:
    1. o LFTs: cholestatic picture = raised alkaline phosphatase and gamma-GT, with features of chronic liver disease in advanced stages.
    2. o Look for anti-mitochondrial antibody and M2 antibody detected at the E2 complex of the inner mitochondrial membrane. Will have raised IgM.
    3. o Liver imaging: USS/ERCP to exclude extra-hepatic biliary disease (GS, primary sclerosis cholangitis)
    4. o Liver biopsy: shows features of PBC and fibrosis
  2. Mx:
    1. o PBC is slowly progressive eventually resulting in cirrhosis
    2. o Tx for pruritis (=cholestyramine) and fatigues is usually disappointing
    3. o Ursodeoxycholic acid may help
    4. o Chronic cholestasis increases the risk of osteopenia
    5. o Liver transplant

 

Primary sclerosing cholangitis (PSC):

  1. Uncommon, complex, autoimmune, and strongly associated to inflammatory bowel disease (IBD)
  2. Pattern varies:
  3. MOST COMMON = inflammation may involve intralobular bile ducts (=intra-hepatic/small duct PSC)
  4. Also, extra-hepatic biliary strictures (=extra-hepatic/large duct PSC).
  5. Many patients have small and large duct disease
  6. P/c: abnormal LFTs, recurrent bacterial cholangitis (=RUQ pain, fever, jaundice), chronic cholestasis and chronic liver disease, IBD!
  7. Ix:
    1. o LFTs: mixed pattern of ‘hepatic’ and ‘cholestatic’ disease. Very high alkaline phosphatase
    2. o Tumour marker: CA-19-9 (may = cholangiocarcinoma)
    3. o Autoantibody: ANCA 80% +ve.
    4. o Liver USS: exclude focal liver lesions
    5. o ERCP: demonstrate multiple biliary strictures
    6. o Liver biopsy: ‘onion skin lesion’ around an obliterated bile duct
  8. Mx:
    1. o Bacterial cholangitis requires Abx
    2. o Pruritis requires ursodeoxycholic acid
    3. o Liver transplant used for those with rapidly progressive clinical course and decompensated liver disease
  9. Prognosis:
    1. o If symptomatic = die in 10-15 years
    2. o If asymptomatic = 75% alive at 15 years! 
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