Primary Sclerosing Cholangitis

Moving on from gallstones- remember that post on ulcerative colitis? The lady I saw at the GP this week with ulcerative colitis also developed PSC as a complication, which led to a liver transplant. This is it.


Primary Sclerosing Cholangitis (PSC)

Definition: chronic cholestatic (impaired bile flow) liver disease characterised by progressive inflammatory fibrosis and obliteration of intrahepatic and extrahepatic bile ducts.

Aetiology/ risk factors:

-postulated immune and genetic predisposition

-genetic association : HLA-A1, HLA-B8, HLA-DR3

-associated with autoimmune hepatitis

-associated with inflammatory bowel disease, usually ulcerative colitis of the whole colon (> 80% of Northern Europeans also have IBD), which often presents before PSC

Epidemiology:

-prevalence 2-7 in 100,000

-more common in males

-usually presents between 25 and 40 years

Symptoms:

-may be asymptomatic

May present with:

-intermittent jaundice

-pruritus (itching)

-fatigue

-right upper quadrant pain

-weight loss

-episodes of fever and rigors caused by cholangitis are less common

-symptoms of ulcerative colitis (e.g. diarrhoea, abdominal pain)

-symptoms of complications

Signs:

May have no signs or:

-jaundice

-hepatosplenomegaly

-spider naevi, palmar erythema, ascites (due to liver cirrhosis)

Image result for spider naevi

Spider naevus

Image result for palmar erythema

Palmar erythema (red hands)

 

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Ascites

 

Investigations

Bloods-

LFTs- ↑Alkaline phosphatase, GGT, mild transaminases. Later stages- Bilirubin & ↓albumin

Serology-hypergammaglobulinaemia and or/↑IgM (IgG in children)

AMA -ve (anti-mitochondrial antibody)

SMA & ANA +ve (anti-smooth muscle & anti-nuclear antibody) positive in ∼30%

pANCA +ve in ∼70% (perinuclear anti-nuclear cytoplasmic antibody)

ERCP (Endoscopic retrograde cholangiopancreatography)- distinguish small duct from large duct disease

Shows stricturing and interspersed dilation, with a typical ‘beaded’ appearance, of the intrahepatic and occasionally, extrahepatic bile ducts. Small diverticula (outpouchings) on the common bile duct may be seen.

ERCP shows many strictures in the biliary tree with a typical ‘beaded’ appearance.

Image result for ercp psc

MRCP (magnetic resonance cholangiopancreatography)- non-invasive option for visualising the biliary tree, and will also show strictures.

Strictures can be hard to differentiate between cholangiocarcinoma.

Liver biopsy- shows a fibrous, obliterative cholangitis. Confirms disease and allows staging.

Management:

-no curative treatment with management focusing on symptom control

COLESTYRAMINE (4-8g/24h PO) for pruritus. (Naltrexone and rifampicin may also help).

-Optimise nutrition and replace deficiencies e.g. fat soluble vitamins, ensure adequate dietary calcium

Endoscopic or percutaneous transhepatic stenting or balloon dilation of major extrahepatic bile duct strictures to relieve biliary obstruction

⇒Liver transplant is the mainstay for end-stage disease

-Antibiotics (e.g. cefuroxime- a cephalosporin- and metronidazole) and resuscitation for bacterial cholangitis.

-screen for cancers with annual colonoscopy + ultrasound, and consider cholecystectomy for gallbladder polyps found on U/S (more likely to become malignant in PSC)

URSODEOXYCHOLIC ACID may protect against colon cancer and improve LFTs (high doses may be harmful)

Complications:

-recurrent acute cholangitis

-biliary cirrhosis

-liver cirrhosis, portal hypertension (encephalopathy, ascites, variceal bleeding)

-end-stage hepatic failure

-cancer: bile duct (cholangiocarcinoma), gallbladder, liver, colon

-metabolic bone disease

Prognosis: following a liver transplant, occurrence occurs in up to 30% and 5 year graft survival is > 60%. Prognosis is worse for those with IBD as 5-10% develop colorectal cancer post-transplant.

Median survival from presentation to liver transplant or death is about 10 years.

References: Cheese & Onion, Rapid Medicine
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