Primary Sclerosing Cholangitis Flashcards
What is primary sclerosing cholangitis?
Intrahepatic and extrahepatic ducts become fibrotic, stiff and hardened causing chronic bile obstruction
I.e. sclerosing= stiffening and hardening
cholangitis= inflammation of bile ducts
What condition is associated with the development of PSC?
Ulcerative colitis i.e. 70% of cases occurs in conjunctive with UC
How would someone with PSC present?
Jaundice Fatigue Pruritus-> scratch marks Chronic RUQ pain Fatigue Signs of liver cirrhosis (in late stages)-> ascites + signs of portal HTN Hepatomegaly More likely to be male
What are the LFTs likely to show in PSC?
Cholestatic picture
- ++ ALP= first to present
- Bilirubin= as strictures become more severe
- ALT/AST may become more derranged as PSC progresses and causes hepatitis and liver cirrhosis
How is PSC diagnosed?
(MRCP) Magnetic resonance cholangiopancreatography
-bile duct lesions or strictures present
How can PSC be managed?
ERCP dilate and stent ducts i.e. endoscope through sphincter of Oddi to enter ampulla of Vater to insert stent into bile ducts
UDCA-> decreased cholesterol
Colestyramine to sequestrate bile acids to help with pruritus
Monitor for complications of cirrhosis and oesophageal varices
Treat IBD-> aim for remission as IBD disease state drives PSC disease state
Liver transplant is CURATIVE
-need to assess early because patients can go downhill very quickly
What are the possible extra-hepatic manifestations of PSC and why do they occur?
Pyoderma gangrenosum
Erythema nodosum
Episcleritis
Due to being associated with active bowel disease eg IBD
What is the use of testing for autoantibodies in PSC?
Not helpful in diagnosis but can indicate if there is autoimmune element to PSC which might respond to immunosuppression
Eg:
- p-ANCA
- ANA
- aCL
Why might someone with PSC be prone to current bouts of cholangitis/acute bacterial cholangitis?
Strictures in bile ducts lead to stagnant bile which increases the risk of bile infection and the risk of cholangitis
What are the associated complications which can occur with PSC?
Acute bacterial cholangitis
Cholangiocarcinoma
Colorectal cancer
Cirrhosis + LF
Biliary strictures
Fat soluble vitamin D deficiencies