Biliary System Flashcards
define primary biliary cholangitis (PBC)
autoimmune disease associated with progressive destruction of the bile ducts, due to granulomatous inflammation
what is PBC associated with?
rheumatoid arthritis
Sjogren’s syndrome
presentation of PBC
- fatigue
- itch without rash
- xantheslama and xanthomas
diagnosis of PBC
anti-mitochondrial antibodies (AMA) LFTs (cholestatic) liver biopsy (non-caseating granulomas)
management of PBC
urseodeoxycholic acid
transplant (liver)
define primary sclerosing cholangitis (PSC)
autoimmune disease of chronic inflammation of the intra and extra-hepatic bile ducts which leads to bile duct loss
what is PSC associated with?
UC
increased risk of cholangiocarcinoma
presentation of PSC
asymptomatic
itch, rigors
diagnosis of PSC
MRCP/ERCP
LFTS (cholestatic)
management of PSC
- bile flow e.g. stent
- monitor for cholangiocarcinoma and colorectal carcinoma
- control of itch
define haemochromatosis
storage disease of excess iron in the liver
causes of haemochromatosis
primary= genetics secondary= overload from diet
presentation of haemochromatosis
MEALS myocardial: dilated cardiomyopathy and arrhythmias Endocrine: diabetes Arthritis Liver: cirrhosis and HCC Skin: grey
diagnosis of haemochromatosis
liver biopsy with Perls stain (blue)
management of haemochromatosis
phlebotomy: removal of blood stimulates the bone marrow to make new red blood cells using up iron in storage
define Wilson’s disease
this is an autosomal recessive disease that leads to abnormal copper metabolism
presentation of Wilson’s disease
kayser-fleischer rings at corneal limbus
Parkinson-like symptoms
diagnosis of Wilson’s
low serum caeruloplasmin
management of Wilson’s
chelating agents e.g. penicillamine