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Primary sclerosing cholangitis (PSC) (Pathophysiology (Environmental…
Primary sclerosing cholangitis (PSC)
Definition
Autoimmune disease
against bile ducts
Pathophysiology
Environmental trigger plus
genetic susceptibility
Progressive cholestasis, duct inflammation,
fibrosis of intra/extra hepatic ducts,
forming strictures in the biliary tree
Associated with autoimmune disease (IBD, AIH)
and malignancy (CRC, cholangiocarcinoma, HCC)
Epidemiology
M>F
Clinical
presentation
Asymptomatic
Lethargy
Pruritus
Diagnosis
History
DH
Meds, allergies
FH
Autoimmune disease
PMH
Autoimmune disease (IBD, AIH)
SH
Living arrnagements, occupation,
alcohol, smoking
PC/HPC
Lethargy, pruritus, jaundice
Examination
Abdo exam
Jaundice, excoriations,
hepatomegaly
Investigations
Imaging
ERCP: DIAGNOSTIC; large vs small duct cholangitis
Liver biospy
Disease staging (fibrosis, necrosis)
Bloods
FBC, CRP, U+E,
LFTs (high ALP/y-GT; later high bilirubin)
Immunology (ANA, SMA, ANCA +ve)
Bedside
Obs (nil)
Management
Medical
Anti-pruritic
Indication: pruritus
E.g. colestyramine
Bile salt replacement
Indication: all patients
E.g. urseodeoxycholic acid
MOA: bile salt replacement
SE: weight gain
Surgery
Liver transplant
End stage disease
Can get recurrence
ERCP
Indication: strictures
MOA: dilation of ducts
Conservative
Information, advice, support
Lifestyle (smoking, alcohol, weight)
Monitoring (1y colonoscopy and USS)
Complications
Other autoimmune disease
AIH, IBD
Cancers
Cholangiocarcinoma, CRC, HCC