Primary Sclerosing Cholangitis Flashcards
Define primary sclerosing cholangitis
Chronic progressive cholestatic liver disease characterised by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts that results in diffuse multi-focal stricture formation
Aetiology and risk factors of primary sclerosing chlolangitis
Inflammation of medium and large-sized bile ducts → Fibrosis → multi-focal stricturing of ducts → stenosis → obstruction → Progressive fibrosis → Smaller ducts (= Ductopenia) and Bile stasis (= Cholestasis)
Associated with:
Autoimmune disease
Inflammatory bowel disease (80% PSC have UC)
HLA haplotypes
RF:
Male
Ulcerative colitis
FHx
Symptoms and signs of primary sclerosing cholangitis
Abdominal Pain (RUQ or epigastric)
Pruritus: generalised, intermittent
Jaundice
Fatigue
Weight loss (due to fat malabsorption)
Fever
Steatorrhoea
portal HTN → splenomegaly, aascites
Encephalopathy signs
Investigations for primary sclerosing cholangitis
Urine copper concentration: ?Wilson’s
LFTs: raised GGT/ALP (+ ALT/AST), raised bilirubin
Clotting: PT normal or prolonged
Albumin: normal or low
FBC
Autoimmune serology: P-ANCA positive (AMA, ANA, ASMA -)
If: IgG/IgM elevated
IgG4: normal/elevated
hepatic opper concentration: elevated
Caeruloplasmin: elevated
Abdo US: Abnormal bile duct ± cirrhotic liver, ascites, splenomegaly
MRCP: multi-focal strictures and dilatations (“Beaded” appearance)
ERCP: “Beaded” appearance
CT abdomen: bile duct thickening
Liver biopsy (rare): periductal fibrosis → onion skin appearance
Management for primary sclerosing cholangitis
Conservative
- Lifestyle changes such as alcohol avoidance, heat avoidance, emollients
- DEXA at diagnosis and 2-4 year intervals (osteoporosis)
- Ileocolonoscopy at diagnosis + repeat every 5 years
Medical
- Symptom management e.g. Pruritus → Antihistamines → cholestyramine
- Supplementation of fat soluble vitamins (A,D,E,K)
- Consider Ursodeoxycholic acid
Surgical
- Strictures may be dilated via ERCP
- Liver transplantation may be indicated in cases complicated by chronic liver disease and/or hepatobiliary malignancies.
Complications of primary sclerosing cholangitis
cholangiocarcinoma (in 10%)
increased risk of colorectal cancer