Primary Sclerosing Cholangitis

#med #disease #y2023

Presentation :

More common in men than women, which is unique among the autoimmune liver diseases
Often asymptomatic with a cholestatic LFT pattern; though up to 40% have abdominal pain, pruritis, diarrhea, jaundice fatigue, and fevers
- Steatorrhea and deficiencies of fat-soluble vitamins can develop
- Symptomatic gallstones tend to develop in about 75% of patients
Extremely strong association with IBD (up to 85% of cases)
- Concomitant IBD may have a unique PSC-IBD phenotype, characterized by rectal sparing, mild pancolitis, and backwash ileitis. Carries a higher risk for colon cancer, necessitating colonoscopy with surveillance biopsies every year from the time of diagnosis

Autoimmune fibroinflammatory disease of the large bile ducts, though it can also affect the small bile ducts (small-duct PSC).

Diagnosed noninvasively w/ MRCP showing multiple strictures/dilations in the intrahepatic and extrahepatic bile ducts
All patients with PSC without known IBD should have a colonoscopy at the time of PSC diagnosis and every 1 to 2 years thereafter
Antimitochondrial antibody is characteristically negative, unlike in Primary Biliary Cholangitis
Yearly MRCP and measurement of CA19-9 are recommended for cholangiocarcinoma surveillance given a 15% lifetime risk.
Regular screening with ultrasonography should be considered for increased risk of gallbladder cancer.

There is no effective medical therapy
Often requires liver transplantation
- Transplantation should be considered for patients with decompensated cirrhosis, recurrent bacterial cholangitis, and hilar cholangiocarcinoma
ERCP is used to dilate strictures and remove stones

Highest mortality rate among the autoimmune liver diseases
Median transplant-free survival for patients with PSC is 12 years
- Transplant outcomes for patients with PSC are excellent, with 1-year survival rates of at least 90% and recurrence rates of approximately 20% at 5 years after liver transplantation
15% lifetime risk for cholangiocarcinoma. The incidence of cholangiocarcinoma is highest in the first 2 years after PSC diagnosis.
Also w/ increased risk for gallbladder cancer