Primary sclerosing cholangitis

Question 1

describe primary sclerosing cholangitis ?

Answer 1

chronic progressive inflammation of both intra and extra hepatic bile ducts

unman etiology = infection , exotoxins which causes billary epithelial cels to be damaged

leading to host response that persistence causing billary inflammation and sclerosis

the sclerosis can lead to cirrhosis , which can lead to cholangiocarcinoma and chornic cholestasis

Question 2

what Lethe risk factors ?

Answer 2

male

associated mostly with inflammatory bowel disease = pan colitis with ulcerative colitis

HLA - B8

Question 3

what are the clinical manifestation of primary sclerosing cholangitis ?

Answer 3

Multiple episodes of life-threatening acute cholangitis , giving fever
Right upper quadrant pain
Dark urine due to excess conjugated bilirubin
STEATORRHEA
PRURITIS
jaundice

cirrhosisi
= hepatomegaly, asciti , portal hypertension

Question 4

how do you diagnose primary sclerosing cholangitis ?

Answer 4

pANCA increased
ANA increased

serum alkaline phosphatase (ALP) > 1.5x the upper limit of normal for longer than 6 months - firstto appear
GGT ,
conjugated bilirubin high
serum aminotransferase lower than 200IU/L

AMA(antimitochondiral antibodies) is negative in primary sclerosing cholangitis

but positive in primary billary cirrhosis and primary billary cholangitis

MRCP
ERCP
= findings of multifocal BAND LIKE strictures with dilation of bead like appearance or divertcicular out pouching

Question 5

what is the differential diagnosis work out for primary billary cholangitis

Answer 5

primary billary cholnagitis
more common in WOMEN
only INTRAHEPATIC SMALL AND MEDIUM SIZED BLE DUCTS AFFECTED

clinical presentation similar

contains AMA positive
(alp , GGT and conjugated bilirubin are all high)

Question 6

differential diagnosis work out between IgG4 sclerosing cholangitis and primary sclerosing cholangitis ?

Answer 6

in IgG4 sclrosing cholangitis
stricture os the lower common bile duct
and dilation after confluent stricture

in

Question 7

what is the treatment for primary sclerosing cholangitis ?

Answer 7

UDCA
reduces cholestaisis but does not prevent disease progression
IV antibiotics to treat bacterial infections in ascending cholangitis= ciproflaxin

pruritus = cholestyramine

ERCP - dilate and stent strictures

PSC are often deficient in fat-soluble vitamins (vitamin A, vitamin D, vitamin E, and vitamin K)

liver transplant is the only curative option

Question 8

complications ?

Answer 8

develop IBD
cholangiocarcinoma
colrectal cabcer

fat soluble vit deficieny

Question 9

indication for liver transplant in psc?

Answer 9

Indications for transplantation include recurrent bacterial ascending cholangitis, decompensated cirrhosis, hepatocellular carcinoma, hilar cholangiocarcinoma, and complications of portal hypertension.