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Medicine

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Liver & GIT

Question
13 out of 13
 

All of the following statements about primarysclerosing cholangitis are true, Except: (AI 1992)



A Increased risk associated with smoking

B Associated with Ulcerative colitis

C OGT elevation occurs early

D Pruritus is a common presenting symptom

Ans. A Increased risk associated with smoking

Primary sclerosing cholangitis (PSC)

i. Is a fibrosing cholangitis of bile ducts leading to inflammatory strictures and obliteration of both intrahepatic and extrahepatic ducts with dilatation of preserved segments.

ii. It's a progressive disease eventually resulting in secondary biliary cirrhosis.

iii. Autoimmune disorder, common in males.

iv. Association is seen with HLA -DR3, -DQ2 and - DRw52A

v. Its seen to be associated with ulcerative colitis and occasionally Crohn's disease (Ulcerative colitis seen in approx 2/3rd of patients of PSC).

vi. PSC is a risk factor for Cholangiocarcinoma

vii. Cholangiocarcinoma can present at any time during the disease process and does not correlate with the extent of sclerosing cholangitis.

Clinical Feature

1. Mean age of presentation is 30 to 45 yrs.

2. M: F ratio - 2 : 1

3. Usual presentation is intermittent jaundice, fatigue, weight loss, pruritus and abdominal pain.

4. Asymptomatic patients may come to attention because of persistent elevation of serum alkaline phosphatase

- GGT is typically elevated

Asymptomatic elevation of GGT is the earliest finding

- ALP & bile acids are increased

5. Endoscopic retrograde cholangiography is the definitive investigation.

ERC shows multiple dilatation and strictures involving both intrahepatic and extrahepatic ducts.

6. MRCP is the best non-invasive investigation. It is less sensitive than ERCP for intrahepatic ducts.

7. Treatment

a. There is no known effective medical treatment. b. Liver transplantation is the only option.

Important Points: Two important conditions more common in nonsmokers !!!

a. Ulcerative colitis (UC)

b. Primary sclerosing cholangitis (PSC)

Liver & GIT Flashcard List

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