Hepatology: primary sclerosing cholangitis: practice question

A General Medicine question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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General Medicine · Hepatology: primary sclerosing cholangitis

Sample item

A 34-year-old man with long-standing ulcerative colitis has pruritus and an alkaline phosphatase five times the upper limit of normal. MRCP shows multifocal beaded strictures of the intrahepatic and extrahepatic bile ducts. Which statement about his management is correct?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

A. Liver transplantation is the only treatment that alters survival in end-stage disease

Primary sclerosing cholangitis is a progressive fibrosing cholangiopathy strongly associated with ulcerative colitis, with a risk of cholangiocarcinoma. No drug has been shown to improve survival, and high-dose ursodeoxycholic acid is not recommended. Transplantation is the only life-prolonging therapy for end-stage disease, with dominant strictures treated endoscopically.

Option by option

  • A. Liver transplantation is the only treatment that alters survival in end-stage disease (correct)

    Correct. Transplant is the only proven survival-altering therapy.

  • B. Prednisolone with azathioprine induces lasting remission

    Immunosuppression is not effective in this disease, unlike autoimmune hepatitis.

  • C. Colectomy cures the biliary disease

    Colectomy does not stop the biliary disease, which progresses independently of colonic disease.

  • D. High-dose ursodeoxycholic acid improves survival

    High-dose ursodeoxycholic acid did not improve outcome and was associated with more serious adverse events.

Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.

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Questions about this General Medicine question

What is the answer to this General Medicine question?

The best answer is A, Liver transplantation is the only treatment that alters survival in end-stage disease. Primary sclerosing cholangitis is a progressive fibrosing cholangiopathy strongly associated with ulcerative colitis, with a risk of cholangiocarcinoma.

Why is "Prednisolone with azathioprine induces lasting remission" not the answer?

Immunosuppression is not effective in this disease, unlike autoimmune hepatitis.

Why is "Colectomy cures the biliary disease" not the answer?

Colectomy does not stop the biliary disease, which progresses independently of colonic disease.

Why is "High-dose ursodeoxycholic acid improves survival" not the answer?

High-dose ursodeoxycholic acid did not improve outcome and was associated with more serious adverse events.

What concept does this question test?

It tests Hepatology: primary sclerosing cholangitis in General Medicine. No drug has been shown to improve survival, and high-dose ursodeoxycholic acid is not recommended. Transplantation is the only life-prolonging therapy for end-stage disease, with dominant strictures treated endoscopically.

How hard is this question?

We tag it as an application question: it asks you to use a fact on a patient or a finding. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Hepatology: primary sclerosing cholangitis?

For each disease know the classic presentation, the best test, and the first-line treatment. Then use the flashcards and the other General Medicine questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.