Respiratory medicine: lymphangioleiomyomatosis: 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 · Respiratory medicine: lymphangioleiomyomatosis

Sample item

A 32-year-old woman presents with her second spontaneous pneumothorax, progressive exertional breathlessness and a milky pleural effusion. CT shows numerous thin-walled cysts evenly distributed through both lungs, and a renal angiomyolipoma. Which drug slows the decline in lung function?

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

The answer and the reasoning

D. Sirolimus

Lymphangioleiomyomatosis involves smooth muscle proliferation driven by mTOR activation, linked to tuberous sclerosis complex mutations. Sirolimus, an mTOR inhibitor, stabilises lung function. Chylous effusions and renal angiomyolipomas are typical associations.

Option by option

  • A. Pirfenidone

    Pirfenidone is an antifibrotic used in idiopathic pulmonary fibrosis.

  • B. Methotrexate

    Methotrexate has no proven role in this disease.

  • C. Cyclophosphamide

    Cyclophosphamide is not effective in this cystic lung disease.

  • D. Sirolimus (correct)

    Correct. Sirolimus inhibits mTOR, the pathway that is overactive in this disease.

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 D, Sirolimus. Lymphangioleiomyomatosis involves smooth muscle proliferation driven by mTOR activation, linked to tuberous sclerosis complex mutations.

Why is "Pirfenidone" not the answer?

Pirfenidone is an antifibrotic used in idiopathic pulmonary fibrosis.

Why is "Methotrexate" not the answer?

Methotrexate has no proven role in this disease.

Why is "Cyclophosphamide" not the answer?

Cyclophosphamide is not effective in this cystic lung disease.

What concept does this question test?

It tests Respiratory medicine: lymphangioleiomyomatosis in General Medicine. Sirolimus, an mTOR inhibitor, stabilises lung function. Chylous effusions and renal angiomyolipomas are typical associations.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. 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 Respiratory medicine: lymphangioleiomyomatosis?

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.