Rheumatology: limited systemic sclerosis: 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 · Rheumatology: limited systemic sclerosis

Sample item

A 50-year-old woman has had Raynaud phenomenon for 15 years, skin thickening limited to the fingers and face, telangiectasia, calcinosis and oesophageal dysmotility. Which autoantibody is most characteristic, and which complication is she most at risk of?

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

The answer and the reasoning

B. Anti-centromere antibody; pulmonary arterial hypertension

Limited cutaneous systemic sclerosis, formerly called CREST, is associated with anti-centromere antibodies and late pulmonary arterial hypertension, so annual echocardiography and lung function screening are advised. Diffuse disease is associated with anti-Scl-70 and fibrosis, or anti-RNA polymerase III and renal crisis.

Option by option

  • A. Anti-RNA polymerase III antibody; renal crisis

    Anti-RNA polymerase III with renal crisis is linked to rapidly progressive diffuse skin disease.

  • B. Anti-centromere antibody; pulmonary arterial hypertension (correct)

    Correct. Anti-centromere antibody with a risk of pulmonary hypertension fits limited disease.

  • C. Anti-U1 RNP antibody; mixed connective tissue disease

    Anti-U1 RNP defines mixed connective tissue disease, which overlaps features of lupus, scleroderma and myositis.

  • D. Anti-topoisomerase I (Scl-70) antibody; pulmonary fibrosis

    Anti-Scl-70 with pulmonary fibrosis is the pattern of diffuse 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 B, Anti-centromere antibody; pulmonary arterial hypertension. Limited cutaneous systemic sclerosis, formerly called CREST, is associated with anti-centromere antibodies and late pulmonary arterial hypertension, so annual echocardiography and lung function screening are advised.

Why is "Anti-RNA polymerase III antibody; renal crisis" not the answer?

Anti-RNA polymerase III with renal crisis is linked to rapidly progressive diffuse skin disease.

Why is "Anti-U1 RNP antibody; mixed connective tissue disease" not the answer?

Anti-U1 RNP defines mixed connective tissue disease, which overlaps features of lupus, scleroderma and myositis.

Why is "Anti-topoisomerase I (Scl-70) antibody; pulmonary fibrosis" not the answer?

Anti-Scl-70 with pulmonary fibrosis is the pattern of diffuse disease.

What concept does this question test?

It tests Rheumatology: limited systemic sclerosis in General Medicine. Diffuse disease is associated with anti-Scl-70 and fibrosis, or anti-RNA polymerase III and renal crisis.

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 Rheumatology: limited systemic sclerosis?

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.