Rheumatology: scleroderma renal crisis: 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.

Try it

General Medicine · Rheumatology: scleroderma renal crisis

Sample item

A 45-year-old woman with diffuse systemic sclerosis has been on prednisolone 30 mg daily. She develops headache, blood pressure 210/120 mmHg, rising creatinine and microangiopathic haemolytic anaemia with raised plasma renin. What is the treatment of choice?

AACE inhibitor, such as captopril, titrated upwardsBCyclophosphamide pulse therapyCIncrease the dose of prednisoloneDPlasma exchange as first-line therapy

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

The answer and the reasoning

A. ACE inhibitor, such as captopril, titrated upwards

Scleroderma renal crisis is a hypertensive emergency driven by renin from a kidney with obliterative vascular disease. Risk factors include diffuse disease, anti-RNA polymerase III antibodies and high-dose glucocorticoids. ACE inhibitors, rapidly titrated, are the cornerstone and dramatically improved survival.

Option by option

A. ACE inhibitor, such as captopril, titrated upwards (correct)
Correct. ACE inhibition counters renin-driven vasoconstriction and improves outcome.
B. Cyclophosphamide pulse therapy
Cyclophosphamide treats interstitial lung disease but does not treat renal crisis.
C. Increase the dose of prednisolone
High-dose steroids are a known precipitant, so increasing them would worsen the crisis.
D. Plasma exchange as first-line therapy
Plasma exchange benefits TTP, and it is not first-line here.

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.

Questions about this General Medicine question

What is the answer to this General Medicine question?

The best answer is A, ACE inhibitor, such as captopril, titrated upwards. Scleroderma renal crisis is a hypertensive emergency driven by renin from a kidney with obliterative vascular disease.

Why is "Cyclophosphamide pulse therapy" not the answer?

Cyclophosphamide treats interstitial lung disease but does not treat renal crisis.

Why is "Increase the dose of prednisolone" not the answer?

High-dose steroids are a known precipitant, so increasing them would worsen the crisis.

Why is "Plasma exchange as first-line therapy" not the answer?

Plasma exchange benefits TTP, and it is not first-line here.

What concept does this question test?

It tests Rheumatology: scleroderma renal crisis in General Medicine. Risk factors include diffuse disease, anti-RNA polymerase III antibodies and high-dose glucocorticoids. ACE inhibitors, rapidly titrated, are the cornerstone and dramatically improved survival.

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 Rheumatology: scleroderma renal crisis?

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.