Rheumatology: adult-onset Still disease: 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: adult-onset Still disease

Sample item

A 28-year-old woman has had 3 weeks of daily fever spikes to 39.5 degrees Celsius that return to normal between episodes, with an evanescent salmon-pink rash on the trunk during the fever, sore throat and arthralgia. Neutrophil leucocytosis, markedly raised ferritin (12,000 ng/mL), negative RF and ANA, and negative blood cultures are noted. What is the most likely diagnosis?

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

The answer and the reasoning

A. Adult-onset Still disease

Adult-onset Still disease is a systemic autoinflammatory disorder presenting with quotidian or double-quotidian fevers, a transient salmon rash, arthritis, sore throat and neutrophilic leucocytosis, with very high ferritin and negative autoantibodies. It is a diagnosis of exclusion after infection, malignancy and other rheumatic disease are ruled out. Treatment is NSAIDs and corticosteroids, with methotrexate or IL-1 and IL-6 blockers for refractory disease.

Option by option

  • A. Adult-onset Still disease (correct)

    Correct. The fever pattern, evanescent rash and extreme hyperferritinaemia with negative serology fit this disease.

  • B. Systemic lupus erythematosus

    Lupus is ANA-positive and usually causes leucopenia rather than neutrophilia, with a much lower ferritin.

  • C. Acute rheumatic fever

    Rheumatic fever follows streptococcal pharyngitis in children and gives carditis with a migratory arthritis and erythema marginatum.

  • D. Infective endocarditis

    Endocarditis would show positive cultures, murmurs and embolic phenomena.

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.

More General Medicine questions

Questions about this General Medicine question

What is the answer to this General Medicine question?

The best answer is A, Adult-onset Still disease. Adult-onset Still disease is a systemic autoinflammatory disorder presenting with quotidian or double-quotidian fevers, a transient salmon rash, arthritis, sore throat and neutrophilic leucocytosis, with very high ferritin and negative autoantibodies.

Why is "Systemic lupus erythematosus" not the answer?

Lupus is ANA-positive and usually causes leucopenia rather than neutrophilia, with a much lower ferritin.

Why is "Acute rheumatic fever" not the answer?

Rheumatic fever follows streptococcal pharyngitis in children and gives carditis with a migratory arthritis and erythema marginatum.

Why is "Infective endocarditis" not the answer?

Endocarditis would show positive cultures, murmurs and embolic phenomena.

What concept does this question test?

It tests Rheumatology: adult-onset Still disease in General Medicine. It is a diagnosis of exclusion after infection, malignancy and other rheumatic disease are ruled out. Treatment is NSAIDs and corticosteroids, with methotrexate or IL-1 and IL-6 blockers for refractory disease.

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: adult-onset Still disease?

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.