Vasculitis: granulomatosis with polyangiitis: practice question

A Pathology 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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Pathology · Vasculitis: granulomatosis with polyangiitis

Sample item

A 40-year-old man has chronic sinusitis with nasal crusting, haemoptysis with cavitating lung nodules and rapidly progressive glomerulonephritis. Which pairing of serology and histology is most likely?

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

The answer and the reasoning

A. c-ANCA (anti-PR3) with necrotising granulomatous inflammation and pauci-immune crescentic glomerulonephritis

Granulomatosis with polyangiitis affects the upper airways, lungs and kidneys. The hallmark is necrotising granulomatous inflammation with vasculitis, and the kidney shows pauci-immune crescentic glomerulonephritis on immunofluorescence. c-ANCA directed against proteinase 3 is positive in most patients.

Option by option

  • A. c-ANCA (anti-PR3) with necrotising granulomatous inflammation and pauci-immune crescentic glomerulonephritis (correct)

    Correct. The triad of upper airway, lung and kidney disease with c-ANCA defines this vasculitis.

  • B. Anti-GBM antibody with linear IgG deposition along the basement membrane

    Linear anti-GBM deposition describes Goodpasture disease, which has no sinus disease or granulomas.

  • C. Cryoglobulins with immune complex deposition and hypocomplementaemia

    Cryoglobulinaemic vasculitis shows immune complex deposition with low complement, not pauci-immune injury.

  • D. p-ANCA (anti-MPO) with eosinophil-rich granulomas, asthma and peripheral eosinophilia

    p-ANCA with eosinophilia and asthma describes eosinophilic granulomatosis with polyangiitis.

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 Pathology question

What is the answer to this Pathology question?

The best answer is A, c-ANCA (anti-PR3) with necrotising granulomatous inflammation and pauci-immune crescentic glomerulonephritis. Granulomatosis with polyangiitis affects the upper airways, lungs and kidneys.

Why is "Anti-GBM antibody with linear IgG deposition along the basement membr…" not the answer?

Linear anti-GBM deposition describes Goodpasture disease, which has no sinus disease or granulomas.

Why is "Cryoglobulins with immune complex deposition and hypocomplementaemia" not the answer?

Cryoglobulinaemic vasculitis shows immune complex deposition with low complement, not pauci-immune injury.

Why is "p-ANCA (anti-MPO) with eosinophil-rich granulomas, asthma and periphe…" not the answer?

p-ANCA with eosinophilia and asthma describes eosinophilic granulomatosis with polyangiitis.

What concept does this question test?

It tests Vasculitis: granulomatosis with polyangiitis in Pathology. The hallmark is necrotising granulomatous inflammation with vasculitis, and the kidney shows pauci-immune crescentic glomerulonephritis on immunofluorescence. c-ANCA directed against proteinase 3 is positive in most patients.

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 Vasculitis: granulomatosis with polyangiitis?

Learn each disease as a bundle: cause, key histology, markers, and the clinical hallmark. Then use the flashcards and the other Pathology 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.