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Microbiology · Immunology: c-ANCA and proteinase 3
Sample itemA 42-year-old man has chronic sinusitis with nasal crusting, cough with haemoptysis and haematuria with red cell casts. Which autoantibody is most strongly associated with this picture?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. Antibody to proteinase 3, giving a cytoplasmic ANCA pattern
Upper airway disease, lung involvement and necrotising glomerulonephritis define granulomatosis with polyangiitis, which is typically associated with anti-proteinase 3 antibodies and a cytoplasmic (c-ANCA) immunofluorescence pattern. Anti-myeloperoxidase (p-ANCA) is more typical of microscopic polyangiitis. Anti-GBM disease gives lung and kidney involvement without granulomatous upper airway disease.
Option by option
A. Antibody to myeloperoxidase, giving a perinuclear pattern
Anti-myeloperoxidase gives a p-ANCA pattern and is more typical of microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis.
B. Antibody to double-stranded DNA
Anti-dsDNA is a marker of lupus, which does not typically cause this granulomatous upper airway disease.
C. Antibody to proteinase 3, giving a cytoplasmic ANCA pattern (correct)
Correct. Proteinase 3 antibodies give the c-ANCA pattern of granulomatosis with polyangiitis.
D. Antibody to glomerular basement membrane type IV collagen
Anti-GBM antibodies cause Goodpasture disease, which spares the upper airways.
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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