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General Medicine · Rheumatology: granulomatosis with polyangiitis
Sample itemA 40-year-old man has chronic sinusitis with bloody nasal discharge, a saddle-nose deformity, cavitating lung nodules and rapidly progressive glomerulonephritis. Which antibody is most likely to be positive?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Cytoplasmic ANCA directed against proteinase 3
Granulomatosis with polyangiitis causes necrotising granulomatous inflammation of the upper and lower respiratory tract with pauci-immune glomerulonephritis. C-ANCA against proteinase 3 is present in most generalised cases. Induction uses glucocorticoids with rituximab or cyclophosphamide.
Option by option
A. Perinuclear ANCA directed against myeloperoxidase
MPO-ANCA is more typical of microscopic polyangiitis and eosinophilic granulomatosis.
B. Cytoplasmic ANCA directed against proteinase 3 (correct)
Correct. PR3-ANCA with the c-ANCA pattern is most frequent in this disease.
C. Anti-glomerular basement membrane antibody
Anti-GBM disease causes pulmonary-renal syndrome but not nasal destruction or cavitating nodules.
D. Anti-centromere antibody
Anti-centromere antibodies are found in limited systemic sclerosis.
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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