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ANCA Vasculitis — SCE Rheumatology MCQ

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HardVasculitisANCA VasculitisSCE Rheumatology

A 58-year-old man with granulomatosis with polyangiitis, previously involving the lungs and kidneys, has been in remission on azathioprine. Over 3 months he develops progressive nasal crusting, intermittent epistaxis and a new saddle-nose deformity. CRP and ESR remain normal, PR3-ANCA is negative, urinalysis and renal function are unchanged, and chest imaging shows no active disease. Nasal CT demonstrates diffuse septal cartilage erosion without a discrete mass, and bacterial and fungal cultures are negative. He denies intranasal cocaine or vasoconstrictor use. What is the most likely explanation?

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Correct answer: E — Localised granulomatous GPA activity despite controlled systemic disease

The progressive crusting, epistaxis, septal cartilage destruction and saddle-nose deformity indicate active, localised granulomatous ENT disease from GPA. This phenotype can relapse without renal or pulmonary activity and may remain ANCA-negative with normal systemic inflammatory markers; these tests must not be used alone to exclude activity. Granulomatous ENT manifestations may also be incompletely controlled by conventional maintenance therapy and require coordinated vasculitis and ENT reassessment. Cocaine-related destruction is undermined by the exposure history. Nasal NK/T-cell lymphoma would usually produce a discrete destructive mass and requires histological confirmation. Allergic fungal rhinosinusitis does not characteristically cause this pattern of septal cartilage collapse. Option D is incorrect because the presentation is localised granulomatous GPA activity, not evidence of a systemic necrotising vasculitic relapse; rituximab should not be inferred solely from serology or this phenotype.

Reference: Puéchal X et al. Localized versus systemic granulomatosis with polyangiitis: data from the French Vasculitis Study Group Registry. Rheumatology. 2022. https://pubmed.ncbi.nlm.nih.gov/34542599/