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GPA Clinical Phenotype — ESENeph MCQ

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EasyRenal VasculitisGPA Clinical PhenotypeESENeph

A 55-year-old man with PR3-ANCA vasculitis develops hearing loss, nasal crusting, and saddle-nose deformity in addition to his renal involvement. This phenotype is most consistent with which AAV subtype?

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Correct answer: E — Granulomatosis with polyangiitis (GPA, formerly Wegener's)

Upper airway involvement (nasal crusting, saddle-nose deformity from cartilage destruction, subglottic stenosis, sinusitis) combined with renal involvement and PR3-ANCA positivity is the classic phenotype of GPA. MPA typically lacks significant upper airway disease and is more commonly MPO-ANCA positive. EGPA has asthma and eosinophilia. PAN is ANCA-negative. The distinction matters for prognosis (GPA has higher relapse rates than MPA) and monitoring strategy.

Reference: KDIGO 2024 – ANCA-Associated Vasculitis Guideline