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ANCA vasculitis GPA — SCE Rheumatology MCQ

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EasyVasculitisANCA vasculitis GPASCE Rheumatology

A 58-year-old man has chronic sinusitis, haemoptysis and renal impairment. Examination shows nasal crusting and pulmonary crackles. Investigations show PR3-ANCA positivity and urinary red-cell casts. What is the most likely diagnosis?

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Correct answer: B — Granulomatosis with polyangiitis

The diagnosis is granulomatosis with polyangiitis (GPA). The discriminating pattern is upper-respiratory disease with nasal crusting, pulmonary involvement causing haemoptysis, and glomerulonephritis indicated by renal impairment and red-cell casts. PR3-ANCA positivity provides particularly strong support for GPA. Microscopic polyangiitis can cause pulmonary–renal disease but usually lacks granulomatous upper-airway involvement and is more commonly associated with MPO-ANCA. Goodpasture disease can also cause pulmonary haemorrhage and glomerulonephritis, but does not explain the chronic destructive sinonasal features or PR3-ANCA. SLE nephritis and sarcoidosis do not fit the combined ANCA-associated pulmonary–renal and sinonasal presentation.

Reference: Robson JC et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis. Arthritis & Rheumatology. 2022;74:393–399. https://pubmed.ncbi.nlm.nih.gov/35106964/