• Clin Med (Lond) · Nov 2023

    Case Reports

    ANCA-negative ANCA-associated vasculitis: pitfalls of the 'vasculitis screen'.

    • Hannah Cooke, Matthew Wells, Keith Miller, MedfordAndrew R LARLNorth Bristol Trust, Bristol, UK., and Sam Patel.
    • Musgrove Park Hospital, Taunton, UK.
    • Clin Med (Lond). 2023 Nov 1; 23 (6): 630632630-632.

    AbstractDespite its recognition as an 'ANCA-associated vasculitis' (AAV), eosinophilic granulomatosis with polyangiitis (EGPA) is ANCA negative in up to 60% of cases. Herein, we report the case of a young man with a clinical syndrome highly suggestive of EGPA but with repeated negative ANCA serology, ultimately presenting with cardiac arrest before recognition of the primary systemic vasculitis, whereupon he received successful induction therapy with high dose glucocorticoids and cyclophosphamide. The case illustrates the importance of awareness of ANCA negative AAV among general physicians in order to minimise morbidity and mortality.© Royal College of Physicians 2023. All rights reserved.

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