Eosinophilic Granulomatosis with Polyangiitis (EGPA): A case report with atypical presentation

Pak J Med Sci. 2023 Jan-Feb;39(1):307-309. doi: 10.12669/pjms.39.1.6436.

Abstract

A 72-year-old asthmatic gentleman with a history of recurrent sinusitis and chronic bronchitis presented with shortness of breath and progressively worsening hypoxemic respiratory failure. His CT chest demonstrated airspace disease bilaterally with ground-glass opacifications. He had peripheral eosinophilia with raised inflammatory markers but negative work up of infection. On further investigation, ANA was positive, titer 1:160, speckled pattern and both pANCA and cANCA were present. The patient was diagnosed with Eosinophilic Granulomatosis with Polyangiitis (EGPA) and started on intravenous steroids and cyclophosphamide. A rare multi-organ vasculitis, EGPA is hallmarked by asthma, sinusitis and eosinophilia. In initial stages vasculitic involvement is not usually seen thereby making EGPA a diagnostic challenge.

Keywords: ANCA-associated Vasculitis; Eosinophilia; Eosinophilic Granulomatosis with Polyangiitis; Pulmonary Vasculitis.

Publication types

  • Case Reports