Noninfectious granulomatous inflammation

Eosinophilic granulomatosis with polyangiitis (EGPA)

Last author update: 1 September 2011
Last staff update: 1 May 2022

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PubMed search: allergic [title] granulomatosis angiitis pulmonary

Elliot Weisenberg, M.D.
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Cite this page: Weisenberg E. Eosinophilic granulomatosis with polyangiitis (EGPA). website. Accessed June 16th, 2024.
Definition / general
  • Also called Churg-Strauss syndrome
  • Very rare
  • Systemic vasculitis resembling polyarteritis nodosa or microscopic polyangiitis; associated with asthma, peripheral eosinophilia, pulmonary involvement and fever
  • Rarely presents without pulmonary disease as fever of unknown origin
  • < 50% have positive ANCA, often antimyeloperoxidase
  • Steroids: effective, but patients may relapse
Microscopic (histologic) description
  • Lung and extrapulmonary sites (skin, heart, nervous system, GI) have prominent eosinophilic infiltrate, granulomatous reaction around necrotic foci with radially arranged histiocytes and pallisading giant cells near small arteries or arterioles, eosinophilic vasculitis
  • May have fibrin rich edema, lymphocytes, sarcoid-like granulomas, focal fibrosis and eosinophilic microabscesses
Differential diagnosis
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