Lung
Noninfectious granulomatous inflammation
Eosinophilic granulomatosis with polyangiitis (EGPA)
Author: Elliot Weisenberg, M.D.
Last author update: 1 September 2011
Last staff update: 1 May 2022
Copyright: 2003-2024, PathologyOutlines.com, Inc.
PubMed search: allergic [title] granulomatosis angiitis pulmonary
Table of Contents
Definition / general | Treatment | Microscopic (histologic) description | Differential diagnosisCite this page: Weisenberg E. Eosinophilic granulomatosis with polyangiitis (EGPA). PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lungnontumorallergicgran.html. Accessed December 22nd, 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
Treatment
- 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
- Granulomatosis with polyangiitis (Wegener's): kidney involvement, no asthma, no tissue or serum eosinophilia
- Rheumatoid nodules: no tissue or serum eosinophilia, no asthma
