Eosinophilic granulomatosis with polyangiitis (EGPA) – archaic eponym: Churg–Strauss syndrome (CSS) or allergic granulomatosis. The eponym ‘Churg-Strauss syndrome’ was replaced by ‘EGPA’ in part to achieve nomenclature symmetry with Microscopic Polyangiitis (MPA) and Granulomatosis with Polyangiitis (GPA, Wegener).
- EGPA is eosinophil-rich, necrotizing granulomatous inflammation often involving the respiratory tract; with necrotizing vasculitis predominantly affecting small to medium vessels; and associated with asthma and eosinophilia
- EGPA is a systemic small/medium vessel necrotizing vasculitis characterised by extravascular granulomas; eosinophilia; and eosinophilic tissue infiltration.
- EGPA most often occurs in people with adult-onset asthma; allergic rhinitis and nasal polyps and the diagnosis confirmed on tissue biopsy. ANCA is more frequent when glomerulonephritis is present
- Treatment is primarily with corticosteroids.
1951 – Churg and Strauss described 12 cases bearing a striking resemblance to GPA. However the cases demonstrated a marked eosinophilia and most of the patients were atopic they concluded that they were dealing with a new entity, which they entitled ‘allergic granulomatosis.‘
2012 – The Chapel Hill Consensus Conference (CHCC 2012) on the Nomenclature of Systemic Vasculitis, revised the classification of vasculitides and approved the transition name of ‘Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)‘ prior to full recognition of ‘Eosinophilic granulomatosis with polyangiitis (EGPA)‘ as the official terminology
- Eosinophilic granulomatosis with polyangiitis (EGPA)
- Allergic granulomatosis
- Allergic granulomatous angiitis
- Churg–Strauss syndrome
Chapel Hill Consensus Conference (CHCC 2012) provided a consensus for the use of descriptive wording to make nomenclature more consistent. ANCA-associated vasculitides include Microscopic Polyangiitis (MPA); Granulomatosis with Polyangiitis (GPA, Wegener); and Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss). The polyangiitis is similar in all three conditions
- Churg J, Strauss L. Allergic Granulomatosis, Allergic Angiitis, and Periarteritis Nodosa. Am J Pathol. 1951 Apr; 27(2):277–301. [PMC1937314]
- Strauss L, Churg J, Zak FG. Cutaneous lesions of allergic granulomatosis: a histopathologic study. J Invest Dermatol. 1951 Dec ;17(6):349-359. [PMID 14908172]
- Jennette JC, Falk RJ et al 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013 Jan;65(1):1-11. [PMID 23045170]