Churg–Strauss syndrome

Description

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.

History

1951Churg 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


Associated Persons

Alternative names
  • Eosinophilic granulomatosis with polyangiitis (EGPA)
  • Allergic granulomatosis
  • Allergic granulomatous angiitis
  • Churg–Strauss syndrome

Controversies

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


References

eponymictionary CTA

eponymictionary

the names behind the name

Doctor in Australia. Keen interest in internal medicine, medical education, and medical history.

British doctor (BMBS) working in Emergency Medicine in Perth, Australia. I am currently figuring out what to specialise in and making the most of what WA has to offer.

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