Eosinophilic granulomatosis with polyangiitis (EGPA): clinical features

Pathology

• EGPA is a small to medium size vessel ANCA vasculitis
• It causes eosinophilic necrotizing granulomatous inflammation
• It may be related to HLA-DRB1*04, HLA-DRB1*07, HLA-DRB4 and IL10.2 haplotypes

Possible triggers

Clinical phases

Allergic features

Systemic features

Cardiovascular features

Neurological features

Dermatological features

Gastrointestinal features

Renal and metabolic features

Respiratory features

Ophthalmic features

Other features

Differential diagnosis

Synonym

References

1. Gioffredi A, Maritati F, Oliva E, Buzio C. Eosinophilic granulomatosis with polyangiitis: an overview. Front Immunol 2014; 5:549.
2. Roger A, Groh M, Lorillon G, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) induced by immune checkpoint inhibitors. Ann Rheum Dis 2019; 78:e82.
3. Cottin V, Bel E, Bottero P, et al. Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Eur Respir J 2016; 48:1429-1441.
4. Uematsu H, Takata S, Sueishi K, Inoue H. Polyangiitis overlap syndrome of granulomatosis with polyangiitis (Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). BMJ Case Rep 2014; 2014:bcr2013010195.
5. Cottin V, Bel E, Bottero P, et al. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Autoimmun Rev 2017; 16:1-9.
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Related checklists

• Eosinophilic granulomatosis with polyangiitis (EGPA): management