Eosinophilic granulomatosis with polyangiitis (EGPA): clinical features
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Eosinophilic granulomatosis with polyangiitis (EGPA): clinical features. EGPA is a small to medium size vessel ANCA vasculitis.
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
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Clinical phases
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Allergic features
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Systemic features
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Cardiovascular features
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Neurological features
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Dermatological features
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Gastrointestinal features
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Renal and metabolic features
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Respiratory features
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Ophthalmic features
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Other features
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Differential diagnosis
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Synonym
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References
- Gioffredi A, Maritati F, Oliva E, Buzio C. Eosinophilic granulomatosis with polyangiitis: an overview. Front Immunol 2014; 5:549.
- 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.
- Cottin V, Bel E, Bottero P, et al. Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Eur Respir J 2016; 48:1429-1441.
- 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.
- 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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