Eosinophilic granulomatosis with polyangiitis

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Eosinophilic granulomatosis with polyangiitis
Diagnosis in short

Eosinophilic granulomatosis with polyangiitis. H&E stain.

Synonyms Churg-Strauss syndrome, Churg-Strauss disease

LM small vessel vasculitis with abundant eosinophils and granulomas
LM DDx eosinophilic vasculitis associated with a connective tissue disease
Site blood vessels

Associated Dx asthma
Signs fever
Prevalence rare
Blood work ANCA +ve/-ve

Eosinophilic granulomatosis with polyangiitis, abbreviated EGPA,[1] is a type of vasculitis.

It was previously known as Churg-Strauss syndrome.[2]

It should not be confused with granulomatosis with polyangiitis, previously known as Wegener's granulomatosis.

General

Defining features - memory device GAFE:

  • Granulomata.
  • Asthma.
  • Fever.
  • Eosinophilia.

Other clinical features - may be present:[3]

  • ANCA.
  • Weight loss.
  • Myalgias.
  • Arthralgias
  • Glomerulonephritis.
  • Hematuria.

Notes:

Microscopic

Features:

DDx:

Images

See also

References

  1. Xu, XL.; Song, W.; Sui, X.; Song, L.; DU, QN.; Wang, X. (Oct 2016). "Radiological and Clinical Features of Eosinophilic Granulomatosis with Polyangiitis.". Zhongguo Yi Xue Ke Xue Yuan Xue Bao 38 (5): 617-620. doi:10.3881/j.issn.1000-503X.2016.05.023. PMID 27825424.
  2. Stone, JR.; Bruneval, P.; Angelini, A.; Bartoloni, G.; Basso, C.; Batoroeva, L.; Buja, LM.; Butany, J. et al. "Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases.". Cardiovasc Pathol 24 (5): 267-78. doi:10.1016/j.carpath.2015.05.001. PMID 26051917.
  3. Cottin, V.; Bel, E.; Bottero, P.; Dalhoff, K.; Humbert, M.; Lazor, R.; Sinico, RA.; Sivasothy, P. et al. (Sep 2016). "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. doi:10.1016/j.autrev.2016.09.018. PMID 27671089.
  4. URL: http://emedicine.medscape.com/article/334024-overview. Accessed on: 22 January 2011.
  5. Greco, A.; Rizzo, MI.; De Virgilio, A.; Gallo, A.; Fusconi, M.; Ruoppolo, G.; Altissimi, G.; De Vincentiis, M. (Apr 2015). "Churg-Strauss syndrome.". Autoimmun Rev 14 (4): 341-8. doi:10.1016/j.autrev.2014.12.004. PMID 25500434.
  6. Chen, KR.; Su, WP.; Pittelkow, MR.; Conn, DL.; George, T.; Leiferman, KM. (Aug 1996). "Eosinophilic vasculitis in connective tissue disease.". J Am Acad Dermatol 35 (2 Pt 1): 173-82. PMID 8708015.