Eosinophilic granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as the Churg-Strauss syndrome (CSS), refers to a small to medium vessel necrotizing pulmonary vasculitis. It is also classified under the spectrum of eosinophilic lung disease and as a type of pulmonary angiitis and granulomatosis.

The incidence typically peaks around the 3rd to 4th decade with an annual incidence rate of around 0.24 per 100,000 2.

Almost all patients have asthma and eosinophilia. Patients also have extrapulmonary signs and symptoms such as sinusitis, diarrhea, skin purpura, and/or arthralgias.

  • asthma: present in almost all patients 2
  • blood eosinophilia ( >10% of the total white blood cell count): present in almost all patients 2,13
  • mono/polyneuropathy
  • transient pulmonary infiltrates
  • paranasal sinus abnormalities: pain or radiographic abnormality
  • presence of extravascular eosinophils on a biopsy specimen

Can be histologically identical to classic polyarteritis nodosa or microscopic polyangiitis. Around 25% of patients will have renal disease 12.

Chest radiographs are nonspecific but may show:

Imaging features are nonspecific:

  • peripheral or random parenchymal opacification (consolidation or ground glass)
    • this tends to be the most frequent feature 1; can be transient
    • often bilateral and can sometimes be symmetrical 14
    • parenchymal opacification is predominantly peripheral or random in distribution 1
  • less common features include
  • regular and smooth interlobular septal thickening: may reflect the presence of edema secondary to cardiac involvement or eosinophilic septal infiltration 14
  • cavitation: rare and if present other co-existing pathology should be considered, e.g. granulomatosis with polyangiitis, infection 6

Delayed myocardial enhancement is a commonly described finding; can be intramyocardial, subepicardial or subendocardial 9-10.

Corticosteroids (most commonly prednisone) are the mainstay of treatment. Patients with cardiac, renal, gastrointestinal, or CNS involvement, require additional immunosuppression (e.g. cyclosporine, azathioprine).

The condition generally has a low mortality rate compared with other systemic vasculitides 11. Cardiac involvement may, however, be a significant contributor to disease-related death and may occur in up to 60% of cases 8,13.

It was first described in 1951 by Jacob Churg and Lotte Strauss, American physicians based in New York 4.

Article information

rID: 9724
System: Chest
Section: Syndromes
Synonyms or Alternate Spellings:
  • Churg-Strauss syndrome (CSS)
  • CSS
  • Churg Strauss syndrome
  • Allergic granulomatosis and angiitis
  • Churg-Strauss vasculitis
  • Churg-Strauss vasculitis (CSV)
  • Allergic angiitis and granulomatosis
  • Churg-Strauss syndrome
  • Eosinophilic granulomatosis with polyangiitis (EGPA)
  • EGPA

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Cases and figures

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