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 0.24-1.78 per 100,000 2,16.

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

The 1990 American College of Rheumatology criteria requires a positive biopsy for vasculitis and at least four of the six criteria listed below (sensitivity 85% and specificity 99.7%) 16

  • 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.

Biopsy of parenchymal opacities may show a mixture of necrotizing granulomas, eosinophilic pneumonia, and granulomatous vasculitis 16

Chest radiographs are nonspecific but may show:

  • peripheral consolidation, which may be transient/migratory
  • small pleural effusions - up to 50% of cases 16

Imaging features include:

  • 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
  • mediastinal lymphadenopathy is seen in less than 25% of cases 16

Cardiac involvement clinically manifests in about 66% of patients and can be found in up to 92% of autopsies 16, they include: 

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

ADVERTISEMENT: Supporters see fewer/no ads

Cases and figures

  • Case 1
    Drag here to reorder.
  • Case 2
    Drag here to reorder.
  • Case 3
    Drag here to reorder.
  • Updating… Please wait.

     Unable to process the form. Check for errors and try again.

     Thank you for updating your details.