Eosinophilic granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as the Churg-Strauss syndrome (CSS), is a small to medium vessel necrotising 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, diarrhoea, skin purpura, and/or arthralgias.

Diagnostic criteria
  • asthma: present in almost all patients 2
  • blood eosinophilia ( >10% of the total white blood cell count): present in almost all patients 2
  • mono/polyneuropathy
  • non-fixed pulmonary infiltrates
  • 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.

Markers
Plain radiograph

Chest radiographs are nonspecific but may show:

  • peripheral consolidation, which may be transient/migratory
  • small pleural effusions
CT

Imaging features are nonspecific:

MRI
Cardiac MRI

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

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 8.

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

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Article Information

rID: 9724
System: Chest
Sections: Pathology, Syndromes
Tag: cases
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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