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Churg-Strauss syndrome

Dr Yuranga Weerakkody et al.

The Churg-Strauss syndrome (CSS) is a small to medium vessel necrotizing pulmonary vasculitis. It is also classified under the spectrum of eosinophilic lung disease 3.

Clinical presentation

Almost all patients have asthma and eosinophilia. Patients also have extrapulmonary signs and symptoms such as sinusitis, diarrhoea, skin purpura, arthraglias etc.

Diagnostic criteria
  • asthma
  • blood eosinophilia ( >10% of the total white blood cell count)
  • mono / polyneuropathy
  • non-fixed pulmonary infiltrates
  • sinus abnormalities (pain or radiographic abnormality)
  • presence of  extra vascular eosinophils on a biopsy specimen

Pathology

Can be histologically identical to classic polyarteritis nodosa or microscopic polyangiitis. 

Markers

Radiographic features

CT

Imaging features are non specific 

  • peripheral or random parenchymal opacification (consolidation or ground glass) 
    • this tends to be the most frequent feature 1 ; this can be transient
    • parenchymal opacification was predominantly peripheral or random in distribution 1
  • less common features include, centrilobular nodules and bronchial wall thickening and or dilatation 5
  • cavitation is rare and if present other co-existing pathology should be considered - e.g. Wegener's, infection 6

Etymology

First described by Jacob Churg and Lotte Strauss : Amercian physicians ; New York  in 1951 4

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