Churg-Strauss syndrome

Discussion:

Our case demonstrated more than four out of the six criteria of Churg Strauss syndrome including asthma, hypereosinophilia (>15%), paranasal sinusitis, pulmonary infiltrates, and peripheral polyneuropathy.

The neurological radiographic manifestations were in the form of hypertrophic pachymeningitis, which was suggestive of a granulomatous process. Also, small parenchymal patches of abnormal signal are seen at the midbrain, pons and middle cerebellar peduncles, likely vasculitic.

Paranasal sinus CT revealed chronic sinusitis and chest CT showed pulmonary infiltrates with patchy ground-glass attenuation and smooth interlobular thickening which are suggestive of eosinophilic lung disease.

Churg Strauss syndrome is a multisystemic disorder characterized by necrotizing vasculitis of the small vessels, associated with extravascular eosinophilic granulomas that occur exclusively in patients with asthma and peripheral eosinophilia.

At least four of the following six criteria should be present; asthma, eosinophilia greater than 10% in peripheral blood, paranasal sinusitis, migratory or transient pulmonary infiltrates, polyneuropathy, and biopsy-proven extra-vascular eosinophils.

Chest CT in patients with Churg Strauss syndrome usually show ground glass opacities in a lobular distribution without zonal predilection and are predominantly peripheral, resembling those in chronic eosinophilic pneumonia, and can be transient, resembling findings of simple pulmonary eosinophilia. Other manifestations include smooth interlobular septal thickening, diffuse interstitial reticular or reticulonodular, and bronchial wall thickening.

Meningeal involvement in Churg Strauss disease is not common and has been reported a few times 1,2.

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