Drug reaction with eosinophilia and systemic symptoms (DReSS) may be difficult to
recognize in the setting of an inflammatory primary illness such as sJIA. Parenchymal
lung disease (LD) is not a known feature of sJIA. We have collected data on 63 children
with sJIA and LD. 48/63 entered cases developed an unusual, high-fatality LD in association
with interleukin-1 or interleukin-6 inhibition. Extensive rash and eosinophilia often
were noted prior to the LD diagnosis. Drug hypersensitivity merits consideration.
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