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The lungs are at risk for involvement in systemic inflammatory disorders because of their enormous vascular network, both systemic and pulmonary; extensive immunologic network; and vast structural framework. Systemic inflammatory diseases such as collagen vascular (or rheumatic) diseases,1,2  vasculitidies,3,4  and granulomatous diseases5  may all have respiratory system involvement. Because most of these systemic diseases occur relatively infrequently in children, much of what we know about the lung involvement is extrapolated from adult literature. With national and international registries for pediatric-specific diseases, there is a growing body of information regarding the pediatric experience.

Systemic...

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