OBJECTIVE. The aim of this study was to determine whether a CT pattern
that may represent early or subtle changes of pulmonary vasculitis in
children exists. MATERIALS AND METHODS. High-resolution CT scans of t
he chest for 107 children were retrospectively reviewed by two radiolo
gists who were unaware of the original study findings. Chest CT scans
(conventional) for another 54 children who had symptoms or a diagnosis
of vasculitis also were reviewed. RESULTS. We identified hazy or fluf
fy centrilobular, perivascular densities in 10 children, two of whom h
ad small airways disease and eight of whom had vasculitis (Wegener's g
ranulomatosis [n = 5], systemic lupus erythematosus [n = 1], scleroder
ma-polymyositis overlap syndrome [n = 1], and Churg-Strauss syndrome [
n = 1]). The latter eight children underwent 35 scans, 17 of which wer
e positive for these perivascular densities. All positive scans were a
ssociated with active disease of new onset (5/17) or with clinical exa
cerbation of preexisting systemic disease (12/17). The positive scans
also were associated with an elevated erythrocyte sedimentation rate (
13/17) and biopsy evidence of vasculitis from a variety of sites, incl
uding the lungs (n = 1), kidneys (n = 7), oropharynx (n = 5), skin (n
= 9), lymph nodes (n = 1), and myocardium (n = 2). The single lung bio
psy showed an angiocentric inflammatory-hemorrhagic process. Of the fi
ve patients who had positive scans, underwent therapy, and then had re
peat studies, four patients had scans revert to normal in association
with inactive disease. The remaining patient whose scan did not normal
ize failed to respond to treatment. CONCLUSION. The fluffy centrilobul
ar pattern likely represents subtle changes of pulmonary vasculitis. I
n the appropriate clinical setting, such a finding may obviate the nee
d for a lung biopsy.