Objective. To evaluate the value of a systematic approach to the diagn
osis of pediatric interstitial lung disease (ILD). Methods. In this de
scriptive, observational, prospective study, we evaluated 51 children
presenting with ILD of unknown etiology during a 3-year period. Specif
ic clinical information regarding history, physical examination, diagn
ostic evaluation, and final diagnosis was recorded on each patient. Re
sults. A specific diagnosis was established lay history and physical e
xamination alone in I patient, noninvasive tests alone in 8 others, an
d invasive tests, including lung biopsy, in another 26, Of the remaini
ng patients, 8 had a suggestive diagnosis, and 8 had no specific diagn
osis. Conclusions. A systematic approach to the diagnosis of pediatric
ILD is useful, and not all patients need lung biopsy for diagnosis.