Objective: To evaluate the value of a systematic approach to the diagnosis of pediatric interstitial lung disease (ILD).
Methods: In this descriptive, observational, prospective study, we evaluated 51 children presenting with ILD of unknown etiology during a 3-year period. Specific clinical information regarding history, physical examination, diagnostic evaluation, and final diagnosis was recorded on each patient.
Results: A specific diagnosis was established by history and physical examination alone in 1 patient, noninvasive tests alone in 8 others, and invasive tests, including lung biopsy, in another 26. Of the remaining patients, 8 had a suggestive diagnosis, and 8 had no specific diagnosis.
Conclusions: A systematic approach to the diagnosis of pediatric ILD is useful, and not all patients need lung biopsy for diagnosis.