Factors influencing survival in children with chronic interstitial lung disease.

To investigate factors influencing survival in children with chronic interstitial lung disease (ILD), we extracted specific clinical information from a data base of 99 children with ILD who met entry criteria for our study. The effects of a weight below the fifth percentile for the patient's age, crackles, clubbing, family history of ILD, symptom duration, and severity-of-illness score on survival were examined with univariate and multivariate statistical analyses. There were 15 recorded deaths in the group, with a probability that a patient would survive to 24 mo, 48 mo, and 60 mo after onset of symptoms of 83%, 72%, and 64%, respectively. Of the clinical features, only increasing severity-of-illness score was significantly associated with a higher probability of decreased survival. Children with ILD have decreased survival probabilities that can be predicted with a severity-of-illness score.