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Updated: Jun 14, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Factors influencing survival in children with chronic interstitial lung disease
1Pediatric Pulmonary Section, Baylor College of Medicine, Houston, Texas, USA.
Insights
In children with chronic interstitial lung disease (ILD), survival rates decrease over time. A higher severity-of-illness score is the only significant predictor of reduced survival in pediatric ILD patients.
Area of Science:
- Pediatric Pulmonology
- Critical Care Medicine
- Chronic Lung Diseases
Background:
- Chronic interstitial lung disease (ILD) in children presents significant survival challenges.
- Understanding prognostic factors is crucial for managing pediatric ILD.
Purpose of the Study:
- To identify clinical factors influencing survival in children diagnosed with ILD.
- To determine the predictive value of specific clinical indicators on long-term survival.
Main Methods:
- Retrospective analysis of clinical data from 99 pediatric ILD patients.
- Univariate and multivariate statistical analyses were employed to assess various factors.
- Key factors examined included weight percentile, respiratory signs (crackles, clubbing), family history, symptom duration, and severity-of-illness score.
Main Results:
- Overall survival probabilities at 24, 48, and 60 months were 83%, 72%, and 64%, respectively.
- Out of the investigated factors, only an increasing severity-of-illness score was significantly associated with decreased survival.
- Other factors like low weight, crackles, clubbing, family history, and symptom duration did not show a significant impact on survival.
Conclusions:
- Pediatric patients with ILD exhibit reduced survival probabilities.
- The severity-of-illness score is a significant and reliable predictor of survival outcomes in children with ILD.
Abstract:
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.
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