Acute exacerbations in children's interstitial lung disease

Area of Science:

• Pediatric Pulmonology
• Rare Diseases
• Interstitial Lung Disease

Background:

• Acute exacerbations (AEs) are known to increase morbidity and mortality in chronic pulmonary diseases.
• The specific characteristics and impact of AEs on children's interstitial lung disease (chILD) remain poorly understood.

Purpose of the Study:

• To characterize the features of AEs in pediatric patients with chILD.
• To evaluate the impact of AEs on the clinical course and quality of life in chILD patients.

Main Methods:

• Utilized data from the Kids Lung Register, collecting information on AEs, clinical course, and patient-reported outcomes (PROs).
• Analyzed data from 719 patients with chILD, encompassing 2822 AEs and 2887 register visits.

Main Results:

• The most common AE symptoms included increased dyspnea (74.1%), respiratory rate (58.6%), and oxygen demand (57.4%), with infections suspected in 94.4%.
• AEs were associated with significant declines in predicted FEV1, FVC, chILD-specific quality of life, and physical health scores compared to periods without AEs.
• Mortality was associated with AEs in 60.5% of the 81 patient deaths during the study period.

Conclusions:

• This study provides the first comprehensive analysis of AEs in chILD, highlighting their significant negative impact.
• AEs have a detrimental effect on the clinical trajectory and health-related quality of life for children with interstitial lung disease.