Childhood interstitial lung disease survivors in adulthood: a European collaborative study

Area of Science:

• Pulmonology
• Pediatric Medicine
• Internal Medicine

Background:

• Childhood interstitial lung disease (chILD) is rare but increasingly diagnosed.
• Prognosis for chILD was historically poor, but survival into adulthood is improving.
• This study characterizes chILD survivors and their impact on adult care.

Purpose of the Study:

• To characterize childhood interstitial lung disease survivors.
• To identify the impact of chILD survivors on adult interstitial lung disease centers.
• To understand the transition of care from pediatric to adult settings.

Main Methods:

• A European study involving 34 adult and chILD centers.
• Collected epidemiological, clinical, physiological, and genetic data from incident/prevalent chILD survivors (Jan-July 2023).
• Analyzed data from 244 identified patients.

Main Results:

• 244 chILD survivors diagnosed at a median age of 12.5 years, with a median age of 25 at study inclusion.
• Significant lung function impairment noted: median % predicted FVC 70%, DLCO 48%.
• Most patients (93%) transitioned to adult centers, with common diagnoses including sarcoidosis, surfactant-related disorders, and unclassified chILD; 60% remained on corticosteroids.

Conclusions:

• chILD survivors are frequently managed in adult interstitial lung disease centers, with significant lung function loss.
• The heterogeneity of chILD aetiologies and treatments impacts adult centers.
• Re-specification of diagnosis and treatment is crucial for personalized management of chILD survivors.