Childhood Interstitial Lung Diseases: Lessons Learned From 15-Year Observation at a Polish Referral Center.

Abstract

Background: Childhood interstitial lung diseases (chILD) are rare, chronic lung diseases characterized by symptoms such as tachypnea, dyspnea, hypoxemia, crackles, and diffuse parenchymal abnormalities on chest imaging.

Objective: To evaluate the etiologic spectrum, clinical presentation, management, and outcomes of chILD at a Polish referral center.

Methods: We retrospectively reviewed data from patients (0-18 years) diagnosed with chILD, admitted to the Department of Pediatric Pulmonology and Allergy, Medical University of Warsaw, from June 2009 to February 2024, classified according to the chILD-EU categorization system.

Results: A total of 275 patients (65.5% male) were included, with a median age at diagnosis of 13 months (range: 1-221). Persistent tachypnea of infancy (PTI)/neuroendocrine cell hyperplasia of infancy (NEHI) was the most common diagnosis (52.4%), followed by disorders related to systemic diseases (11.3%) and related to exposures (10.2%). 13.8% of diseases remained undefined. The predominant symptoms included crackles (81.5%), dyspnea (72.7%) and tachypnea (68.3%). All children underwent chest computed tomography. Bronchoscopy, genetic testing, and lung biopsy were performed in 46.2%, 34.9%, and 21.4% of cases, respectively. Most children (92.7%) received some form of treatment, including inhaled bronchodilators/steroids (68.8%), systemic steroids (26.5%), long-term macrolides (16.3%), and immunosuppressants (11.6%). Oxygen supplementation and nutritional support were required in 50.5% and 29.8% of patients, respectively. At a median follow-up of 31.5 months, 92.9% of patients achieved clinical improvement or stabilization, and 6.2% deteriorated, including seven deaths. The 5-year survival rate was 95.66%.

Conclusion: This study highlights the significant diversity within chILD, with PTI/NEHI being the most common condition.