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

• DOI: 10.1002/ppul.71112
• Publication Date: 2025-05-09T12:25:25Z
• Authors: Honorata Marczak; Katarzyna Krenke; Katarzyna Solarsk...; Joanna Lange; Teresa Bielecka; Marek Kulus
• Keywords: Crackles; Tachypnea; Pulmonology; Lung biopsy

ABSTRACT Background Childhood interstitial lung diseases (chILD) are rare, chronic 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, outcomes of chILD at a Polish referral center. Methods We retrospectively reviewed data from patients (0−18 years) diagnosed with chILD, admitted to Department Pediatric Pulmonology Allergy, Medical University Warsaw, June 2009 February 2024, classified according chILD‐EU categorization system. Results A total 275 (65.5% male) were included, median age diagnosis 13 months (range: 1‐221). Persistent tachypnea infancy (PTI)/neuroendocrine cell hyperplasia (NEHI) was most common (52.4%), followed disorders related systemic (11.3%) exposures (10.2%). 13.8% remained undefined. The predominant included crackles (81.5%), dyspnea (72.7%) (68.3%). All children underwent computed tomography. Bronchoscopy, genetic testing, biopsy performed in 46.2%, 34.9%, 21.4% cases, respectively. Most (92.7%) received some form treatment, including inhaled bronchodilators/steroids (68.8%), steroids (26.5%), long‐term macrolides (16.3%), immunosuppressants (11.6%). Oxygen supplementation nutritional support required 50.5% 29.8% patients, At follow‐up 31.5 months, 92.9% achieved improvement or stabilization, 6.2% deteriorated, seven deaths. 5‐year survival rate 95.66%. Conclusion This study highlights significant diversity within PTI/NEHI being condition.