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

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.