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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

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Published on: June 16, 2020

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

Honorata Marczak¹, Katarzyna Krenke¹, Katarzyna Solarska-Rydz¹

¹Department of Pediatric Pneumonology and Allergy, Medical University of Warsaw, Warsaw, Poland.

Pediatric Pulmonology

|May 10, 2025

Insights

Childhood interstitial lung diseases (chILD) are diverse, with Persistent Tachypnea of Infancy/Neuroendocrine Cell Hyperplasia of Infancy (PTI/NEHI) being most common. Most children with chILD showed improvement or stabilization with treatment.

Area of Science:

Pediatric Pulmonology
Rare Diseases
Interstitial Lung Disease

Background:

Childhood interstitial lung diseases (chILD) are rare, chronic respiratory conditions.
Symptoms include rapid breathing, shortness of breath, low oxygen, and abnormal lung imaging.
These diseases present a diagnostic and management challenge in pediatric care.

Purpose of the Study:

To analyze the causes, clinical features, treatments, and outcomes of chILD.
To characterize the spectrum of chILD in a Polish pediatric referral center.
To provide insights into the management of these rare lung conditions.

Main Methods:

Retrospective review of 275 pediatric patients (0-18 years) diagnosed with chILD.
Data collected from June 2009 to February 2024 at the Medical University of Warsaw.
Classification based on the chILD-EU categorization system.

Main Results:

Persistent Tachypnea of Infancy/Neuroendocrine Cell Hyperplasia of Infancy (PTI/NEHI) was the most frequent diagnosis (52.4%).
Crackles, dyspnea, and tachypnea were the predominant symptoms.
Over 92% of patients improved or stabilized with treatment, and the 5-year survival rate was 95.66%.

Conclusions:

chILD encompasses a wide range of conditions, with PTI/NEHI being the most prevalent.
Effective management strategies lead to favorable outcomes in the majority of pediatric patients.
Further research is needed to understand and manage undefined chILD cases.

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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.

Keywords:

DPLD chILD childhood interstitial lung disease diffuse parenchymal lung disease

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