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Distinct clinical phenotypes and outcomes in pediatric chronic eosinophilic pneumonia: a 12- year retrospective
Changchang Li1, Lili Zhu1, Gang Yu1
1Department of Children's Respiratory Disease, the Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, 109 West Xueyuan Road, Lucheng District, Wenzhou , 325027, People's Republic of China.
Insights
Pediatric chronic eosinophilic pneumonia (CEP) is linked to allergies and often presents with cough and wheezing. Younger children with more severe lung involvement and lower initial steroid doses may have prolonged disease courses.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Interstitial Lung Disease
Background:
- Chronic eosinophilic pneumonia (CEP) is a rare interstitial lung disease primarily characterized in adults, often linked to asthma or allergies.
- Diagnosis of CEP relies on identifying pulmonary eosinophilia, with systemic corticosteroid therapy as the established treatment.
- Limited data exists for pediatric CEP, leading to diagnostic and management challenges.
Purpose of the Study:
- To analyze demographic, clinical, laboratory, imaging, bronchoalveolar lavage fluid (BALF), and treatment data in children diagnosed with CEP.
- To compare characteristics and outcomes between children with 'Cured' and 'Protracted' CEP phenotypes.
- To identify factors associated with a protracted disease course in pediatric CEP.
Main Methods:
- Retrospective cohort study of pediatric patients with CEP between 2014 and 2025.
- Data collected included patient demographics, clinical symptoms, laboratory results, chest CT imaging, BALF analysis, and treatment details.
- Patients were categorized into 'Cured' and 'Protracted' groups based on symptom and imaging resolution over 3 months.
Main Results:
- Thirteen pediatric patients (median age 2.92 years, 61.5% male) with CEP were identified, 84.6% had allergic comorbidities.
- Common symptoms included productive cough (92.3%) and wheeze (61.5%); chest CT showed interlobular septal thickening and ground-glass opacities (92.3%).
- Marked eosinophilia (median 46%) was observed in BALF. 61.5% achieved cure, while 38.5% had a protracted course, associated with younger age, extensive radiographic involvement, and lower initial corticosteroid exposure.
Conclusions:
- Pediatric CEP is strongly associated with atopy and can present with early onset and male predominance.
- A protracted CEP phenotype in children may be linked to younger age, extensive radiographic findings, specific biomarker profiles (elevated platelets, lower acute-phase reactants), and less initial corticosteroid therapy.
- Early diagnosis and adequate initial corticosteroid treatment are critical for improving outcomes in pediatric chronic eosinophilic pneumonia.
Abstract:
Chronic eosinophilic pneumonia (CEP) is a rare interstitial lung disease. Although CEP has been partially characterized in adults, pediatric data remain scarce, which often leads to diagnostic delays and management uncertainty. This retrospective cohort study analyzed demographic, clinical, laboratory, imaging, bronchoalveolar lavage fluid (BALF), and treatment data from children with CEP between 2014 and 2025. Patients were stratified into 'Cured' (complete resolution) and 'Protracted' (persistent symptoms or imaging abnormalities > 3 months) groups for exploratory comparison. Thirteen patients (median age 2.92 years; 61.5% male) were included, with 84.6% having allergic comorbidities. The most common symptoms were productive cough (92.3%) and wheeze (61.5%). Chest CT displayed interlobular septal thickening and ground-glass opacities (each 92.3%). BALF revealed marked eosinophilia (median 46%). After a median follow-up of 35.4 months, 8 patients (61.5%) achieved cure, while 5 (38.5%) had a protracted course. Compared with the Cured group, the Protracted group tended to be younger at onset, displayed more extensive radiographic involvement (100% bilateral, 40% lobar consolidation), received a lower initial corticosteroid dose (0.42 vs. 0.82 mg/kg/day) for a shorter duration (7 vs. 34 days), and exhibited a distinct biomarker profile (lower neutrophil-to-lymphocyte ratio, CRP, and LDH but higher platelet count), along with lower BALF neutrophils (5.0 vs. 24.0%) and higher lymphocytes (13.0 vs. 7.0%).
Conclusion:
Pediatric CEP is strongly associated with atopy. The protracted phenotype may be associated with younger age, more extensive radiographic involvement, a platelet-predominant and low-acute-phase biomarker profile, lymphocyte-skewed BALF, and lower initial corticosteroid exposure. Early recognition and adequate initial corticosteroid therapy appear crucial for optimizing outcomes.
What Is Known:
• CEP is a rare interstitial lung disease that classically affects middle-aged women and is frequently associated with asthma or allergy. • Diagnosis relies on demonstrating pulmonary eosinophilia, and prolonged systemic corticosteroid therapy remains the standard treatment.
What Is New:
• This pediatric cohort reveals that CEP often presents with early onset and male predominance, with outcomes following either a 'Cured' or 'Protracted' course. • The Protracted phenotype may be associated with younger age, specific radiologic findings, lower initial corticosteroid dosing, and elevated platelet counts with lower acute-phase reactants.
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