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Author Spotlight: Investigating the Pathophysiology of Eosinophilic Esophagitis
Published on: May 10, 2024
Idiopathic eosinophilic pneumonia in children: the French experience
Lisa Giovannini-Chami1, Alice Hadchouel, Nadia Nathan
1Pediatric Pulmonology and Allergy Department, Hôpitaux pédiatriques de Nice CHU-Lenval, Nice F-06200, France. giovannini-chami.l@pediatrie-chulenval-nice.fr.
Insights
Idiopathic eosinophilic pneumonia is rare in children. This study details chronic (ICEP) and acute (IAEP) forms, proposing new definitions and highlighting treatment responses and disease progression in pediatric cases.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Respiratory Medicine
Background:
- Idiopathic eosinophilic pneumonia (IEP) is exceptionally rare in pediatric populations.
- This study presents the first series detailing idiopathic chronic eosinophilic pneumonia (ICEP) and idiopathic acute eosinophilic pneumonia (IAEP) specifically in children.
- Understanding these rare lung conditions is crucial for accurate diagnosis and management.
Purpose of the Study:
- To describe the clinical characteristics and outcomes of pediatric ICEP and IAEP.
- To propose a specific pediatric definition and classification algorithm for ICEP.
- To compare pediatric IAEP with adult cases and identify potential triggers.
Main Methods:
- Retrospective analysis of pediatric ICEP and IAEP cases.
- Data collected from French Reference Centers for rare pediatric lung diseases.
- Review of clinical presentations, treatment responses, and disease progression.
Main Results:
- Five pediatric ICEP cases identified; three showed significant improvement with corticosteroids or immunosuppressants.
- Two pediatric ICEP cases exhibited persistent interstitial patterns progressing to cystic lesions.
- Three adolescent IAEP cases reported, one requiring extracorporeal membrane oxygenation.
Conclusions:
- Pediatric ICEP presents polymorphically, with some cases progressing to cystic changes, blurring lines with interstitial lung diseases.
- A specific pediatric definition and classification for ICEP are proposed.
- Pediatric IAEP appears as an inflammatory lung response to acute toxic exposure, primarily tobacco, similar to adults; international collaboration is needed for further research.
Background:
Idiopathic eosinophilic pneumonia is extremely rare in children and adults. We present herein the first series describing the specificities of idiopathic chronic (ICEP) and acute (IAEP) eosinophilic pneumonia in children.
Methods:
We retrospectively analyzed all cases of ICEP and IAEP in children that were retrieved from French Reference Centers for rare pediatric lung diseases.
Results:
Five cases of pediatric ICEP were identified. Corticosteroid or immunosuppressive therapy dramatically improved the outcome in three cases. The remaining two cases had a persistent interstitial pattern with progressive development of cystic airspace lesions. Three cases of IAEP in adolescents were reported, with one requiring four days of extracorporeal membrane oxygenation.
Conclusion:
ICEP is a rare disease with a polymorphic clinical presentation in children. We identified patients with persistent interstitial patterns progressing to cystic airspace regions, for which the boundaries with idiopathic interstitial pneumonias are difficult to establish. We therefore propose a specific pediatric definition and classification algorithm. IAEP in children remains an inflammatory reaction of the lung to an acute toxic exposure, mainly tobacco, as in adults. International studies are required to comprehensively assess the various clinical forms of the disease as well as the appropriate therapeutic regimens.
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