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. 2014 Feb 20:9:28.
doi: 10.1186/1750-1172-9-28.

Idiopathic eosinophilic pneumonia in children: the French experience

Lisa Giovannini-Chami  1 , Alice Hadchouel, Nadia Nathan, Francois Brémont, Jean-Christophe Dubus, Michael Fayon, Véronique Houdouin, Michèle Berlioz-Baudoin, Virginie Feret, Thierry Leblanc, Karine Morelle, Marc Albertini, Annick Clement, Jacques de Blic; French Respirare® Group
Affiliations

Idiopathic eosinophilic pneumonia in children: the French experience

Lisa Giovannini-Chami et al. Orphanet J Rare Dis. .

Abstract

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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Figures

Figure 1
Figure 1
HRCT scan in “dramatic improvement subset” ICEP patients. (a) At diagnosis: in case 1, bilateral and peripheral, dense and fluffy alveolar opacities associated with ground-glass opacities predominantly in the right superior lobe; in case 2, peripheral, alveolar, dense and fluffy opacities with areas of ground-glass opacities predominantly in the right and also in the left apex; in case 4, patchy ground-glass opacities and nodules. (b) Last HRCT scan showing complete normalization.
Figure 2
Figure 2
HRCT scan in “persistent diffuse interstitial subset” ICEP patients. (a) At diagnosis: in cases 3 and 5, diffuse ground glass opacities, reticulonodular syndrome. (b) Last HRCT scan showing diffuse ground-glass opacities with development of thin-walled cysts in cases 3 and 5.
Figure 3
Figure 3
Classification algorithm in pediatric eosinophilic pneumonias.
See this image and copyright information in PMC

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