Idiopathic eosinophilic pneumonia in children: the French experience

Abstract

BackgroundIdiopathic 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.MethodsWe retrospectively analyzed all cases of ICEP and IAEP in children that were retrieved from French Reference Centers for rare pediatric lung diseases.ResultsFive 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.ConclusionICEP 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.

Eosinophilic lung disease with plastic bronchitis and bronchiectasis in an adolescent male

“…CT picture depicts confluent pulmonary consolidation with air bronchogram. Studies focusing on ICEP in children have reported diffuse pulmonary alveolar consolidation with air bronchogram and/or ground‐glass opacity with peripheral predominance, BAL eosinophilia >20% or peripheral eosinophilia >1 × 10 9 cells/L, respiratory symptoms for duration >4 weeks in the absence of other known causes of eosinophilic lung disease and consistent open lung biopsy for cases without initial significant clinical and radiological improvement on first line of treatment . Pulmonary eosinophilia in our patient was found to be complicated by bronchiectasis with the presence of firm bronchial casts, an association of which has never been reported with any eosinophilic lung disease.…”

Diffuse Lung Disease

“…The diagnosis can also be made with consistent lung biopsy findings in cases without clinical and radiographic improvement after first-line corticosteroid treatment. Many cases of idiopathic CEP are initially misdiagnosed as asthma, and a subset of persistent cases develop reticulonodular interstitial opacities and cysts (Giovannini-Chami et al 2014).…”

Eosinophilic pneumonias in children: A review of the epidemiology, diagnosis, and treatment

“…Diagnosis currently relies on the demonstration of alveolar eosinophilia on bronchoalveolar lavage (BAL) (eosinophils >25%, this being the predominant cell population except for macrophages). This threshold has been defined in the adult setting but should probably be reduced to 20% in pediatrics . The threshold was lowered after the description of the first series of pediatric ICEP, in order not to exclude patients with a specific clinical history and histology .…”

“…This threshold has been defined in the adult setting but should probably be reduced to 20% in pediatrics . The threshold was lowered after the description of the first series of pediatric ICEP, in order not to exclude patients with a specific clinical history and histology . Thoracoscopic lung biopsy is necessary only in difficult settings, notably to assess the characteristics of the lung architecture after resistance to first line treatment in cases where distinguishing between idiopathic chronic eosinophilic pneumonia (ICEP) and idiopathic interstitial pneumonia (IIP) is not obvious …”

“…In the French internet‐linked database for pediatric interstitial lung disease (ILD) of the National Reference Center for Rare Lung Diseases (Respirare, http://www.respirare.fr), chronic EPs account only for five out of 217 ILD. Moreover, only three pediatric cases of acute idiopathic EPs have been described in France in the last decade . Despite their rarity, the etiological approach to EP has to be well‐defined, as some causes can be rapidly life‐threatening without initiation of the proper treatment.…”