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Pediatrics |

Steroid-Refractory Chronic Eosinophilic Pneumonia in a Child FREE TO VIEW

Walid Hussain, MD; Wendy Estrellado, MD
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Children's Mercy Hospital, Kansas City, MO


Chest. 2014;146(4_MeetingAbstracts):692A. doi:10.1378/chest.1978774
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Abstract

SESSION TITLE: Pediatric Student/Resident Case Report Posters

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Chronic eosinophilic pneumonia (CEP) is a rare cause of lung disease in children. The nonspecific nature of the clinical presentation of this disease process makes diagnosis a unique challenge.

CASE PRESENTATION: A 3-year-old male with congenital AV block was admitted with acute hypoxic respiratory failure. He presented with two weeks of cough and dyspnea. Initial exam revealed tachypnea, subcostal retractions, and diffuse expiratory wheezing. Complete blood count showed peripheral eosinophilia of 8.8%. Chest radiograph showed diffuse bilateral perihilar opacities. CT angiogram revealed extensive airspace disease with areas of patchy ground-glass opacities (FIG 1). Bronchoalveolar lavage revealed 18% eosinophilia. He was diagnosed with eosinophilic lung disease. His symptoms improved following methylprednisolone but shortly after, his respiratory status deteriorated. Attempts to wean his oral steroids had been unsuccessful. A lung biopsy obtained four months later showed findings consistent with chronic eosinophilic lung disease (FIG 2). The patient remained tachypneic and hypoxic despite high dose steroids. The addition of Intravenous gamma globulin (IVIG) and ultimately cyclophosphamide led to stabilization of his respiratory symptoms.

DISCUSSION: CEP is a rare idiopathic disorder characterized by an abnormal accumulation of eosinophils in the lung.1 It affects every age group but is extremely rare in childhood. Diagnosis is usually based on the association of: respiratory symptoms of more than 2 weeks, alveolar and/or blood eosinophilia, pulmonary infiltrates on imaging, and exclusion of any known cause of eosinophilic lung disease.1,2 The pathophysiology of CEP is unclear; however eosinophilic-specific chemoattractants have been shown to be upregulated. CEP is classically highly responsive to corticosteroids. Respiratory symptoms, peripheral eosinophilia, and radiographic findings regress within a few days after starting corticosteroids.1 Although response to treatment is dramatic, relapses are observed in up to 50% of patients while tapering corticosteroids or after weaning off.2 Options for steroid-resistant CEP include the use of immunomodulatory agents including IVIG and cyclophosphamide. The beneficial response to cyclophosphamide may result from inhibition of T-cell stimulation of antigen-presenting cells, which are capable of stimulating tissue epithelial cells to release eosinophil chemoattractants.

CONCLUSIONS: CEP remains an uncommon diagnosis in children. Although steroids should be used as initial therapy, our experience suggests strong consideration should be given to the use of cyclophosphamide and IVIG in children experiencing steroid resistance or excessive side effects.

Reference #1: Cakir E, Ferda A, Cakir FB, Ertem T. Chronic Eosinophilic Pneumonia with Mucous Plugs in a Child. Pediatric Pulmonology 2010;45:1040-1042.

Reference #2: Marchand E, Cordier JF. Idiopathic chronic eosinophilic pneumonia. Orphanet Journal of Rare Diseases 2006;1;11:1-4.

DISCLOSURE: The following authors have nothing to disclose: Walid Hussain, Wendy Estrellado

No Product/Research Disclosure Information


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