Pediatrics: Fellow Case Report Poster- Pediatrics |

Acute Eosinophilic Pneumonia in a Previously Healthy Child With Undiagnosed Chronic Granulomatous Disease (CGD) FREE TO VIEW

Erica Stevens, MD; Scott Bickel, MD; Gerald Lee, MD; Ronald Morton, MD; Adrian O'Hagan, MD
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Department of Pediatrics, University of Louisville, Louisville, KY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):955A. doi:10.1016/j.chest.2016.08.1058
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SESSION TITLE: Fellow Case Report Poster- Pediatrics

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION:Aspergillus rarely causes disease in immunocompetent patients. We report a case of eosinophilic pneumonia in a previously healthy child with pulmonary aspergillosis.

CASE PRESENTATION: A 6-year-old previously healthy male presented with fever, cough and hypoxia. Initial Chest X-ray demonstrated diffuse pulmonary opacities and chest CT showed bilateral air space and interstitial opacities without evidence of bronchiectasis, air trapping or cavity. Laboratory assessment revealed mild eosinophilia (2090/uL). Bronchoscopy revealed BAL eosinophilia (42%). Patient was diagnosed with acute idiopathic eosinophilic pneumonia and placed on systemic steroids.1 Within 24 hours he had resolution of symptoms, hypoxia and peripheral eosinophilia. At 2-week follow-up, fungal IgG was elevated for Histoplasmosis (1:32) and Aspergillus (1:16). Chest x-ray was unchanged. Given return of fevers, fatigue and intermittent tachypnea, he received pulse IV steroids without clinical improvement. Repeat Histoplasmosis titers increased to 1:128 and he was placed on IV Liposomal Amphotericin B for presumed Pulmonary Histoplasmosis. Repeat bronchoscopy with BAL revealed resolution of eosinophilia and both BAL galactomannan (2.01) and Aspergillus fumigatus PCR were positive. The patient was transitioned to IV Voriconazole for broader antifungal coverage. After 1 week of therapy without improvement, a lung biopsy was obtained. Silver staining was consistent with pulmonary aspergillosis and Aspergillus fumigatus was subsequently cultured. Immune evaluation revealed a Dihydrorhodamine-123 Chronic Granulomatous Disease (CGD) assay with a mean fluorescence index of 11, consistent with autosomal recessive CGD. Genetic testing was positive for the NCF1 gene, confirming the diagnosis.

DISCUSSION: Pulmonary aspergillosis is characterized by invasion of the airway or lungs by Aspergillus species, typically in immunocompromised patients. It is known that CGD patients are susceptible to aspergillosis.2 However, it is unusual that patients initially demonstrate eosinophilia and response to steroids. In this case, lung biopsy was necessary to make the diagnosis of Aspergillus, which in turn led to the diagnosis of CGD.

CONCLUSIONS: CGD is a primary immunodeficiency syndrome of the innate immune system. CGD patients are susceptible to Aspergillus infections.2 Recognition of pulmonary aspergillosis in a previously healthy patient that initially presents with peripheral and BAL eosinophilia is difficult, as this presentation is more consistent with acute idiopathic eosinophilic pneumonia. Tissue biopsy early in the course of illness may help to facilitate making the correct diagnosis.3 Immunologic work-up should be obtained in patients with aspergillosis.

Reference #1: Akuthota, P and Weller PF. Eosinophlic pneumonias. Clin Microbiol Rev. 2012;25(4):649-660.

Reference #2: Holland, SM. Chronic Granulomatous Disease. Clin Rev in Allergy & Immunol. 2010;38:3-10.

Reference #3: Segal, BH. Aspergillosis. N Engl J Med. 2009;360(18):1870-1884.

DISCLOSURE: The following authors have nothing to disclose: Erica Stevens, Scott Bickel, Gerald Lee, Ronald Morton, Adrian O'Hagan

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