Diffuse Lung Disease |

Idiopathic Chronic Eosinophilic Pneumonia With Gastrointestinal Involvement FREE TO VIEW

Renee Dixon, MD; Rebecca Krochmal, MD; Kathryn Robinett, MD
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University of Maryland, Baltimore, MD

Chest. 2014;146(4_MeetingAbstracts):419A. doi:10.1378/chest.1993989
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SESSION TITLE: Miscellaneous Cases I

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Sunday, October 26, 2014 at 10:45 AM - 12:00 PM

INTRODUCTION: Idiopathic chronic eosinophilic pneumonia (ICEP) is a rare entity with an estimated incidence of 2.5%, distinct from idiopathic acute eosinophilic pneumonia (IAEP). These eosinophilic pneumonias are diagnoses of exclusion as infection, drugs, and radiation therapy can incite pulmonary eosinophilia. ICEP occurs most often in non-smoking females in the 5th decade of life, frequently with a prior history of asthma or atopy. Our case illustrates a young woman with ICEP with an uncommon extra-pulmonary manifestation.

CASE PRESENTATION: A 29-year-old female with a history of asthma, allergies, and several episodes of pneumonia over the past few years presented to clinic for evaluation of dyspnea, cough productive of yellow phlegm, and malaise. She was a non-smoker. Medications included montelukast, beclomethasone, and albuterol without any recent changes. She was afebrile, with normal vitals, and pulmonary exam was unrevealing. Chest imaging showed bilateral, peripheral, upper lobe ground glass opacities. CBC revealed a leukocytosis of 15 with 15% eosinophils. IgE level was elevated at 288. BAL showed numerous alveolar macrophages, a WBC count of 144 with 20% eosinophils, and a negative culture. Transbronchial biopsy showed focal interstitial inflammation with prominent eosinophils and no evidence of vasculitis. GI consultation was sought at symptom onset due to abdominal pain and hematochezia. Colonic biopsies demonstrated prominent lymphoid aggregates and intraepithelial eosinophils.

DISCUSSION: ICEP is a rare entity most often presenting as an indolent course in non-smokers and asthmatics. Contrastingly, patients with IAEP present with severe, rapid onset of symptoms often requiring mechanical ventilation. IAEP patients are typically smokers and not asthmatics. ICEP and IAEP show rapid clinical and radiologic improvement with corticosteroid treatment; however, patients with ICEP will often relapse when steroids are tapered. Relapses are uncommon in IAEP. Extra-pulmonary manifestations have been noted at diagnosis in 10% or less of patients with ICEP but are not a component of IAEP.

CONCLUSIONS: ICEP is an uncommon condition in asthmatics that rarely has extra-pulmonary manifestations as displayed here.

Reference #1: Marchand E, Reynaud-Gaubert M, Lauque D et al. Idiopathic Chronic Eosinophilic Pneumonia. Medicine 1998;77:299-312.

Reference #2: Cottin V, Cordier JF. Eosinophilic Lung Diseases. Immunol Allergy Clin North Am 2012;32:557-586.

DISCLOSURE: The following authors have nothing to disclose: Renee Dixon, Rebecca Krochmal, Kathryn Robinett

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