Abstract
SESSION TITLE: Fellows Diffuse Lung Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Chronic eosinophilic pneumonia (CEP) is characterized by a clinical syndrome which includes pulmonary symptoms, eosinophilia greater than 25% in bronchoalveolar lavage, and characteristic radiographic abnormalities. Corticosteroids are the mainstay of therapy. There is no standard alternative for steroid-refractory/dependent CEP, however, reports suggest anti-IL5 therapies may be an option. We present the case of a non-asthmatic, active duty service member with steroid dependent CEP who had a dramatic clinical response to mepolizumab. CASE PRESENTATION: A 47-year-old African American female presented to us for persistent productive cough and dyspnea despite multiple rounds of steroids and antibiotics for “recurrent upper respiratory infections”. Our evaluation was notable for thoracic lymphadenopathy and diffuse ground glass opacities on CT, a positive environmental allergy screen, IgE of > 4,000 IU/mL and an absolute serum eosinophil count of over 3,400. Pulmonary function testing, to include bronchoprovocation testing was normal. A diagnosis of allergic bronchopulmonary aspergillosis (ABPA) was excluded due to negative precipitins panel and lack of classic radiographic findings. Bronchoscopic evaluation revealed tissue eosinophilia with eosinophilic granuloma formation and no evidence of vasculitis. BAL from two separate lobes revealed a cell differential > 25% eosinophils with all infectious testing negative. An ANCA panel was positive for PR3, however, subsequent work up to include numerous biopsies of various sites, was not consistent with Eosinophilic Granulomatosis with Polyangitis (EGPA), especially in this non-asthmatic. We started steroid therapy for CEP which resulted in clinical improvement, however, our patient could not be weaned below 20mg daily without resulting in symptom relapse. For her steroid-dependent CEP we trialed mepolizumab, resolving her symptoms and radiographic findings, allowing cessation of all steroids DISCUSSION: IL-5 antagnosts, like mepolizumab, bind and neutralize IL-5 thereby inhibiting eosinophil production, activation and chemotaxis (1). They are used in eosinophilic asthma to relieve airway inflammation. In CEP, eosinophils also drive chronic inflammation. Steroids are classically considered the cornerstone of therapy for these patients and can generally be weaned, however, in this case the patient had steroid dependent disease necessitating consideration for alternative therapies. In the literature, anti-IL-5 therapy is non-asthmatics is discussed, however, to the best of our knowledge, there is only one published case documenting successful use of mepolizumab in CEP (2). CONCLUSIONS: Mepolizumab appears to be a promising therapy for patients with CEP and may be considered in patients who do not respond to, or are dependent on, steroids. More research must be done into such non-steroidal agents to reduce exposure to steroids and their side effects. Reference #1: 1. Garrett JK, Jameson SC, Thomson B, et al. Anti-interleukin-5 (mepolizumab) therapy for hypereosinophilic syndromes. J Allergy Clin Immunol. 2004;113(1):115-9. Reference #2: 2 To M, Kono Y, Yamawaki S, et al. A case of chronic eosinophilic pneumonia successfully treated with mepolizumab. J Allergy Clin Immunol Pract. 2018;6(5):1746-1748.e1. DISCLOSURES: No relevant relationships by Cyrus Askin, source=Web Response No relevant relationships by Tyson Sjulin, source=Web Response
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