ALL THAT WHEEZES IS NOT ASTHMA

Abstract

TOPIC: Diffuse Lung Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Asthma is a common condition among young adults. However, wheezing, dyspnea and sinus disease are known to be manifestations of other conditions. We present a life-long non-smoker with frequent asthma exacerbation refractory to standard treatment. She was eventually found to have eosinophilic granulomatosis with polyangiitis (EGPA) without extra-pulmonary involvement at diagnosis. CASE PRESENTATION: The patient was diagnosed with asthma at age 15 by her PCP. Despite being prescribed fluticasone, she had 3 hospital visits within the first 16 months of diagnosis. Vilanterol, anti-histamines and montelukast were added subsequently in an attempt to control her disease, but she continued to have persistent symptoms manifesting as wheezing, dyspnea and sinus disease. She had an additional 5 episodes of exacerbation. Prednisone was effective, but she relapsed with cessation each time and gained 60 pounds from frequent use. She was eventually admitted for acute respiratory failure at age 18.Initial workup was significant for leukocytosis, peripheral eosinophilia and bilateral pulmonary infiltrates. Because of persistent symptoms, she underwent bronchoscopy with bronchoalveolar lavage, which demonstrated 69% eosinophils. Her serology was negative for rheumatologic (including anti-neutrophil cytoplasmic antibodies (ANCA)), infectious and hypersensitivity workup. Allergic bronchopulmonary aspergillosis was ruled out. The eosinophilia resolved with 1mg/kg of prednisone a day and she was discharged with a diagnosis of EGPA.Nine months later, she managed to wean herself off the steroids. She was started on benralizumab with improvement. Because of her child-bearing age, lack of extra-pulmonary symptoms and robust response to steroids, disease-modifying agents were not added. She then had 2 exacerbations that failed steroids in the span of 3 months. She began to develop sinusitis, diffuse polyarthralgia and stiffness, and was started on azathioprine after the initiation of dual birth control. DISCUSSION: There had been reports of benralizumab achieving rapid symptomatic and spirometric improvement in EGPA patients, with one managed to be free from exacerbation for 2 years.[1] Even though these reported cases had different serologic profile than our patient, it should not undermine their possible efficacy in reducing steroid dependence and the deleterious impacts associated with it.Young patients with ANCA-negative vasculitis may have vasculitic involvement without clinical manifestations. Cardiomyopathy is more common in this subset of patients and represents a major cause of death among these patients, emphasizing the importance of long-term follow-up in this population.[2,3] CONCLUSIONS: Refractory asthma can be a red herring and should prompt further investigation. For a young patient with seronegative EGPA, every attempt should be made to reduce steroid dependence and ensure close follow-up. REFERENCE #1: Miyata Y, Inoue H, Homma T, Tanaka A, Sagara H. Efficacy of Benralizumab and Clinical Course of Igg4 on Eosinophilic Granulomatosis with Polyangiitis. J Investig Allergol Clin Immunol. 2020;31(4). REFERENCE #2: Comarmond C, Pagnoux C, Khellaf M, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum. 2013;65(1):270-281. REFERENCE #3: Sokołowska B, Szczeklik W, Włudarczyk A, et al. ANCA-positive and ANCA-negative phenotypes of eosinophilic granulomatosis with polyangiitis (EGPA): outcome and long-term follow-up of 50 patients from a single Polish centre. DISCLOSURES: No relevant relationships by Vernon Chan, source=Web Response No relevant relationships by Omair Chaudhary, source=Web Response