Abstract
<h3>Introduction</h3> Idiopathic anaphylaxis (IA) is an acute mast-cell mediated reaction without an identifiable antigen. Over 80% of patients are reported to achieve remission with standard therapy consisting of an H1-antagonist and prednisone. Symptoms refractory to standard therapy are dubbed malignant IA. We present a case of malignant IA complicated by severe asthma refractory to high dose antihistamine, prednisone, and Omalizumab. <h3>Case Description</h3> A 44-year-old man with a history of severe asthma presented with recurrent anaphylaxis with unclear trigger while on antihistamine therapy and chronic prednisone. He was admitted and treated for a presumed asthma exacerbation. He required high doses of epinephrine during anaphylactic episodes and continued to have anaphylactic episodes despite high-dose antihistamine therapy and prednisone. A work-up for hereditary angioedema, serum IgE levels for appropriate allergens, mast cell disease, pheochromocytoma, and carcinoid syndrome was negative. Further investigations for systemic mastocytosis is pending results of N-Methylhistamine and 2,3-Dinor-11Beta-Prostaglandin F2 Alpha urine collection. Addition of Omalizumab led to decreased severity of episodes, but the episodes of anaphylaxis continue. Furthermore, the patient's asthma is not controlled despite optimal therapy with Symbicort and Spiriva Respimat. Due to his poor asthma control he may benefit from Dupilumab as a second biologic. We suspect the IL4-13 blockade will help control his asthma and IA. <h3>Discussion</h3> Our case adds to sparse literature on malignant IA complicated by severe asthma. Off label trials of two biologics may be warranted when dealing with treatment failure in such settings.
Published Version
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