DYSPNEA, DYSPHAGIA, AND URTICARIA WITH ANGIOEDEMA AFTER CHILDBIRTH

Abstract

Introduction Our case highlights the challenge in differentiating between hypereosinophilic syndrome (HES) and eosinophilia exacerbating atopic disease. Case Description A 29-year-old woman presents with dyspnea, dysphagia, urticaria and angioedema after childbirth. Evaluation revealed an eosinophil count of 3800/mcL. Infectious, cardiac, and chest imaging studies were normal. Lung function demonstrated FEV1/FVC at 0.85. Bronchial challenge with methacholine was positive. Bone marrow was normocellular with eosinophilia without evidence of PDGFRA, CHIC2, and FIP1L1 rearrangement. Upper endoscopy demonstrated esophageal eosinophilia. She improved on prednisone 40 mg daily, mometasone-formoterol, and montelukast. Dysphagia improved after esophageal dilation. Eosinophil counts remained elevated at 1500/mcL, with difficulty tapering prednisone due to dyspnea. She started interferon-alpha and continued prednisone 20 mg daily for 6 years with significant weight gain. She presented to our center and started mycophenolate-mofetil, discontinued interferon-alpha, and tapered prednisone to 5 mg daily. She then started mepolizumab and stopped prednisone. Six months later she developed dyspnea and urticaria. She switched to omalizumab with improvement and is stable on omalizumab, mycophenolate-mofetil, mometasone-formoterol, and montelukast. Discussion Asthma endotyping prompts checking eosinophil counts to assess for potential benefit with anti-IL5 therapy. Recent data demonstrates mepolizumab improves glucocorticoid sensitive HES, yet our patient responded better to omalizumab. The different biologics can help delineate the differential pathophysiology behind a patient's clinical presentation. Given the emerging overlap in therapeutic management, our case emphasizes the importance to shift the paradigm of understanding eosinophilic inflammation as a spectrum of disease rather than isolated entities, in consideration of both biologic and immune modulating medications.