M282 HYPEREOSINOPHILIC SYNDROME VS. EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: THE SYMPTOMATIC DILEMMA

Autor: A. Khoylyan, R. Chepuru, R. Tachdjian, K. Jew, S. Brown, H. Danelyan
Rok vydání: 2020
Předmět:
Zdroj: Annals of Allergy, Asthma & Immunology
ISSN: 1081-1206
DOI: 10.1016/j.anai.2020.08.310
Popis: Introduction: HyperEosinophilic Syndrome (HES) and Eosinophilic Granulomatosis with Polyangiitis (EGPA) manifest similar symptoms in a patient HES is a class of rare blood disorders that can occur when a patient has elevated eosinophils EGPA is a three staged disease that is characterized by extravascular necrotizing granulomas During workup, these two diseases may appear as the same condition with similar manifestations Case Description: A 61-year-old female with new onset and worsening asthma over 2 years that included increased exacerbations and dyspnea on exertion despite increasing to triple maintenance therapy She was admitted for sepsis due to fever, fatigue, dyspnea, and worsening sinus congestion but was COVID-19 negative Upon admissions, the patient’s Sed Rate was 112 ANCA panel was negative, lung imaging was clear, cardiac workup showed eosinophilic myocarditis, nerve conduction velocity for lower extremities neuropathy showed mononeuritis complex multiplex, with no cutaneous manifestations Stool culture was negative Peripheral eosinophil count was 7640 μL and responded well to corticosteroids, Immunoglobulin E level was 2220 IU/mL, with normal tryptase and bone marrow study with normal JAK2 and PDGFR analysis Discussion: This patient meets case definitions for both HES and EGPA Factors favoring HES include corticosteroids responsiveness, no cutaneous manifestation, negative ANCA antibodies Factors favoring EGPA include asthma, sinus disease, normal bone marrow Myocarditis, neuropathy and other symptoms favor both Therefore, the challenge of determining the appropriate therapy made our team decide on anti-IL-5 therapy which has been shown to help both conditions
Databáze: OpenAIRE