Hypereosinophilic Syndrome (HES) & Eosinophilic Granulomatosis with Polyangiitis (EGPA): Causes, Symptoms & Treatment Guide Hypereosinophilic Syndrome (HES) and EGPA Speaker: Florence Roufosse, Professor of Medicine, Internist and Clinical Immunologist at CUB- Hôpital Erasme, Brussels. Past President of the International Eosinophil Society Explore the connections between Eosinophilic Gastrointestinal Diseases (EGIDs) and Hypereosinophilic Syndromes (HES). Help us to develop more resources like this Topics covered: Introducing the Eosinophil: nature and origins, behaviour and functions Hypereosinophilia and HES: blood and tissue hypereosinophilia HES clinical manifestations Pathogenesis of hypereosinophilia in HES: two major disease variants and their disease presentations Natural disease course in patients with HES HES diagnosis: common causes of increased eosinophil count, evidence of eosinophil-mediated organ damage and dysfunction and disease variants investigations EGPA clinical presentation: asthma, blood and tissue hypereosinophilia, vasculitis EGPA clinical manifestations: eosinophil-mediated and vasculitic complications Typical stepwise EGPA progression Overlap between EGPA and HES Diagnosis of EGPA: comorbid diseases, blood and pulmonary function tests, ENT check, imaging and other tests. Treatment of HES and EGPA: corticosteroid and other treatment options Targeting the eosinophil: critical role of Interleukin (IL)-5 in eosinophil biology and as therapeutic targets in eosinophil disorders Clinical trials for HES with agents targeting the IL-5 pathway - Mepoluzimab, Benralizumab, tapering corticosteroid doses and outcomes: reduction in blood eosinophil count, fatigue How eosinophil-targeted treatments fit in nowadays: tapering corticosteroids, eosinophil-depleting treatments Clinical trials for EGPA targeting IL-5: MIRRA trial (Mepoluzimab), MANDARA (Benralizumab) - achieving remission, fewer relapses, reduction in eosinophil counts, and glucocorticosteroids sparing Take-home messages Overview Hypereosinophilic Syndrome (HES) is a rare but serious condition characterised by elevated blood and tissue eosinophil levels. Understanding this disorder is essential for anyone exploring Eosinophilic Gastrointestinal Diseases (EGIDs) who also has persistently elevated eosinophils in their blood and/or organs beyond their gut. Early recognition and accurate diagnosis are critical to managing inflammation, preventing organ damage, and improving quality of life. What is Hypereosinophilic Syndrome (HES)? HES is a rare disorder characterised by persistently high blood eosinophil counts without an identifiable secondary cause. These elevated eosinophils can infiltrate multiple organs, including the heart, lungs, skin, and gastrointestinal tract, leading to organ damage if untreated. HES requires careful monitoring and often involves targeted therapies to reduce eosinophil levels and control symptoms. What is Eosinophilic Granulomatosis with Polyangiitis (EGPA) Eosinophilic Granulomatosis with Polyangiitis, or EGPA, (formerly Churg-Strauss Syndrome), is a rare but serious condition linked to elevated eosinophil levels in the blood and tissues. EGPA, formerly known as Churg-Strauss syndrome, is a rare autoimmune vasculitis associated with high eosinophil levels. It primarily affects small to medium-sized blood vessels, causing inflammation in the lungs, skin, nerves, and gastrointestinal tract. EGPA often develops in people with a history of asthma or allergies, and early treatment is key to preventing severe organ complications. Understanding this disorder is essential for anyone exploring Eosinophilic Gastrointestinal Diseases (EGIDs) who also has persistently elevated eosinophils in their blood, lungs, and other organs. Early recognition and accurate diagnosis are critical to managing inflammation, preventing organ damage, and improving quality of life. Further Resources What is Eosinophil? Understanding Clinical Trials Biologics for Eosinophilic Diseases: Latest News & Research This resource was developed with support from the National Lottery Community Fund. Manage Cookie Preferences