Patient Journey, Treatment Patterns, and Disease Burden of Patients With Idiopathic Hypereosinophilic Syndrome
Juliana Schwaab, Paul Dolin, Bo Ding, Priya Jain, Lotte Westerink, Chris Edmonds, Tia Pennant, Oliver‐Thomas Carter, Fritha Hennessy, Stephanie Yanjing ChenABSTRACT
Introduction
Idiopathic hypereosinophilic syndrome (I‐HES) is a rare disorder characterized by persistent eosinophilia without an identifiable underlying cause, leading to organ damage and dysfunction. The objectives of this study were to describe the real‐world demographics, patient journey, treatment patterns, and disease burden of patients with I‐HES.
Methods
Data were drawn from the Adelphi Real World HES Disease Specific Programme, a cross‐sectional survey of physicians and their patients with I‐HES in Europe (France, Germany, Italy, Spain, and the UK) and the United States from July to December 2023.
Results
The overall population included 117 physicians and 451 patients. Patients were predominately male (62%), White (87%), and the mean (standard deviation [SD]) age was 44.7 (16.1) years. Mean (SD) and median (range) time between symptom onset and I‐HES diagnosis was 8.2 (11.1) and 4.2 (0–87.3) months, respectively. Most patients (66%) were treated with corticosteroids, and the mean (SD) and median (range) doses of oral and/or parenteral corticosteroids were 19.1 (20.3) and 10.0 (1.0–100.0) mg/day, respectively. The use of interleukin‐5/receptor alpha targeted therapies was low (23%). Patients had a mean (SD) of 7.7 (6.4) symptoms at diagnosis; 58% had organ system damage attributed to I‐HES, and disease was perceived by physicians as moderate or severe in 72%. Patient‐reported health‐related quality of life, work productivity, and fatigue were negatively impacted in patients with deteriorating or moderate‐to‐severe disease.
Conclusion
These findings underscore the need for greater disease awareness to support timely diagnosis with use of targeted, corticosteroid‐sparing treatments to improve patient outcomes.
Trial Registration
The authors have confirmed clinical trial registration is not needed for this submission.