Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) both present with hypereosinophilia and organ damage induced by eosinophils. EGPA is also characterized by vasculitis and is associated with ANCA. Yet, discriminating HES from EGPA may be difficult in clinical practice as biomarkers to reliably differentiate between HES and EGPA are still lacking. In recent years, it has been demonstrated that targeting IL-5 is efficacious to treat HES and EGPA, and the use of anti-IL-5(R)-antibodies has become a mainstay of therapy in HES and these diseases. Nonetheless, it remains unclear whether anti-IL-5 strategies are sufficient to treat organ-threatening eosinophilic manifestations or vasculitic manifestations in EGPA.

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http://dx.doi.org/10.1093/rheumatology/keae608DOI Listing

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Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) both present with hypereosinophilia and organ damage induced by eosinophils. EGPA is also characterized by vasculitis and is associated with ANCA. Yet, discriminating HES from EGPA may be difficult in clinical practice as biomarkers to reliably differentiate between HES and EGPA are still lacking.

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This session presented emerging realworld evidence on novel glucocorticoid (GC) sparing therapies for ANCA-associated vasculitis (AAV). It covered the first-in-class oral C5a receptor antagonist avacopan for severe granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), and antiinterleukin-5 therapies in the management of eosinophilic granulomatosis with polyangiitis (EGPA). Avacopan was approved by the US Food and Drug Administration (FDA) and European Medicines Agency (EMA) for the treatment of AAV in 2021, following the phase 3 ADVOCAT E trial comparing oral avacopan to an oral GC taper for GPA and MPA.

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Objective: To conduct a systematic literature review and meta-analysis for the incidence, prevalence and mortality of eosinophilic granulomatosis with polyangiitis (EGPA) and describe the clinical burden of EGPA.

Methods: Searches were conducted in Ovid MEDLINE and Ovid Embase from January 2019 to June 2023 to identify relevant studies. Data were extracted, and the quality was assessed.

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We describe the case of a 73-year-old man who had been followed up by our clinic for pulmonary hypertension and asthma. He was later hospitalized and found to have significant and persistent eosinophilia compatible with hypereosinophilic syndrome. Various other conditions such as drug reaction with eosinophilia and systemic symptoms (DRESS), malignancy, and eosinophilic granulomatosis with polyangiitis (EGPA) were considered but largely excluded after further investigation.

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