Eosinophilic granulomatosis with polyangiitis (EGPA), as a heterogeneous component of antineutrophil cytoplasmic antibody-associated vasculitis, may be induced by a series of environmental and genetic factors, involved with a variety of immune cells and immune components, and presented with various clinical manifestations, with multiple organs and systems (respiratory, skin, heart, kidney, nerve, etc.) involved. The choice of glucocorticoid (GC) dosage and immunosuppressant in traditional treatment strategies varies greatly from individual to individual and is not universally applicable in all the EGPA phenotype spectrum, especially in relapsing or refractory diseases. With the understanding of the heterogeneity of EGPA, a variety of therapeutic approaches are emerging and improving the traditional treatment model. In this review, we summarized the heterogeneity of EGPA etiology and pathogenesis. Clinical and pathological manifestations of the same organ involved also show significant differences and there are even gender differences. Biological treatments that mainly target type 2 inflammatory pathways are widely used in clinical practice for remission induction and maintenance of EGPA. Targeted biological therapy has shown excellent performance in reducing GC dosage and controlling symptoms and recurrence. However, a large number of high-quality randomized controlled studies are still under research for relapsing or refractory EGPA with special organ involvement. We believe that EGPA has a highly heterogeneous phenotype spectrum, and the treatment patterns targeting key molecules in the pathogenesis are of great value for individual treatment of EGPA.
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http://dx.doi.org/10.1177/17534666251318615 | DOI Listing |
Rheumatology (Oxford)
March 2025
Nephrology and Dialysis Unit, Meyer Children's Hospital IRCCS, Florence, Italy.
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.
View Article and Find Full Text PDFRheumatology (Oxford)
March 2025
Vasculitis and Lupus Clinic, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
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.
View Article and Find Full Text PDFMod Rheumatol
March 2025
Health Economics and Payer Evidence, AstraZeneca, Cambridge, UK.
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.
BMJ Case Rep
March 2025
General Internal Medicine, Kobe City Medical Center General Hospital, Kobe, Hyogo, Japan.
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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