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Article Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis characterized by extravascular granulomas and eosinophilia in both blood and tissues. Eosinophils, which play a critical role in the pathophysiology of EGPA, require interleukin (IL)-5 for maturation in the bone marrow and migration to tissues. Glucocorticoids and immunosuppressants have been the cornerstone of treatment; however, their side effects have imposed a significant burden on many patients. Mepolizumab, an antibody that binds to and neutralizes IL-5, demonstrated efficacy in controlling disease activity in EGPA in the MIRRA trial conducted in 2017. In 2024, benralizumab, an IL-5 receptor alpha antagonist, was shown to be non-inferior to mepolizumab in efficacy against EGPA in the MANDARA trial. Both drugs were originally used for severe asthma and have benefited EGPA by reducing eosinophil counts. Due to differences in pharmacological structure and pharmacokinetics, the degree of eosinophil suppression varies between the two agents, and recent studies suggest that they may also affect inflammatory and homeostatic eosinophils differently. This review summarizes the latest insights into the pathophysiology of EGPA, highlights the similarities and differences between the two drugs, and discusses future treatment strategies for EGPA based on current clinical unmet needs, including drug selection.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC12025051 | PMC |
| http://dx.doi.org/10.3390/biom15040544 | DOI Listing |
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Systematic Literature Review of Real-world Outcomes of Benralizumab in Eosinophilic Granulomatosis with Polyangiitis.
J Allergy Clin Immunol Pract
September 2025
Division of Pulmonary, Critical Care, and Sleep Medicine, National Jewish Health, Denver, Colorado, USA. Electronic address:
Background: Several real-world cohorts and numerous case reports investigating benralizumab outcomes in eosinophilic granulomatosis with polyangiitis have been published. These studies complement the limited clinical trial data available by providing early insights on benralizumab use in a broader, real-world population.
Objective: The objective of this systematic literature review (SLR) was to provide an overview of the real-world outcomes of benralizumab in EGPA.
Benralizumab in a Patient With Refractory Eosinophilic Endocarditis.
J Med Cases
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Department of Hematology/Oncology, Mayo Clinic, Jacksonville, FL, USA.
Hypereosinophilic syndrome (HES) is a hematologic disorder characterized by an increased absolute eosinophil count (AEC) that can lead to tissue infiltration and damage. Idiopathic HES (iHES) comprises a subset of patients with HES, in which a reactive cause such as infections or an inflammatory process cannot be identified, and clonality is not demonstrable. iHES remains a challenge to treat since there is no specific mutation to target.
View Article and Find Full Text PDFThe woman with a swollen tongue : A rare case of an ANCA-negative localized eosinophil-rich granulomatous inflammation affecting solely the tongue.
HNO
September 2025
Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital and University of Zurich, Zurich, Switzerland.
Eosinophilic granulomatosis with polyangiitis (EGPA, previously known as Churg-Strauss syndrome) is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) often presenting with chronic rhinosinusitis, pulmonary infiltrates, gastrointestinal and cardiac disorders, or renal lesions. Sinonasal and inner ear manifestations are common, but other affections of the head and neck area are rarely reported. Here we report a case of a young woman with a histopathological diagnosis of eosinophil-rich granulomatous inflammation affecting solely the tongue without other local or systemic lesions.
View Article and Find Full Text PDFDiscontinuation of Oral Glucocorticoids and Achievement of Remission in Patients With Eosinophilic Granulomatosis With Polyangiitis Treated With Benralizumab or Mepolizumab.
ACR Open Rheumatol
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University of Pennsylvania, Philadelphia.
Objective: In the phase 3 head-to-head MANDARA study (NCT04157348), benralizumab demonstrated noninferiority to mepolizumab in inducing remission (defined as Birmingham Vasculitis Activity Score [BVAS] of 0 and oral glucocorticoid [OGC] dosage ≤4 mg/day at weeks 36 and 48) in patients with eosinophilic granulomatosis with polyangiitis (EGPA). This analysis investigated a more stringent definition of remission that included discontinuation of OGCs and being relapse-free.
Methods: Patients aged ≥18 years with documented relapsing or refractory EGPA receiving OGCs at ≥7.
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Reumatol Clin (Engl Ed)
August 2025
Grupo Peruano de Estudio de Enfermedades Autoinmunes Sistémicas, Universidad Científica del Sur, Lima, Peru; Servicio de Reumatología, Hospital Nacional Guillermo Almenara Irigoyen, EsSalud, Lima, Peru. Electronic address:
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