Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis of small-to-medium-sized vessels. Both eosinophilic infiltration and vasculitis are thought to contribute to multi-organ damage. Some biologics have been used to reduce the required dose of corticosteroids in EGPA, but no single agent can ensure a complete control of this disease. Here, we describe a patient with anti-neutrophil cytoplasmic antibodies-negative relapsing EGPA whose asthma control was improved by omalizumab, but she continued to develop flares of abdominal and cutaneous vasculitis symptoms. After switching to mepolizumab therapy, her blood hypereosinophilia and extra-pulmonary symptoms were significantly improved. Moreover, the dose of daily maintenance corticosteroid could be tapered off. The experience from our case suggests that biologics targeting interleukin-5 may be more effective than omalizumab in the management of extra-thoracic manifestations in EGPA.
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http://dx.doi.org/10.1002/rcr2.878 | 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 PDFInt Forum Allergy Rhinol
March 2025
Department of Otorhinolaryngology-Head and Neck Surgery and Audiology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
Background: Chronic rhinosinusitis with nasal polyps (CRSwNP) significantly impacts patients' quality of life (QoL). Standard treatments include nasal irrigations, nasal steroids, systemic corticosteroids, and functional endoscopic sinus surgery (FESS). Uncontrolled severe CRSwNP treated with monoclonal antibodies (biologic drugs) gain better disease control, although some residual symptoms may persist.
View Article and Find Full Text PDFWe 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.
View Article and Find Full Text PDFCroat Med J
February 2025
Marina Lampalo, Clinical Department for Lung Diseases Jordanovac, University Hospital Center Zagreb, Jordanovac 104, 10000 Zagreb, Croatia,
Unlabelled: Aim- To compare the effectiveness of different biologic medications for the treatment of severe asthma.
Methods: We retrospectively collected data on 74 patients treated with one of four different biologics (omalizumab, mepolizumab, reslizumab, or benralizumab) at the Jordanovac Clinic for Pulmonary Diseases, Zagreb, Croatia for at least two years. The patients were compared in terms of the number of exacerbations, dose of oral corticosteroids (OCS), asthma control test (ACT), forced expiratory volume in 1 second (FEV1), forced vital capacity, fraction of exhaled nitric oxide (FeNO), number of blood eosinophils, and total immunoglobulin E (IgE).
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