Publications by authors named "E GELAIN"
Article Synopsis
- This study evaluated the effectiveness and safety of the IL-5 receptor inhibitor benralizumab for treating patients with eosinophilic granulomatosis with polyangiitis (EGPA) across 28 European centers.
- Out of 121 patients treated, complete responses increased from 12.4% at 3 months to 46.4% at 12 months, while partial responses decreased over the same period.
- Notable improvements were seen in disease activity, as measured by the Birmingham Vasculitis Activity Score (BVAS), and a reduction in various disease manifestations, alongside better lung function.
Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is often associated with glucocorticoid-dependent asthma and/or ear, nose and throat (ENT) manifestations. When immunosuppressants and/or mepolizumab are ineffective, dupilumab could be an option. We describe the safety and efficacy of off-label use of dupilumab in relapsing and/or refractory EGPA.
View Article and Find Full Text PDFArticle Synopsis
- - The study investigates the genetic underpinnings of Erdheim-Chester disease (ECD), a rare condition characterized by abnormal immune cell activity, by conducting the first genome-wide association study to understand its inherited genetic factors.
- - Researchers analyzed data from 255 ECD patients and 7,471 healthy individuals, identifying a significant genetic region (18q12.3) that could increase susceptibility to ECD, linked to the SETBP1 gene.
- - The findings suggest that inherited genetic variants play a role in ECD development and point to new biological pathways that may contribute to the disease's progression.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of several organs. The diagnosis and management of EGPA are often challenging and require an integrated, multidisciplinary approach. Current practice relies on recommendations and guidelines addressing the management of ANCA-associated vasculitis and not specifically developed for EGPA.
View Article and Find Full Text PDF