Benralizumab for eosinophilic granulomatosis with polyangiitis.

Abstract

BACKGROUND:

Benralizumab is effective in the treatment of eosinophilic asthma and is being investigated for the treatment of other eosinophil-associated diseases. Reports on the use of benralizumab for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) are limited to case reports and small case series.

METHODS:

We conducted a multicentre, retrospective study including EGPA patients treated with off-label benralizumab. The primary endpoint was the rate of complete response defined as no disease activity (Birmingham Vasculitis Activity Score=0) and a prednisone dose ≤4 mg/day. Partial response was defined as no disease activity and a prednisone dose ≥4 mg/day.

RESULTS:

Sixty-eight patients were included, including 31 (46%) who had previously received mepolizumab. The use of benralizumab was warranted by uncontrolled asthma in 54 (81%), persistent ear, nose and throat (ENT) manifestations in 27 (40%) and persistent glucocorticoids (GCs) use in 48 (74%) patients. Median (IQR) follow-up after starting benralizumab was 23 (9-34) months. Thirty-three patients (49%) achieved a complete response, 24 (36%) achieved a partial response and 10 (15%) did not respond. Among the 57 patients who initially responded, 10 (18%) eventually required further line treatments. GCs were discontinued in 23 patients (38%). Prior mepolizumab use was associated with a higher rate of primary failure (26.7% vs 5.4%, p=0.034) and less frequent GCs discontinuation (14.8% vs 55.9%, p=0.001). Vasculitis flares occurred in 7 patients (11%) and were associated with histological evidence of vasculitis and/or antineutrophil cytoplasmic antibodies positivity at benralizumab initiation (p=0.004).

CONCLUSIONS:

Benralizumab appears to be an effective treatment for refractory asthma or ENT manifestations in EGPA and allows GC-sparing. However, its efficacy was lower after prior failure of mepolizumab.

• Authors: Adrien Cottu, Matthieu Groh, Charlene Desaintjean, Sylvain Marchand-Adam, Loïc Guillevin, Xavier Puechal, Stacy Beaumesnil, Estibaliz Lazaro, Maxime Samson, Camille Taille, Cécile-Audrey Durel, Elizabeth Diot, Sarah Nicolas, Laurent Guilleminault, Mikael Ebbo, Pascal Cathebras, Clairelyne Dupin, Halil Yildiz, Nabil Belfeki, Grégory Pugnet, Pierre Chauvin, Stephane Jouneau, Francois Lifermann, Jean-Philippe Martellosio, Vincent Cottin, Benjamin Terrier, French Vasculitis Study Group
• Journal: Annals of the rheumatic diseases (Ann Rheum Dis) Vol. 82 Issue 12 Pg. 1580-1586 (12 2023) ISSN: 1468-2060 [Electronic] England
• PMID: 37550002 (Publication Type: Journal Article)
• Copyright: © Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.

Chemical References

• benralizumab
• Prednisone
• Glucocorticoids

Topics

• Humans
• Granulomatosis with Polyangiitis (drug therapy, complications)
• Churg-Strauss Syndrome (drug therapy)
• Prednisone (therapeutic use)
• Retrospective Studies
• Glucocorticoids (therapeutic use)
• Asthma (drug therapy, complications)