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Vasculitis
Benralizumab for eosinophilic granulomatosis with polyangiitis
  1. Adrien Cottu1,
  2. Matthieu Groh2,
  3. Charlene Desaintjean3,
  4. Sylvain Marchand-Adam4,
  5. Loïc Guillevin1,
  6. Xavier Puechal1,
  7. Stacy Beaumesnil5,
  8. Estibaliz Lazaro5,
  9. Maxime Samson6,
  10. Camille Taille7,
  11. Cécile-Audrey Durel8,
  12. Elizabeth Diot9,
  13. Sarah Nicolas9,
  14. Laurent Guilleminault10,11,
  15. Mikael Ebbo12,
  16. Pascal Cathebras13,
  17. Clairelyne Dupin14,
  18. Halil Yildiz15,
  19. Nabil Belfeki16,
  20. Grégory Pugnet17,
  21. Pierre Chauvin18,
  22. Stephane Jouneau19,
  23. Francois Lifermann20,
  24. Jean-Philippe Martellosio21,
  25. Vincent Cottin3,
  26. Benjamin Terrier1,22
  27. For the French Vasculitis Study Group
  1. 1 Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hospital Cochin, Paris, France
  2. 2 National Referral Center for Hypereosinophilic Syndrome (CEREO), Department of Internal Medicine, Hopital Foch, Suresnes, France
  3. 3 Department of Respiratory Diseases, Hospital for Cardiologie and Pneumology Louis Pradel, Lyon, France
  4. 4 Service de pneumologie et d'explorations fonctionnelles respiratoires, CHRU de Tours, Tours, France
  5. 5 Department of Internal Medicine and Infectious Diseases, University Hospital Centre, Bordeaux, France
  6. 6 Department of Internal Medicine and Clinical Immunology, University Hospital Centre, Dijon, France
  7. 7 Reference Center for Rare Pulmonary Diseases and University of Paris Cité, Inserm 1152, Hospital Bichat - Claude-Bernard, Paris, France
  8. 8 Department of Internal Medicine, Edouard Herriot Hospital, Lyon, France
  9. 9 Department of Internal Medicine, CHRU de Tours, Tours, France
  10. 10 Department of Respiratory Medicine, University Hospital Centre Toulouse, Toulouse, France
  11. 11 Toulouse Institute for Infectious and Inflammatory Diseases (Infinity), Inserm U1291, CNRS U5282, Toulouse 2 University, Toulouse, France
  12. 12 Departement of Internal Medicine, Assistance Publique - Hôpitaux de Marseille, Marseille, France
  13. 13 Department of Internal Medicine, CHU, Saint-Etienne, France
  14. 14 Reference Center for Rare Pulmonary Diseases and University of Paris Cité, Hospital Bichat - Claude-Bernard, Paris, France
  15. 15 Department of Internal Medicine and Infectious Diseases, Cliniques universitaires Saint-Luc, Bruxelles, Belgium
  16. 16 Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Sud Ile-de-France, Melun, France
  17. 17 Department of Internal Medicine and Clinical Immunology, CHU Toulouse Rangueil, Toulouse, France
  18. 18 Department of Respiratory Diseases, University Hospital Centre Rennes, Rennes, France
  19. 19 Department of Respiratory Diseases, IRSET UMR 1085, Rennes 1 University, Pontchaillou Hospital, Rennes, France
  20. 20 Department of Internal Medicine, Centre Hospitalier de Dax, Dax, France
  21. 21 Department of Internal Medicine, Centre Hospitalier Universitaire de Poitiers, Poitiers, France
  22. 22 Université Paris Cité, Paris, France
  1. Correspondence to Dr Benjamin Terrier, Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hospital Cochin, Paris, 75014, France; benjamin.terrier{at}aphp.fr

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.

  • Treatment
  • Glucocorticoids
  • Systemic vasculitis
  • Biological Therapy

Data availability statement

Data are available on reasonable request.

http://dx.doi.org/10.1136/ard-2023-224624

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    Data availability statement

    Data are available on reasonable request.

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    Footnotes

    • Handling editor Josef S Smolen

    • Twitter @Maxime_Samson21, @#OrphaLung, @TerrierBen

    • Contributors Data collection and analysis was performed by AC and BT. The first draft of the manuscript was written by AC and BT. All authors commented on previous versions of the manuscript and approved the final manuscript. BT has full responsibility for the finished work and the conduct of the study and controlled the decision to publish.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests The authors declare no financial support. MG reports personal fees from GlaxoSmithKline and AstraZeneca outside the submitted work. CT reports personal fees from AstraZeneca, Sanofi, GlaxoSmithKline, Chiesi and Novartis outside the submitted work. CDupin reports personal fees from AstraZeneca and GlaxoSmithKline outside the submitted work. HY reports personal fees from GlaxoSmithKline outside the submitted work. Other authors has nothing to disclose.

    • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.