Tocilizumab in Behçet Disease: A Multicenter Study of 30 Patients

Mohamed-Yacine Khitri; Alessandra Bartoli; Georgina Maalouf; Alban Deroux; Carlo Salvarani; Giacomo Emmi; Omer Karadag; Gerard Espinosa; Mathilde Leclercq; Gabriele Simonini; Mathieu Vautier; Patrice Cacoub; David Saadoun

doi:10.3899/jrheum.221106

Tocilizumab in Behçet Disease: A Multicenter Study of 30 Patients

J Rheumatol. 2023 Jul;50(7):916-923. doi: 10.3899/jrheum.221106. Epub 2023 Mar 1.

Affiliations

¹ M.Y. Khitri, MD, A. Bartoli, MD, G. Maalouf, MD, M. Vautier, MD, P. Cacoub, MD, PhD, D. Saadoun, MD, PhD, Department of Internal Medicine and Clinical Immunology France, Centre National de Référence Maladies Autoimmunes Systémiques Rares, Centre National de Référence Maladies Autoinflammatoires et Amylose, and Inflammation-Immunopathology-Biotherapy Department (DMU i3), Sorbonne Université, AP-HP, Hôpital Pitié Salpetrière, Paris, France.
² A. Deroux, MD, Department of Internal Medicine, University Hospital of Grenoble, Grenoble, France.
³ C. Salvarani, MD, PhD, Division of Rheumatology, Azienda USL-IRCCS di Reggio Emilia, University of Modena and Reggio Emilia, Modena and Reggio Emilia, Italy.
⁴ G. Emmi, MD, PhD, Department of Experimental and Clinical Medicine, University of Florence, Florence, Behçet Centre, Careggi University Hospital, Florence, Italy, and Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Clayton, Victoria, Australia.
⁵ O. Karadag, MD, PhD, Division of Rheumatology, Department of Internal Medicine, Vasculitis Research Center, Hacettepe University School of Medicine, Ankara, Turkey.
⁶ G. Espinosa, MD, PhD, Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Universitat de Barcelona, Barcelona, Spain.
⁷ M. Leclercq, MD, Internal Medicine Department, CHU Rouen, Rouen, France.
⁸ G. Simonini, MD, Rheumatology Unit, NEUROFARBA Department, Meyer Children's Hospital, University of Florence, Florence, Italy.
⁹ M.Y. Khitri, MD, A. Bartoli, MD, G. Maalouf, MD, M. Vautier, MD, P. Cacoub, MD, PhD, D. Saadoun, MD, PhD, Department of Internal Medicine and Clinical Immunology France, Centre National de Référence Maladies Autoimmunes Systémiques Rares, Centre National de Référence Maladies Autoinflammatoires et Amylose, and Inflammation-Immunopathology-Biotherapy Department (DMU i3), Sorbonne Université, AP-HP, Hôpital Pitié Salpetrière, Paris, France; david.saadoun@aphp.fr.

PMID: 36858435
DOI: 10.3899/jrheum.221106

Abstract

Objective: To evaluate tocilizumab (TCZ) efficacy in patients with refractory Behçet disease (BD).

Methods: This is a multicenter study of 30 patients fulfilling the international criteria for BD and treated with TCZ at different European referral centers. The clinical response was evaluated at 6 months from TCZ initiation.

Results: Ninety percent of patients with BD were refractory or intolerant to anti-tumor necrosis factor (anti-TNF) agents. Overall, TCZ was effective in 25 (83%) patients with BD of whom 18 (60%) and 7 (23%) were complete and partial responders, respectively. The complete response was 67%, 60%, and 42% in patients with uveitis (18/30), neurological manifestations (5/30), and mucocutaneous and/or articular (7/30) manifestations, respectively. TCZ had a significant steroid-sparing effect allowing patients to decrease their median daily prednisone dose from 20 (IQR 10-40) mg/day to 9 (IQR 5-13) mg at 6 months (P < 0.001). The number of patients with BD needing concomitant disease-modifying antirheumatic drug therapy fell from 7 (23%) to 4 (13%) at 6 months. Mild to moderate side effects were observed in 6 (20%) patients, and 3 (10%) presented with serious adverse events (pneumonia, intestinal perforation, and septicemia) requiring therapy discontinuation in 2 cases.

Conclusion: TCZ seems to be an effective alternative to anti-TNF agents in treating BD-related uveitis and neurological manifestations.

Keywords: Behçet disease; neuro-Behçet; tocilizumab; uveitis.

Publication types

Multicenter Study

MeSH terms

Antirheumatic Agents* / therapeutic use
Behcet Syndrome* / complications
Behcet Syndrome* / drug therapy
Humans
Retrospective Studies
Treatment Outcome
Tumor Necrosis Factor Inhibitors / therapeutic use
Tumor Necrosis Factor-alpha
Uveitis* / complications
Uveitis* / etiology

Substances

tocilizumab
Tumor Necrosis Factor Inhibitors
Antirheumatic Agents
Tumor Necrosis Factor-alpha