Efficacy of Anti-TNFα in Severe and Refractory Neuro-Behcet Disease: An Observational Study.
To report the safety and efficacy of anti-tumor necrosis factor α (TNFα) therapy in severe and refractory neuro-Behçet disease (NBD) patients.Observational, multicenter study including 17 BD patients (70.6% of male, with a median age of 39.3 [24-60] years), with symptomatic parenchymal NBD, refractory to previous immunosuppressant and treated with anti-TNFα (infliximab 5 mg/kg [n = 13] or adalimumab [n = 4]). Complete remission was defined by the disappearance of all neurological symptoms and by the improvement of radiological abnormalities at 12 months.Overall improvement following anti-TNF was evidenced in 16/17 (94.1%) patients including 6 (35.3%) complete response and 10 (58.8%) partial response. The median time to achieve remission was 3 months (1-6). The median Rankin score was 2 (1-4) at the initiation of anti-TNFα versus 1 (0-4) at the time of remission (P = 0.01). Corticosteroids have been stopped in 4 (23.5%) patients, and reduced by more than 50% as compared with the dosage at baseline in 10 (58.8%) patients. Side effects occurred in 23.5% of patients and required treatment discontinuation in 17% of cases.TNF blockade represents an effective therapeutic approach for patients with severe and refractory NBD, a difficult to treat population.
From APHP Groupe Hospitalier Pitié-Salpétrière Paris, France: Service de Medecine Interne et Immunologie Clinique (ACD, PC, DS), Service de Neurologie (RD), and Service de Medecine Interne 2 (FC) Service de Neuroradiologie Diagnostique et Fonctionnelle (AB); DHU Inflammation Immunopathologie, Biothérapie, Université Pierre et Marie Curie, Paris, France (ACD, PC, DS); Inserm U1127, CNRS UMR 7225, Sorbonne Universités, Université Pierre et Marie Curie Paris 6 and UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, Inria Paris-Rocquencourt, F-75013, Paris, France (AB); Service de Médecine Interne, CHU Grenoble, France (AD); Service de Médecine Interne, Groupement Hospitalier-Hôpital Edouard Herriot, Lyon, France (LP); Service de Médecine Interne, Hôpital Claude Huriez, CHRU Lille, France (EH, ML, DL) ; Service de Médecine Interne Hôpital Foch, Suresnes, France (BS, FA); Service de Rhumatologie, CHU Le Kremlin Bicêtre, France (XM) ;Service de Médecine Interne, CHU Bois-Guillaume, Rouen, France (IM); and Rheumatology Unit, Department of Internal Medicine, Reggio Emilia, Italy (OA, CS).
Anne Claire
AC
Desbois
Olga
O
Addimanda
Anne
A
Bertrand
Alban
A
Deroux
Laurent
L
Pérard
Raphael
R
Depaz
Eric
E
Hachulla
Marc
M
Lambert
David
D
Launay
Benjamin
B
Subran
Felix
F
Ackerman
Xavier
X
Mariette
Fleur
F
Cohen
Isabelle
I
Marie
Carlo
C
Salvarini
Patrice
P
Cacoub
David
D
Saadoun
D000068879:Adalimumab / Q000627:therapeutic use*; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D018501:Antirheumatic Agents / Q000627:therapeutic use; D001528:Behcet Syndrome / Q000175:diagnosis / Q000188:drug therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D000069285:Infliximab / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D018124:Receptors, Tumor Necrosis Factor / Q000037:antagonists & inhibitors*; D012074:Remission Induction; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
D000893:Anti-Inflammatory Agents; D018501:Antirheumatic Agents; D018124:Receptors, Tumor Necrosis Factor; D000069285:Infliximab; D000068879:Adalimumab