Biological Therapy in Neurobehçet: Multicenter Study of 31 Patients

Iñigo Gonzalez-Mazon¹, Lara Sanchez-Bilbao ², Jose Luis Martín-Varillas ², Belén Atienza-Mateo ², Vanesa Calvo Río ², Santos Castañeda ³, Esther Vicente-Rabaneda ⁴, Olga Maiz ⁵, ana blanco ⁵, Clara Moriano ⁶, Elvira Díez ⁶, JL Andreu ⁷, Concepción Delgado-Beltrán ⁸, Marta Loredo-Martinez ⁸, Javier Narváez ⁹, Angel Ramos-Calvo ¹⁰, Francisca Sivera ¹¹, E. Raya ¹², Norberto Ortego Centeno ¹², Jose luis callejas rubio ¹², Anahy Brandy-Garcia ¹³, Alejandro Olivé-Marqués ¹⁴, Sabela Fernández ¹⁵, Ricardo Gómez De La Torre ¹⁶, Iñaki Torre-Salaberri ¹⁷, Julio Sanchez ¹⁸, A. Urruticoechea-Arana ¹⁹, Eva salgado-Pérez ²⁰, Rafael Melero ²¹, Olga Martinez ²², Susana Romero-Yuste ²³, Miguel A Gonzalez-Gay ² and Ricardo Blanco ², ¹Rheumatology, Hospital Universitario Marques de Valdecilla, Santander, Cantabria, Spain, ²Rheumatology, Hospital Universitario Marques de Valdecilla, Santander, Spain, ³Rheumatology, Hospital Universitario La Princesa, Madrid, Madrid, Spain, ⁴Rheumatology Department, Hospital Universitario La Princesa, Madrid, Spain, ⁵Rheumatology, Hospital Universitario Donostia, san sebastian, Pais Vasco, Spain, ⁶Complejo Asistencial Universitario de León, León, Castilla y Leon, Spain, ⁷HU Puerta del Hierro, Madrid, ⁸H. Clinico Lozano Blesa Zaragoza, Zaragoza, Aragon, Spain, ⁹Rheumatology Department, Hospital Universitari de Bellvitge, Barcelona, Catalonia, Spain, ¹⁰Rheumatology, Complejo Hospitalario de Soria, soria, Castilla y Leon, Spain, ¹¹Hospital General Universitario de Elda, Elda, Spain, ¹²Hospital San Cecilio, Granada, Andalucia, Spain, ¹³Hospital Universitari Germans Trias i Pujol, Badalona, Spain, ¹⁴Hospital Universitari Germans Trias i Pujol, Badalona, Catalonia, Spain, ¹⁵Hospital Universitario San Agustín, Avilés, Asturias, Spain, ¹⁶Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain, Oviedo, Spain, ¹⁷BASURTO UNIVERSITY HOSPITAL, Bilbao, Spain, ¹⁸H. 12 de Octubre, Madrid, Spain, ¹⁹HU Can Misses, Ibiza, Spain, ²⁰Complejo Hospitalario Universitario de Ourense, Ourense, Galicia, Spain, ²¹Complexo Hospitalario Universitario de Vigo, Vigo, Galicia, Spain, ²²Hospital Clínico Universitario de Salamanca, Salamanca, Spain, ²³Complejo Hospitalario Universitario Pontevedra, Pontevedra, Galicia, Spain

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: Behcet's syndrome and vasculitis, Neurologic involvement

Session Information

Date: Monday, November 11, 2019

Session Title: Vasculitis – Non-ANCA-Associated & Related Disorders Poster III: Behçet’s Disease & Other Vasculitides

Session Type: Poster Session (Monday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Behçet’s disease (BD) is a variable vessel vasculitis and typically presents with mucocutaneous involvement. However, any organ can be affected, being the neurological affectation (neurobehçet, NB) one of the most serious manifestations.
Our aim was to assess the efficacy and safety of biological therapy as treatment of NB.

Methods: We set up a multicenter observational study of 31 patients with NB on treatment with biological therapy (BT). NB diagnosis was made by neuroimaging, CSF analysis and/or suggestive clinical signs of central and/or peripheral nervous system involvement, excluding infectious causes or more prevalent pathology. Results are expressed as mean±SD or as median and interquartile range (IQR) as appropriate.

Results: 31 patients (16♂ / 15♀) with an average age of 39.7 ± 10.5 years. HLA-B51 was positive in 51.6% of the patients. Table shows the non-neurological manifestations. Regarding the neurological manifestations, 23 patients (74.2%) had parenchymal involvement (hemiparesis (n=6), brainstem involvement (n=1), encephalopathy (n = 4), optic neuropathy (n=3), dysphasia (n=1), polyneuropathy (n=6), cognitive impairment (n=4), and non-steroidal psychosis (n=1), while the remaining 8 patients (25.8%) presented aseptic meningitis as a non-parenchymal affectation (Table).

Prior to BT, patients had received the following treatment: oral prednisone (n=29), methylprednisolone bolus (n=9), CsA (n=10), AZA (n=17), MTX (n=16) and mycophenolate (n=2).

After a median of 34 [10-72] months since the beginning of the neurological symptoms, the following BT was initiated: infliximab (IFX)(n=19), adalimumab (ADA)(n=7), tocilizumab (TCZ) (n=2), golimumab (GOL) (n=2) and Etanercept (ETN) (n=1). A first switch to ADA was necessary in 9 patients with IFX due to primary failure. In addition, 2 of them needed a second switch to TCZ, getting a partial response. The BT was discontinued in 5 patients, 2 of them for obtaining clinical remission and the remaining 3 for inefficacy.

After a median follow-up of 5.4±4.6 years, complete response was obtained in 15 patients, partial response in 11 and no response in the remaining 3. We observed an anaphylactic reaction and psoriasis induced by IFX, without other serious adverse events (Table).

Conclusion: BT, especially anti-TNF, seems effective and safe for treatment in NB.

Disclosure: I. Gonzalez-Mazon, None; L. Sanchez-Bilbao, None; J. Martín-Varillas, None; B. Atienza-Mateo, None; V. Calvo Río, None; S. Castañeda, None; E. Vicente-Rabaneda, None; O. Maiz, None; a. blanco, None; C. Moriano, None; E. Díez, None; J. Andreu, None; C. Delgado-Beltrán, None; M. Loredo-Martinez, None; J. Narváez, None; A. Ramos-Calvo, None; F. Sivera, None; E. Raya, None; N. Ortego Centeno, None; J. callejas rubio, None; A. Brandy-Garcia, None; A. Olivé-Marqués, None; S. Fernández, None; R. Gómez De La Torre, None; I. Torre-Salaberri, None; J. Sanchez, None; A. Urruticoechea-Arana, None; E. salgado-Pérez, None; R. Melero, None; O. Martinez, None; S. Romero-Yuste, None; M. Gonzalez-Gay, Abbvie, 2, 5, 8, Celgene, 5, 8, Janssen, 2, MSD, 2, 5, 8, Pfizer, 5, 8, Roche, 2, 5, 8, Sanofi, 5, 8, Sobi, 5, 8; R. Blanco, None.

To cite this abstract in AMA style:

Gonzalez-Mazon I, Sanchez-Bilbao L, Martín-Varillas J, Atienza-Mateo B, Calvo Río V, Castañeda S, Vicente-Rabaneda E, Maiz O, blanco a, Moriano C, Díez E, Andreu J, Delgado-Beltrán C, Loredo-Martinez M, Narváez J, Ramos-Calvo A, Sivera F, Raya E, Ortego Centeno N, callejas rubio J, Brandy-Garcia A, Olivé-Marqués A, Fernández S, Gómez De La Torre R, Torre-Salaberri I, Sanchez J, Urruticoechea-Arana A, salgado-Pérez E, Melero R, Martinez O, Romero-Yuste S, Gonzalez-Gay M, Blanco R. Biological Therapy in Neurobehçet: Multicenter Study of 31 Patients [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/biological-therapy-in-neurobehcet-multicenter-study-of-31-patients/. Accessed November 29, 2021.

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