Anti-IL6-Receptor Tocilizumab in Refractory Uveitis Associated to Extraocular Manifestations in Patients with Behçet’s Disease. Multicenter Study

Belén Atienza-Mateo¹, José Luis Martín-Varillas¹, Lucia C. Domínguez-Casas¹, Nuria Vegas-Revenga¹, Vanesa Calvo-Río¹, Natalia Palmou Fontana², Ricardo Blanco³, Javier Loricera¹, MC Gonzalez-Vela⁴, Emma Beltrán⁵, Lucía Martínez Costa⁶, Elia Valls Pascual⁶, Marisa Hernández Garfella⁵, Antonio Atanes⁷, Miguel Cordero Coma⁸, Joan Miquel Nolla Solé⁹, Carmen Carrasco Cubero¹⁰, Enar Pons¹¹ and Miguel Angel González-Gay², ¹Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL. Santander. Universidad de Cantabria. Spain, Santander, Spain, ²Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL. Santander. Universidad de Cantabria., Santander, Spain, ³Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁴Pathology Anatomy, Hospital Universitario Marqués de Valdecilla. IDIVAL. Santander. Universidad de Cantabria. Spain, Santander, Spain, ⁵Hospital General Universitario de Valencia, Valencia, Spain, ⁶Hospital Peset, Valencia, Spain, ⁷Hospital Universitario de A Coruña, A Coruña, Spain, ⁸Hospital de León, León, Spain, ⁹Hospital Universitari de Bellvitge, Barcelona, Spain, ¹⁰Hospital Universitari Germans Trias i Pujol, Barcelona, Spain, ¹¹Hospital Universitario Marqués de Valdecilla. IDIVAL. Santander. Universidad de Cantabria., Santander, Spain

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: anti-TNF therapy and tocilizumab, Behcet's syndrome

Session Information

Date: Tuesday, November 7, 2017

Title: Vasculitis Poster III: Other Vasculitis Syndromes

Session Type: ACR Poster Session C

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

Background/Purpose: To assess the efficacy of Tocilizumab (TCZ) in refractory uveitis associated to extraocular manifestations due to Behçet’s disease (BD).

Methods: Multicenter study of patients with BD refractory to standard systemic treatment (conventional immunosuppressive drugs and/or anti-TNF-α agents).

Results: We studied 11 patients (7 men/4 women) (20 affected eyes); mean age 38.4±20.4 years. Uveitis was bilateral in 9 patients. The pattern of ocular involvement was: panuveitis (n=8; with retinal vasculitis in 4), anterior uveitis (n=2) and posterior uveitis (n=1). Cystoid macular edema (CME) was present in 7 patients. The clinical course was recurrent (n=7) or chronic (n=4). Apart from the visual complications, at TCZ onset the following extraocular manifestations were present: oral and/or genital ulcers (n=7), arthritis (n=4), folliculitis/pseudofolliculitis (n=4), erythema nodosum (n=2), livedo reticularis (n=1), and neurological involvement (n=2). Before TCZ, they had received systemic corticosteroids, conventional immunosuppressive drugs and biologic agents, adalimumab (n=8), infliximab (n=4), golimumab (n=3), canakinumab (n=1), or etanercept (n=1). TCZ was used in monotherapy or combined with conventional immunosuppressive drugs at 8 mg/kg/iv/4 weeks (n=10) or 162 mg/sc/week (n=1). TCZ yielded rapid and maintained improvement in all ocular parameters (TABLE). After a mean follow-up of 9.5±8.05 months using TCZ, all patients experienced ocular improvement, with complete remission in 8 of them. However, TCZ was only effective in 3 of the 11 patients with extraocular manifestations. This biologic agent had to be withdrawn in 2 cases, 1 due to a severe infusion reaction and 1 due to arthritis impairment.

Conclusion: TCZ appears to be useful in highly refractory BD-related uveitis. However, there are controversial results regarding its efficacy in the treatment of extraocular manifestations of BD.

Table.

Case	Sex / Age (years)	Ocular pattern and course	Extraocular manifestations	Manifestations that improved with TCZ therapy
1	Male/27	Bilateral posterior uveitis + unilateral CME Chronic	Oral ulcers, asymptomatic white matter lesions on MRI, arthritis, folliculitis	Uveitis and CME
	Female/42	Bilateral panuveitis + unilateral CME Chronic	Oral and genital ulcers, erythema nodosum	Uveitis and CME
3	Male / 50	Bilateral panuveitis/papillitis + unilateral CME Relapsing	Papillitis, arthritis	All of them (uveitis, papillitis, CME and arthritis)
4	Male / 35	Bilateral panuveitis + retinal vasculitis Relapsing	Oral ulcers, folliculitis	Uveitis and retinal vasculitis
5	Female/67	Bilateral panuveitis + retinal vasculitis + bilateral CME	Livedo reticularis	Uveitis, retinal vasculitis and CME
6	Male / 31	Unilateral panuveitis + retinal vasculitis + unilateral CME Relapsing	Oral and genital ulcers, folliculitis	Uveitis, retinal vasculitis and CME
7	Female/22	Bilateral panuveitis + bilateral CME Chronic	None	Uveitis and CME
8	Male / 75	Bilateral panuveitis + retinal vasculitis + unilateral CME Relapsing	Oral and genital ulcers, arthritis, folliculitis	Uveitis, retinal vasculitis and arthritis
9	Male / 10	Bilateral anterior uveitis Relapsing	Oral and genital ulcers, hemorrhagic stroke, erythema nodosum	Uveitis, oral and genital ulcers and erythema nodosum
10	Female/48	Bilateral anterior uveitis Relapsing	Oral and genital ulcers, arthritis, pseudofolliculitis, erythema nodosum, intestinal involvement	Uveitis
11	Male / 16	Unilateral panuveitis Chronic	Oral ulcers, arthritis	Uveitis

Abbreviations: CME, cystoid macular edema; MRI, magnetic resonance imaging.

Disclosure: B. Atienza-Mateo, None; J. L. Martín-Varillas, None; L. C. Domínguez-Casas, None; N. Vegas-Revenga, None; V. Calvo-Río, None; N. Palmou Fontana, None; R. Blanco, None; J. Loricera, None; M. Gonzalez-Vela, None; E. Beltrán, None; L. Martínez Costa, None; E. Valls Pascual, None; M. Hernández Garfella, None; A. Atanes, None; M. Cordero Coma, None; J. M. Nolla Solé, None; C. Carrasco Cubero, None; E. Pons, None; M. A. González-Gay, None.

To cite this abstract in AMA style:

Atienza-Mateo B, Martín-Varillas JL, Domínguez-Casas LC, Vegas-Revenga N, Calvo-Río V, Palmou Fontana N, Blanco R, Loricera J, Gonzalez-Vela M, Beltrán E, Martínez Costa L, Valls Pascual E, Hernández Garfella M, Atanes A, Cordero Coma M, Nolla Solé JM, Carrasco Cubero C, Pons E, González-Gay MA. Anti-IL6-Receptor Tocilizumab in Refractory Uveitis Associated to Extraocular Manifestations in Patients with Behçet’s Disease. Multicenter Study [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/anti-il6-receptor-tocilizumab-in-refractory-uveitis-associated-to-extraocular-manifestations-in-patients-with-behcets-disease-multicenter-study/. Accessed .

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