Abatacept: a potential therapy in refractory cases of juvenile idiopathic arthritis-associated uveitis

Abstract

Background: Juvenile idiopathic arthritis (JIA) is the most common of all systemic conditions associated with childhood uveitis. Visual impairment has been shown to be as high as 40% of which 10% being blind (6/60 or worse). Due to the lack of well-designed randomized control trials for paediatric uveitis and arthritis there are limited comparative data regarding the efficacy of single or combination treatments. Recently, abatacept was shown to control ocular inflammation in a case of psoriatic arthritis- associated uveitis, seven cases of JIA- associated uveitis and in JIA. We present two cases with JIA-associated uveitis who have responded dramatically to abatacept therapy following unsuccessful therapy with other immunosuppressants. Control of arthritis still represents a challenge with this treatment.

Methods: Prospective review of two patients with refractory JIA- associated uveitis not responding to maximum conventional treatment. Patients were regularly reviewed in the ophthalmology and rheumatology clinics. Assessment of their ocular condition was characterized according to the Standardization of Uveitis Nomenclature (SUN) group.

Results: In case 1, ocular inflammation was brought under control after repeated abatacept infusions. Case 2 showed complete resolution of cystoids macular edema CME and improvement of 5 Snellen's lines in best corrected visual acuity. After 9 months, the ocular condition of both patients remains in remission with steroid sparing. Joint disease was brought to clinical remission in case 2, but not in case 1.

Conclusions: Abatacept is a promising alternative treatment in refractory cases of JIA uveitis but may not be as successful in controlling joint disease. Larger series with long term follow up of biological therapies in paediatric uveitis are essential to assess the efficacy and cost effectiveness.