Sudden visual loss in a child with juvenile idiopathic arthritis-related uveitis

An 8-year-old Italian girl with early-onset oligo-juvenile idiopathic arthritis (JIA) and bilateral anterior uveitis (AU), was treated at first with prednisone (0.5–1 mg/kg/day) and methotrexate (10 mg/m2/week), then, due to severe arthritis, with etanercept (0.4 mg/kg twice/week). Despite the improvement in arthritis, in 2 years’ time, the child underwent six relapses of bilateral AU with development of posterior synechiae and cystoid macular oedema (CMO). Etanercept was then discontinued and, after a 2-month washout period, infliximab (5 mg/kg at 0, 2, 6 weeks and then every 4 weeks) was administered. Visual acuity soon improved, and CMO disappeared. After 12 months of treatment, the child complained of a sudden visual loss on the right side. Since ocular examination was impaired by the presence of synechiae and cataract, several instrumental tests have been performed. Visual evoked potentials (VEPs) howed a reduced amplitude and prolonged latency of P100 component of the right eye. Automated perimetry (AP) evidenced no responses to the stimuli in the right eye, while the electroretinogram (ERG) was normal. MRI of the orbits revealed enhancement in the intraconic portion of the right optic nerve, with hypointensity in the short-tau inversion recovery (STIR) coronal images after gadolinium injection (fig 1). MRI of the brain was otherwise normal. Infliximab treatment was interrupted, and the patient was treated with three consecutive intravenous methylprednisolone pulses (15 mg/kg) followed by oral prednisone (0.3 mg/kg/day). Three weeks later, orbital MRI (fig 2) and VEPs revealed normalisation in the right optic nerve. The decimal BCVA in both eyes was 0.63 a month before ON, and decreased to 0.1 in the RE when ON was detected. At the end of the follow-up, 10 months after ON onset, the BCVA went up to 0.5 in the RE.