Background: Juvenile idiopathic arthritis-associated uveitis (JIA-U) is a rare condition, and assessment of the efficacy of disease-modifying antirheumatic drugs, synthetic (sDMARD) or biological (bDMARD), in randomized trials is hindered by this fact. Methods: In this prospective longitudinal study, we observed 38 children aged 1.3 to 15.2 years, with 69 eyes affected with JIA-U for 1970 overall eye examinations (6–59, median 16) irregularly scattered across 4.4–87.6 months (median 21.6) of follow-up, with on- and off-periods of DMARD use and use of topical treatments. Results: With adjustment for several time-invariant and time-varying covariates, periods of exposure to sDMARD vs. no DMARD exposure were associated with peak benefits of 15–20% lower probability of having more severe anterior chamber (AC) inflammation and a similar relative reduction in the daily use of topical corticosteroids (TCS). Periods of bDMARD exposure or of bDMARD + sDMARD exposure vs. no DMARD use were associated with peak benefits of an around 50% reduction in the probability of having more severe AC inflammation, and peak benefits of an around 60–65% reduction in TCS use. Conclusions: The observations regarding bDMARD (only) or bDMARD + sDMARD exposure are in agreement with the extent of benefits suggested for adalimumab vs. placebo (+background sDMARD) in the only existing randomized trial in this setting evaluating AC inflammation and TCS use.

Background: Central retinal artery occlusion (CRAO) is an ophthalmic emergency attributed to a vessel occlusion with an embolus or a thrombus and may occur during the hypercoagulable state, inflammation, or vasculitis. CRAO may occur in children; however its incidence is very rare. Most pediatric cases have detectable etiologies. Case Presentation: We describe the case of an otherwise-healthy six-year-old female, who presented with the sudden and complete vision loss of the left eye lasting over twelve hours after a six-day chickenpox exanthema, followed by a high fever. All the ophthalmological, laboratory, and instrumental investigations led to the diagnosis of a left CRAO. Laboratory testing was unremarkable except for the transient elevation of D dimers (1363 µg/L), IgM anticardiolipin antibodies (238.5 CU), and IgG anti-beta-2 glycoprotein-1 antibodies (76.1 CU) on admission. Thrombolytic treatment was not exerted because of late presentation to the hospital. Treatment with steroids, antiviral medications, antibiotics, and anticoagulants was obtained, but the visual outcome was poor during the hospitalization and at the last follow-up. We could not ascribe features of this case to any etiological condition apart from the documented ongoing chickenpox infection. Conclusions: This is the first case report of CRAO in a child with transient aPL elevation and acute chickenpox infection.