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IntroductionUveitis, a leading cause of preventable blindness around the world, is a critically underserved disease in regard to the medications approved for use. Multiple immunomodulatory therapy (IMT) drugs are appropriate for uveitis therapy but are still off-label. These IMT agents, including antimetabolites, calcineurin inhibitors, alkylating agents, and biologic agents, have been designated as “orphan drugs” and are widely used for systemic autoimmune diseases or organ transplantation.Area coveredThe purpose of this paper is to comprehensively review and summarize the approved orphan drugs and biologics that are being used to treat systemic diseases and to discuss drugs that have not yet received approval as an “orphan drug for treating uveitis” by the US Food and Drug Administration (FDA).Our perspectiveIMT, as a steroid-sparing agent for uveitis patients, has shown promising clinical results. Refractory and recurrent uveitis requires combination IMT agents. IMT is continued for a period of 2 years while the patient is in remission before considering tapering medication. Our current goals include developing further assessments regarding the efficacy, optimal dose, and safety in efforts to achieve FDA approval for “on-label” use of current IMT agents and biologics more quickly and to facilitate insurance coverage and expand access to the products for this orphan disease.

Aim To evaluate the long-term outcomes of corneal cross-linking (CXL) in patients with progressive keratoconus. Method In this retrospective non-comparative study, forty-five eyes of 31 patients with progressive keratoconus were treated with 30 min “epi-off” corneal cross-linking. The visual, refractive, topographic and tomographic outcomes were evaluated preoperatively and at least 10 years after cross-linking. Results Ten years post-corneal cross-linking, the mean anterior maximum keratometry decreased (−2.10 ± 3.25 D, P = 0.0001). Conversely, the posterior maximum keratometry increased (6.38 ± 9.79 D, P = 0.065). Both uncorrected and corrected distance visual acuity improved (LogMAR −0.08 ± 0.30) and (LogMAR −0.05 ± 0.21), respectively (P > 0.05, both). A statistically significant hyperopic shift was observed postoperatively (0.70 ± 1.31 D, P = 0.0009). The anterior topographic cylinder values revealed no change (−0.17 ± 1.31 D, P = 0.3), whereas the mean posterior cylinder values decreased (absolute value increased) significantly compared to baseline from −1.31 ± 0.97 D to −1.82 ± 1.78 D, (P < 0.05). The minimum corneal thickness values decreased significantly (−35.11 ± 48.63 µm, P = 0.0001). Four eyes (8.8%) showed more than 1 D increase in the anterior maximum keratometry. Conclusion This protocol and duration of Epi-off corneal cross-linking was found to be effective in halting keratoconus progression over the follow up period (10 years). Anterior corneal flattening and a hyperopic shift were observed. A statistically significant increase in the posterior corneal cylinder was observed. Although, not reaching statistical significance, the logMAR uncorrected and corrected visual acuity were improved.

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The possibility of electroretinography and perimetry results worsening after early IMT discontinuation remained if the patients couldn't achieve remission.

PurposeTo evaluate the efficacy and safety of Ahmed glaucoma valve (AGV) in eyes with noninfectious uveitis that had fluocinolone acetonide intravitreal implant (Retisert™)-induced glaucoma.MethodsThis retrospective study reviewed the safety and efficacy of AGV implantation in patients with persistently elevated intraocular pressure (IOP) after implantation of a fluocinolone acetonide intravitreal implant at the Massachusetts Eye Research and Surgery Institution between August 2006 and November 2015.ResultsNine patients with 10 uveitic eyes were included in this study, none of which had preexisting glaucoma in the study eye. Mean patient age was 42 years; 6 patients were female and 3 were male. Baseline mean IOP was 30.6 mmHg prior to AGV placement while mean IOP-lowering medications were 2.9. In the treatment groups, there was a statistically significant reduction in post-AGV IOP. IOP was lowest at 1-week after AGV implantation (9.0 mmHg). Nine out of 10 eyes achieved an IOP below target value of 22 mmHg and/or a 20% reduction in IOP from baseline 1 month and 1 year following AGV placement. All other postoperative time points showed all 10 eyes reaching this goal. A statistically significant decrease in IOP-lowering medication was seen at the 1-week, 1-month, and 3-year time points compared to baseline, while a statistically significant increase was seen at the 3-month, 6-month, and 2-year post-AGV time points. No significant change in retinal nerve thickness or visual field analysis was found.ConclusionAGV is an effective and safe method of treatment in fluocinolone acetonide intravitreal implant-induced glaucoma. High survival rate is expected for at least 3 years.

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