Intraocular Lymphoma: Descriptive Data of 26 Patients Including Clinico-pathologic Features, Vitreous Findings, and Treatment Outcomes.

PURPOSE: To describe clinical manifestations, diagnostic approaches, therapy, and outcomes of biopsy-proven intraocular lymphoma. METHODS: Review of tertiary referral center records between 2005 and 2015. RESULTS: A total of 51 eyes of 26 patients were included; mean age of onset was 60.42 years. Common ocular complaints included floaters (42%) and blurred vision (35%); 62% of patients had ocular and central nervous system involvement; 11% had systemic lymphoma; and 27% had only ocular involvement. Vitreous analysis was positive for malignant cells in 77% of patients on initial biopsy, and in 100% of patients on repeat biopsy. In total, 20/26 patients received systemic and topical treatment before IOL diagnosis was made; 25 patients received intravitreal methotrexate and/or rituximab; one patient received intracameral rituximab. All patients achieved remission by their final visit. CONCLUSIONS: Intraocular lymphoma often masquerades as intraocular inflammation, resulting in delayed or misdiagnosis with subsequent inappropriate management. Optimal therapy is a challenge for oncologists and ophthalmologists.

Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations.

Granulomatosis with polyangiitis (GPA) is a potentially lethal systemic disorder that is characterized by necrotizing vasculitis of small arteries and veins. The respiratory system is most commonly affected in limited forms of the disease, however upper and lower respiratory system, systemic vasculitis, and necrotizing glomerulonephritis are the characteristic components of the disease triad. The peak incidence is observed at 64-75 years of age, with a prevalence of 8-10 per million depending on geographic location. In this review we focus on the ocular manifestations of the disease which occur in nearly in one third of the patients. In addition we describe the neuro-ophthalmic complications which occur in up to half of cases. We also discuss the current systemic treatment options including corticosteroids, cyclophosphamide, azathioprine, and the available biologic response modifiers including rituximab. The disease remains difficult to diagnose due to the generalized symptomatic presentation of patients with GPA. As a result, several sets of diagnostic criteria have been developed which include clinical, serological, and histopathological findings to varying extents. Early diagnosis and multi-specialty collaboration among physicians is necessary to adequately manage the disease and the potential complications that may result from drugs used in the treatment of the disease. Despite recent advances, more research is necessary to prevent the high rates of mortality from the disease itself and from therapeutic side effects.

Ocular morbidities of juvenile idiopathic arthritis-associated uveitis in adulthood: results from a tertiary center study.

PURPOSE: To describe the clinical and visual outcomes of juvenile idiopathic arthritis (JIA)-associated uveitis in adults and to examine risk factors for ongoing inflammation in adulthood. METHODS: Medical records were reviewed for patients with JIA-associated uveitis who were >16 years old at the final visit (the last visit prior to data collection). RESULTS: In total, 135 eyes of 77 patients (70 female, 7 male) were included. The mean age of patients at the final visit was 29.72 ± 11.27 years. The number of eyes with visual acuity of ≤20/50 and ≤20/200 at the final visit was 37 (28 %) and 20 (15 %), respectively; at least one ocular complication was present in 72 % of eyes. Band keratopathy was the most frequent complication (42 %), followed by cataract (25 %), posterior synechiae (22 %), maculopathy (22 %), ocular hypertension (13 %), and hypotony (5 %). At the final visit, patients who were >16 years of age at presentation to the Massachusetts Eye Research and Surgery Institution had more ocular complications and a greater degree of vision loss than patients who were ≤16 years of age. Ongoing inflammation at the final visit was noted in 40 patients (52 %). The presence of posterior synechiae, hypotony, cataract at presentation, and a history of cataract surgery prior to presentation were predictive of ongoing inflammation in adulthood in univariate analysis. The presence of hypotony and posterior synechiae at the initial visit were predictive factors in multivariate analysis. CONCLUSIONS: JIA-associated uveitis may be associated with ongoing inflammation, ocular complications, and severe visual impairment in adulthood. The presence of posterior synechiae and hypotony at the initial visit is predictive of ongoing inflammation.

Current Treatment Modalities of JIA-associated Uveitis and its Complications: Literature Review.

Uveitis is a common and serious complication of juvenile idiopathic arthritis. Up to 75% of all cases of anterior uveitis in childhood are associated with juvenile idiopathic arthritis. Despite the remarkable progress in early detection and treatment of inflammation, vision-threatening complications of uveitis still occur in almost 60% of patients. Structural complications include band keratopathy, maculopathy (macular edema, macular cysts, and epiretinal membrane), glaucomatous optic neuropathy, and cataracts. The management of complications in juvenile idiopathic arthritis is usually complex and requires early surgical intervention. In this paper, we review the general concepts of common ocular complications seen in patients with JIA-associated uveitis, with special attention to the recent diagnostic and preferred treatment approaches at the Massachusetts Eye Research and Surgery Institution. Received 9 March 2015; revised 30 September 2015; accepted 30 October 2015; published online 14 January 2016.

A case of bilateral uveitis and papillitis in a patient treated with pembrolizumab.

PURPOSE: Drug-induced uveitis is a well-known effect of ocular inflammation that has been reported with many medications. Pembrolizumab is a newer generation of the anti-programmed cell death-1 monoclonal antibodies that was recently approved by the Food and Drug Administration for the treatment of advanced melanoma. Immune-mediated adverse events involving different organs have been reported in recent literature in association with this drug. We present the first reported case of uveitis in association with pembrolizumab therapy. CASE REPORT: An 82-year-old man with stage IV melanoma was started on pembrolizumab infusion treatment every 3 weeks. Two months after initiating therapy, he presented with bilateral severe anterior uveitis and papillitis with fast and complete recovery after withholding further pembrolizumab infusions and treatment with topical steroid. Uveitis recurred after restarting pembrolizumab therapy. CONCLUSIONS: In current clinical practice, many new drugs are being approved, requiring better characterization of the prevalence, onset, and nature of adverse events in order to aid development of effective management strategies. Ophthalmologists should keep in mind that drugs are always a possible cause of ocular inflammation in patients presenting with uveitis.

Decompression Retinopathy after ExPRESS Shunt Implantation for Steroid-Induced Ocular Hypertension: A Case Report.

Purpose. To present a unique case of decompression retinopathy after the implantation of ExPRESS drainage device. Method. A 25-year-old female patient underwent implantation of ExPRESS drainage device in the left eye for the management of steroid-induced ocular hypertension. Results. On the postoperative day one, best-corrected visual acuity in the left eye was 20/50. Fundus examination revealed diffuse intraretinal hemorrhages, some white-centered, throughout the retina. There was also marked tortuosity to the retinal vasculature and no evidence of choroidal effusion. Intravenous fluorescein angiography and indocyanine green did not contribute to the aetiopathogenesis. Conclusion. Decompression retinopathy can occur following the implantation of ExPRESS drainage device. It is very important to be aware of this complication in patients with relatively high intraocular pressure who is planned for filtration surgery, including the ExPRESS implant.