Types of intraocular lenses for cataract surgery in eyes with uveitis.

BACKGROUND: Cataract formation often occurs in people with uveitis. It is unclear which intraocular lens (IOL) type is optimal for use in cataract surgery for eyes with uveitis. OBJECTIVES: To summarize the effects of different IOLs on visual acuity, other visual outcomes, and quality of life in people with uveitis. SEARCH METHODS: We searched CENTRAL (which contains the Cochrane Eyes and Vision Group Trials Register) (The Cochrane Library 2013, Issue 7), Ovid MEDLINE, Ovid MEDLINE In-Process and Other Non-Indexed Citations, Ovid MEDLINE Daily, Ovid OLDMEDLINE (January 1946 to August 2013), EMBASE (January 1980 to August 2013), Latin American and Caribbean Literature on Health Sciences (LILACS) (January 1982 to August 2013), the metaRegister of Controlled Trials (mRCT) (www.controlled-trials.com), ClinicalTrials.gov (www.clinicaltrials.gov) and the WHO International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en). We did not use any date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 14 August 2013. We also performed forward and backward searching using the Science Citation Index and the reference lists of the included studies, respectively, in August 2013. SELECTION CRITERIA: We included randomized controlled trials (RCTs) comparing hydrophobic or hydrophilic acrylic, silicone, or poly(methyl methacrylate) (PMMA) IOLs with or without heparin-surface modification (HSM), with each other, or with no treatment in adults with uveitis, for any indication, undergoing cataract surgery. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by The Cochrane Collaboration. Two review authors screened the search results and for included studies, assessed the risk of bias and extracted data independently. We contacted study investigators for additional information. We did not perform a meta-analysis due to variability in reporting and follow-up intervals for the primary and secondary outcomes of interest. MAIN RESULTS: We included four RCTs involving 216 participants (range of 2 to 140 participants with uveitic cataract per trial) and comparing up to four types of IOLs. The largest study was an international study with centers in Brazil, Egypt, Finland, France, Japan, the Netherlands, Slovak Republic, Spain, and the USA; two studies were conducted in Germany and one in Saudi Arabia. There was substantial heterogeneity with respect to the ages of participants and etiologies of uveitis within and across studies. The length of follow-up among the studies ranged from 1 to 24 months after cataract surgery. The studies were at low risk of selection bias, but two of the four studies did not employ masking and only one study included all randomized participants in the final analyses. The funding source was disclosed by investigators of the largest study (professional society) and not reported by the other three. Due to heterogeneity in lens types evaluated and outcomes reported among the trials, we did not combine data in a meta-analysis.In the largest study (140 participants), the study eye of each participant was randomized to receive one of four types of IOLs: hydrophobic acrylic, silicone, HSM PMMA, or unmodified PMMA. Proportions of participants with one or more Snellen lines of visual improvement were similar among the four treatment groups at one year' follow-up: 45 of 48 (94%) in the hydrophobic acrylic IOL group, 39 of 44 (89%) in the silicone IOL group, 18 of 22 (82%) in the HSM PMMA IOL group, and 22 of 26 (85%) in the unmodified PMMA IOL group. When comparing hydrophobic acrylic IOLs with silicone IOLs, the risk ratio (RR) was 1.06 (95% confidence interval (CI) 0.93 to 1.20). At one year' follow-up, fewer eyes randomized to hydrophobic acrylic IOLs developed posterior synechiae when compared with eyes receiving silicone IOLs (RR 0.18, 95% CI 0.04 to 0.79); the effects between these groups were less certain with respect to developing posterior capsule opacification (PCO) (RR 0.74, 95% CI 0.41 to 1.37), corneal edema (RR 0.49, 95% CI 0.22 to 1.12), cystoid macular edema (RR 0.10, 95% CI 0.01 to 1.84), or mild IOL decentration (RR 0.92, 95% CI 0.06 to 14.22).Two intra-individual studies also compared HSM PMMA IOLs with unmodified PMMA IOLs at three or six months of follow-up. These studies, including a combined total of 16 participants with uveitis, were insufficiently powered to detect differences in outcomes among eyes of people with uveitis randomized to receive HSM PMMA IOLs when compared with fellow eyes receiving unmodified PMMA IOLs.In the fourth study (60 participants), the study eye of each participant was randomized to receive a hydrophobic or hydrophilic acrylic IOL. At three months, there were no statistical or clinical differences between hydrophobic and hydrophilic acrylic IOL types in the proportions of participants with two or more Snellen lines of visual improvement (RR 1.03, 95% CI 0.87 to 1.22). There were similar rates in the development of PCO between hydrophobic or hydrophilic acrylic IOLs at six months' follow-up (RR 1.00, 95% CI 0.80 to 1.25). The effect of the lenses on posterior synechiae was uncertain at six months' follow-up (RR 0.50, 95% CI 0.05 to 5.22).None of the included studies reported quality of life outcomes. AUTHORS' CONCLUSIONS: Based on the trials identified in this review, there is uncertainty as to which type of IOL provides the best visual and clinical outcomes in people with uveitis undergoing cataract surgery. The studies were small, not all lens materials were compared in all studies, and not all lens materials were available in all study sites. Evidence of a superior effect of hydrophobic acrylic lenses over silicone lenses, specifically for posterior synechiae outcomes comes from a single study at a high risk of performance and detection bias. However, due to small sample sizes and heterogeneity in outcome reporting, we found insufficient information to assess these and other types of IOL materials for cataract surgery for eyes with uveitis.

Clinical features and incidence rate of ocular complications in punctate inner choroidopathy.

PURPOSE: To study the clinical features and incidence rate of ocular complications in patients with punctate inner choroidopathy. METHODS: This is a retrospective cohort study conducted in a single-center academic practice setting. Patients diagnosed with punctate inner choroidopathy at the Wilmer Eye Institute, Johns Hopkins University from 1984 to 2012 were identified. Demographics and clinical features including the presence of choroidal neovascularization (CNV) and structural complications were collected. Main outcome measures, including visual impairment and incidence rate of ocular complications, were analyzed. RESULTS: Thirty-one patients (59 eyes) were included in the study. Follow-up data were available for 24 patients (47 eyes) with a mean follow-up time of 3.4 years (range, 2 months to 8.7 years). In the affected eyes with follow-up, the incidence rate of visual impairment to 20/50 or worse was 0.06 per eye-year (EY) (95% confidence interval, 0.022/EY-0.114/EY). The incidence rate of visual loss to 20/200 or worse was 0.006/EY (95% confidence interval, 0.0001/EY-0.034/EY). Thirty-six eyes (77%) had an ultimate visual acuity of 20/40 or better. All of the 13 patients with more than ≥ 3 years of follow-up had a visual acuity of ≥ 20/40 in at least 1 eye at 3 years after presentation. Two thirds of the follow-up patients (67%) on immunomodulatory drug therapy did not have new or recurrent CNV. However, this was not a statistically significant difference. Three eyes with follow-up had recurrence of CNV for an incidence rate of 0.04/EY (95% confidence interval, 0.008/EY-0.12/EY). Two eyes developed new CNV during follow-up for an incidence rate of 0.02/EY (95% confidence interval, 0.002/EY-0.066/EY). CONCLUSION: The visual prognosis in most cases of punctate inner choroidopathy is very good. The incidence rate of new CNV and recurrent CNV was 0.02/EY and 0.04/EY, respectively.

Emerging drugs for the treatment of uveitis.

INTRODUCTION: Uveitis is a potentially visually debilitating disease when untreated or poorly controlled. Chronic intermediate, posterior, or panuveitis often requires systemic immunosuppressive therapy to prevent such visual loss. AREAS COVERED: This review discusses existing treatments for ocular inflammation including corticosteroids, antimetabolites, alkylating agents, T-cell inhibitors, and biologic agents. Potential drugs being studied in clinical trials are introducing new routes for local corticosteroid delivery, and novel immunomodulators are exploring new targets of the inflammatory cascade. EXPERT OPINION: Treatment options for uveitis have expanded from even a decade ago. However, more clinical trials and research are needed to further our understanding of the mechanisms of ocular inflammation and the safety and efficacy of novel therapies.

Occurrence of and Risk Factors for Ocular Hypertension and Secondary Glaucoma in Juvenile Idiopathic Arthritis-associated Uveitis.

PURPOSE: To describe the incidence of ocular hypertension (OHT) and secondary glaucoma (SG) in JIA-associated uveitis, identify risk factors for development of these complications, and describe their effect on visual outcomes. METHODS: A retrospective cohort of 108 patients (196 eyes) with JIA-associated uveitis seen over 30 years at an academic practice. RESULTS: Of examined eyes, 40% had OHT or SG at presentation. These eyes had a nearly three-fold higher incidence of legal blindness during follow-up, compared with eyes without OHT or SG. An additional 41 eyes developed OHT or SG during follow-up. Presenting with anterior uveitis, active inflammation, and using systemic corticosteroids were risk factors for developing OHT, while use of immunosuppressive medication at presentation reduced this risk. Risk factors for developing SG included anterior uveitis and use of systemic corticosteroids. CONCLUSIONS: OHT and SG were common in patients with JIA-associated uveitis. Use of immunosuppressive drugs may decrease the risk of developing OHT.

Acute Retinal Necrosis: Presenting Characteristics and Clinical Outcomes in a Cohort of Polymerase Chain Reaction-Positive Patients.

PURPOSE: To identify determinants of adverse outcomes in acute retinal necrosis (ARN), presenting characteristics and incidence rates of vision loss and ocular complications in a cohort of polymerase chain reaction (PCR)-positive eyes were analyzed. DESIGN: Retrospective observational cohort study. METHODS: Forty-one eyes of 36 patients with clinically diagnosed ARN, PCR-positive for herpes simplex virus or varicella zoster virus and evaluated between January 2002 and June 2013, were included. Main outcome measures included incidence rates of vision loss and retinal detachment (RD). RESULTS: Presenting visual acuity was generally poor (20/50 to >20/200 in 27%; 20/200 or worse in 56%). The incidence rate of ≤20/200 was 0.66/eye-year (EY), (95% confidence interval [CI], 0.32/EY to 1.22/EY); the rate of light perception or no light perception vision was 0.07/EY (95% CI, 0.02/EY to 0.16/EY). During follow-up, 59% of eyes developed at least 1 RD (rate = 0.40/EY, 95% CI, 0.19/EY to 0.58/EY). Eyes with retinitis involving ≥25% of the retina at presentation detached at nearly 12 times the rate, as compared to those with <25% retinal involvement (0.70/EY vs 0.06/EY; P = .001). Development of an RD was the greatest determinant of adverse visual outcomes, with 4% of eyes, that had experienced at least 1 RD, achieving a best-corrected visual acuity of ≥20/40 compared to 53% of eyes that never detached (P = .0003). CONCLUSIONS: Poor outcomes in ARN were common in this cohort. RD confers the greatest risk of incident vision loss, and once 25% or more of the retina is involved the risk of RD and visual loss increases significantly.

Autoimmune bullous diseases with skin and eye involvement: Cicatricial pemphigoid, pemphigus vulgaris, and pemphigus paraneoplastica.

Autoimmune blistering diseases are a heterogeneous group of disorders that mostly affect the skin and mucous membranes. Occasionally, other organ systems may be involved, depending on the unique pathophysiology of each disease. Cicatricial pemphigoid, pemphigus vulgaris, and paraneoplastic pemphigus are distinct entities, but all have the potential to have cutaneous and ocular involvement. Awareness and early recognition of ocular involvement in these diseases is important given the increased risk for vision loss and blindness with delay in management. Several skin diseases may be associated with involvement of the external eye. The most common autoimmune diseases are cicatricial pemphigoid, pemphigus vulgaris, and paraneoplastic pemphigus.

Risk of Hypotony in Juvenile Idiopathic Arthritis-Associated Uveitis.

OBJECTIVE: To describe risk factors for hypotony in patients with juvenile idiopathic arthritis (JIA)-associated uveitis. DESIGN: Retrospective cohort study. METHODS: All patients with JIA-associated uveitis (N = 108; affected eyes = 196) evaluated and followed at the Wilmer Eye Institute from July 1984 through June 2014 were included in this study. Prevalence and incidence of hypotony (intraocular pressure [IOP] <5 mm Hg) and low IOP (5 mm Hg ≤ IOP < 8 mm Hg) and risk factors for developing hypotony were analyzed. RESULTS: At presentation, 9.3% of patients (7.1% of affected eyes) had hypotony. During a median follow-up of 5.3 years, the rate of developing hypotony and low IOP were 0.04 per eye-year (/EY; 95% confidence interval [CI]: 0.02/EY, 0.05/EY) and 0.06/EY (95% CI: 0.04/EY, 0.08/EY), respectively. Risk factors for development of hypotony during follow-up appeared to be associated with more severe uveitic disease, such as the presence of panuveitis (adjusted hazard ratio [aHR], 43.1; P = .004), anterior chamber cells or flare ≥ 3+ (aHR, 25.6, P < .001), posterior synechiae (aHR, 5.9, P = .02), and the use of oral corticosteroid (aHR 28.9; P = .003) at the presenting examination. Receiving immunosuppressive drug therapy at the time of presentation was associated with a lower risk of development of hypotony (aHR, 0.02; P = .002). CONCLUSIONS: Hypotony affects a small but significant proportion of patients with JIA-associated uveitis and is associated with signs of active and severe uveitis. Immunosuppression was associated with significantly lower risk of hypotony, suggesting that aggressive control of the inflammation may reduce risk of hypotony in JIA-associated uveitis.

Clinical features and incidence rates of ocular complications in patients with ocular syphilis.

PURPOSE: To describe the clinical outcomes of ocular syphilis. DESIGN: Retrospective chart review. METHODS: The charts of patients with ocular syphilis (regardless of human immunodeficiency virus [HIV] status) seen in a uveitis referral center between 1984 and 2014 were reviewed. RESULTS: The study included 35 patients (61 eyes). Panuveitis was the most common type of ocular inflammation (28 eyes), independent of HIV status. Thirty-three of 35 patients received systemic antibiotics with 24 patients treated with intravenous (IV) penicillin only. When compared to the HIV-positive patients, HIV-negative patients with ocular syphilis were older (P < .001), were more likely to be female (P = .004), and had poorer visual acuity at presentation (P = .01). During follow-up, the incidence rates of visual impairment were 0.29 per eye-year (EY; 95% confidence interval [CI]: 0.06/EY-0.86/EY) and 0.12/EY (95% CI: 0.01/EY-0.42/EY) among the HIV-negative and the HIV-positive patients, respectively. The incidence of blindness was 0.07/EY (95% CI: 0.009/EY-0.27/EY) and 0.06/EY (95% CI: 0.002/EY-0.35/EY) among the HIV-negative and the HIV-positive patients, respectively. Longer duration of uveitis prior to diagnosis and chorioretinitis in the macula at presentation were associated with ≥ 2 Snellen lines of visual loss (P < .01) and visual acuity loss to 20/50 or worse (P = .03) in HIV-negative patients, respectively. CONCLUSIONS: Syphilis is an uncommon cause of ocular inflammation in both HIV-negative and HIV-positive patients. Visual loss and ocular complications were common among HIV-negative patients even with systemic antibiotic treatment. Delay of diagnosis and chorioretinitis in the macula were associated with visual loss in these patients.

Spectral domain optical coherence tomography findings in acute syphilitic posterior placoid chorioretinitis.

BACKGROUND: We describe the spectral domain optical coherence tomography (SD-OCT) findings in three patients with acute syphilitic posterior placoid chorioretinitis (ASPPC). The SD-OCT images demonstrate the pathologic changes in ASPPC with a high level of anatomic detail and may provide information about the pathophysiology of the disease. FINDINGS: We report a series of three consecutive patients seen at the Wilmer Eye Institute in 2012 and 2013 who presented with clinical and laboratory findings consistent with a diagnosis of unilateral ASPPC. Two of the three patients had HIV co-infection with good immune recovery. SD-OCT images from their initial (pre-treatment) presentation demonstrated thickening and hyperreflective nodularity of the choroid-retinal pigment epithelium (RPE) complex, with focal disruption of the overlying photoreceptor inner segment-outer segment junction in the areas corresponding to the retinal lesions seen on clinical examination. These changes improved with intravenous antibiotic treatment over a 3-month period of follow-up. CONCLUSIONS: SD-OCT imaging in ASPPC demonstrates reversible, focal thickening, and nodularity of the RPE with disruption of the overlying photoreceptor inner segment-outer segment junction. We believe that these SD-OCT images support the concept that ASPPC involves an inflammatory process at the level of the choroid-RPE with resultant structural and functional changes in the retinal photoreceptors. Further study with OCT imaging may be helpful in better understanding this disease.

Tattoo-associated uveitis.

PURPOSE: To describe the clinical presentation of uveitis with coincident onset of raised and indurated tattooed skin. DESIGN: Case series. METHODS: Seven consecutive patients were evaluated at a tertiary ophthalmologic facility with coincident uveitis and cutaneous tattoo induration over an 18-month period. All subjects underwent complete ophthalmic examination and a focused systemic medical evaluation including serologic testing and imaging studies. Two participants underwent biopsy of their tattoos. The patients' clinical courses and responses to treatment over a follow-up period of 1-20 months are reported (mean follow-up = 9 months). Main outcome measures included degree of intraocular inflammation, ocular complications, visual acuity, clinically observable tattooed skin changes, and biopsy results. RESULTS: Five of 7 patients had bilateral nongranulomatous anterior uveitis: 4 with chronic and 1 with recurrent disease. The remaining 2 patients had bilateral chronic granulomatous panuveitis. Biopsies of raised and indurated tattoos were performed in 2 patients and demonstrated noncaseating granulomatous inflammation surrounding tattoo ink in the dermis. The skin changes resolved in all patients, with a faster response noted in those treated with high-dose oral prednisone for intraocular inflammation. Five patients subsequently experienced recurrent flares of intraocular inflammation in conjunction with the recurrence of raised and indurated tattoos. CONCLUSIONS: These cases represent a subset of patients in whom skin tattooing may have incited an immune response leading to simultaneous inflammation of the eyes and tattooed skin.

Necrotizing scleritis as a complication of cosmetic eye whitening procedure.

BACKGROUND: We report necrotizing scleritis as a serious complication of a cosmetic eye whitening procedure that involves the use of intraoperative and postoperative topical mitomycin C. FINDINGS: This is a single case report. A 59-year-old Caucasian male with a history of blepharitis status post uncomplicated LASIK refractive surgery reported chronic conjunctival hyperemia for 15 years prior to undergoing a cosmetic eye whitening procedure. He presented to our clinic 12 months after the cosmetic eye whitening procedure with progressive bilateral necrotizing scleritis and scleral calcification. CONCLUSIONS: Chronic conjunctival hyperemia may prompt patients to seek surgical correction with cosmetic eye whitening procedures. However, conjunctival hyperemia secondary to tear deficiency and evaporative dry eye may predispose to poor wound healing. Serious complications including necrotizing scleritis may result from cosmetic eye whitening procedures and the use of topical mitomycin C.