Bacillary Layer Detachment as a Presenting Feature of Ocular Sarcoidosis.

BACKGROUND AND OBJECTIVE: Bacillary layer detachment (BALAD) is a recently described finding on optical coherence tomography (OCT) that has been reported in a variety of uveitic and retinal diseases. To add to the growing literature on conditions associated with this finding, we report on the first cases of BALADs in ocular sarcoidosis. PATIENTS AND METHODS: Observational, retrospective chart review of patients with BALADs as a feature of sarcoid-associated uveitis. RESULTS: Three patients presented with blurry vision and bacillary layer detachments on OCT who were either known to have or found to have sarcoid-associated uveitis. All three patients had resolution of the BALAD and improvement in visual acuity with a combination of high-dose oral steroids and/or systemic immunosuppression. CONCLUSIONS: Patients presenting with BALAD should be assessed for sarcoidosis, if appropriate, given the systemic implications of this diagnosis. [Ophthalmic Surg Lasers Imaging Retina 2023;54:686-690.].

Use of Immunosuppression and the Risk of Subsequent Overall or Cancer Mortality.

PURPOSE: To determine the incidence of all-cause and cancer mortality (CM) in association with immunosuppression. DESIGN: Retrospective cohort study at ocular inflammatory disease (OID) subspecialty centers. We harvested exposure and covariate data retrospectively from clinic inception (earliest in 1979) through 2010 inclusive. Then we ascertained overall and cancer-specific mortalities by National Death Index linkage. We constructed separate Cox models to evaluate overall and CM for each class of immunosuppressant and for each individual immunosuppressant compared with person-time unexposed to any immunosuppression. PARTICIPANTS: Patients with noninfectious OID, excluding those with human immunodeficiency infection or preexisting cancer. METHODS: Tumor necrosis factor (TNF) inhibitors (mostly infliximab, adalimumab, and etanercept); antimetabolites (methotrexate, mycophenolate mofetil, azathioprine); calcineurin inhibitors (cyclosporine); and alkylating agents (cyclophosphamide) were given when clinically indicated in this noninterventional cohort study. MAIN OUTCOME MEASURES: Overall mortality and CM. RESULTS: Over 187 151 person-years (median follow-up 10.0 years), during which 15 938 patients were at risk for mortality, we observed 1970 deaths, 435 due to cancer. Both patients unexposed to immunosuppressants (standardized mortality ratio [SMR] = 0.95, 95% confidence interval [CI], 0.90-1.01) and those exposed to immunosuppressants but free of systemic inflammatory diseases (SIDs) (SMR = 1.04, 95% CI, 0.95-1.14) had similar mortality risk to the US population. Comparing patients exposed to TNF inhibitors, antimetabolites, calcineurin inhibitors, and alkylating agents with patients not exposed to any of these, we found that overall mortality (adjusted hazard ratio [aHR] = 0.88, 0.89, 0.90, 1.11) and CM (aHR = 1.25, 0.89, 0.86, 1.23) were not significantly increased. These results were stable in sensitivity analyses whether excluding or including patients with SID, across 0-, 3-, or 5-year lags and across quartiles of immunosuppressant dose and duration. CONCLUSIONS: Our results, in a cohort where the indication for treatment was proven unassociated with mortality risk, found that commonly used immunosuppressants-especially the antimetabolites methotrexate, mycophenolate mofetil, and azathioprine; the TNF inhibitors adalimumab and infliximab, and cyclosporine-were not associated with increased overall and CM over a median cohort follow-up of 10.0 years. These results suggest the safety of these agents with respect to overall and CM for patients treated with immunosuppression for a wide range of inflammatory diseases. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

A United States expert consensus to standardise definitions, follow-up, and treatment targets for extra-intestinal manifestations in inflammatory bowel disease.

BACKGROUND AND AIMS: Extra-intestinal manifestations (EIMs) are a common complication of inflammatory bowel diseases (IBD), affecting up to half of the patients. Despite their high prevalence, information on standardised definitions, diagnostic strategies, and treatment targets is limited. METHODS: As a starting point for a national EIM study network, an interdisciplinary expert panel of 12 gastroenterologists, 4 rheumatologists, 3 ophthalmologists, 6 dermatologists, and 4 patient representatives was assembled. Modified Delphi consensus methodology was used. Fifty-four candidate items were derived from the literature review and expert opinion focusing on five major EIMs (erythema nodosum, pyoderma gangrenosum, uveitis, peripheral arthritis, and axial arthritis) were rated in three voting rounds. RESULTS: For use in a clinical practice setting and as part of the creation of a prospective registry of patients with EIMs, the panel developed definitions for erythema nodosum, pyoderma gangrenosum, uveitis, peripheral arthritis, and axial arthritis; identified the appropriate and optimal subspecialists to diagnose and manage each; provided methods to monitor disease course; offered guidance regarding monitoring intervals; and defined resolution and recurrence. CONCLUSIONS: Consensus criteria for appropriate and optimal means of diagnosing and monitoring five EIMs have been developed as a starting point to inform clinical practice and future trial design. Key findings include straightforward diagnostic criteria, guidance regarding who can appropriately and optimally diagnose each, and monitoring options that include patient and physician-reported outcomes. These findings will be used in a national multicenter study network to optimise the management of EIMs.

Retinal Vasculitis Associated With Dermatomyositis Treated With Intravenous Steroids.

Purpose: This work aims to present treatment and long-term follow-up of a 31-year-old woman with dermatomyositis who presented with hemorrhagic retinal vasculitis and macular edema. Methods: A retrospective case report is presented. Results: A 31-year-old woman with dermatomyositis treated with systemic immunosuppression was evaluated for acute, reduced vision. Best-corrected visual acuity was hand motion in the right eye and 20/200 in the left eye. Fundus examination revealed diffuse intraretinal hemorrhages, cotton-wool spots, and vascular sheathing with a frosted branch angiitis-like appearance. Optical coherence tomography revealed significant macular edema and subretinal fluid that quickly resolved after admission and treatment with intravenous steroids. Multimodal imaging at 7-year follow-up disclosed long-term sequelae including peripheral nonperfusion and retinal neovascularization. Conclusions: When vasculitis associated with dermatomyositis is treated aggressively with intravenous steroids at initial presentation, good visual acuity outcomes can be achieved, but long-term consequences of retinal nonperfusion and neovascularization persist.

Ocular Syphilis With Phlebitis and Paravenous Pigmentary Retinopathy.

Purpose: Ocular syphilis can present as a wide variety of clinical phenotypes, among them panuveitis with vasculitis. Primary retinal phlebitis with resulting paravenous atrophy and pigmentary retinal degeneration is a rare presentation. Methods: A 53-year-old man presented with a 1-year history of bilateral blurry vision. Physical examination demonstrated bilateral anterior chamber and vitreous cell with vitreous haze, hyperemic optic nerves, and atrophic-appearing retina. The left eye demonstrated a nasal area of perivenular vascular sheathing with adjacent retinal whitening. Ancillary testing demonstrated predominantly perivenular leakage involvement. Results: Uveitic workup was positive for syphilis and HIV. The patient was treated with antiretroviral therapy and intravenous penicillin G. He developed progressive paravenous pigmentary changes and atrophy. Conclusions: Syphilis can present with a wide variety of phenotypic manifestations and should also be considered in patients presenting with acute retinal phlebitis or paravenous atrophy in long-standing cases.

A CASE REPORT OF CHRONIC ENDOPHTHALMITIS SECONDARY TO AQUAMICROBIUMterrae.

PURPOSE: Chronic postoperative endophthalmitis is a known complication of cataract surgery. Here, we report a case of chronic endophthalmitis following uncomplicated cataract surgery secondary to the atypical bacterium Aquamicrobium terrae . METHODS: Retrospective case report of a single patient with endophthalmitis secondary to A. terrae. RESULTS: A 61-year-old healthy patient presented with recurrent iridocyclitis following uncomplicated cataract surgery. Repeated attempts to taper off topical steroid eyedrops were unsuccessful. Anterior chamber paracentesis cultures and vitrectomy cultures grew the same gram-negative bacillus, A. terrae , on six different occasions. The patient was treated initially with a series of intravitreal ceftazidime injections and adjuvant oral minocycline with recurrence of inflammation necessitating pars plana vitrectomy with intraocular lens removal and total capsulectomy. Inflammatory episodes recurred until residual capsule fragments were ablated using endoscopic vitrectomy. Since ablation over 18 months ago, no further recurrences have occurred and the patient has excellent visual outcome. CONCLUSION: A. terrae is a gram-negative bacillus recovered from polluted soil. This is the first case of postoperative endophthalmitis secondary to A. terrae and the first description of human infection caused by this newly identified microbe.

Patient-Reported Complications after Intravitreal Injection and Their Predictive Factors.

PURPOSE: The intravitreal injection (IVI) of pharmacologic agents is the most commonly performed ocular procedure and is associated with a host of complications. Most IVI-related complications data are derived from randomized controlled clinical trials, which report a high adverse event rate. The nature of these protocol-driven trials limit their applicability to the diverse circumstances seen in routine clinical practice. The goal of this study was to determine the prevalence of patient-reported IVI-related complications, their risk factors, and the manner in which patients sought treatment at a tertiary eye care center. DESIGN: Retrospective, institutional review board-approved study. PARTICIPANTS: Forty-four thousand seven hundred thirty-four injections in 5318 unique patients at the Cleveland Clinic Cole Eye Institute from 2012 through 2016. METHODS: Intravitreal injection. MAIN OUTCOME MEASURES: Complication occurrence within 15 days of injection. RESULTS: From 2012 through 2016, a total of 44734 injections were performed in 5318 unique patients. Overall, complication rates were low, representing 1.9% of all injections, with 1031 unique complications in 685 patients (12.9%). The most common minor complications, or those not requiring intervention, were irritation (n = 312) and subconjunctival hemorrhage (n = 284). The most common serious complications, or those requiring intervention, were corneal abrasion (n = 46) and iritis (n = 31). Most complications (66%) were managed adequately by a telephone or Epic (Epic Systems Corp., Verona, WI) electronic message encounter only. Importantly, no injection protocol parameter, such as type of anesthesia, preparation, or post-injection medication, increased the risk of a complication. However, a patient's gender, age, number of previous injections, and provider strongly influenced the risk of patient-reported complications. CONCLUSIONS: Overall, complication rates seen in routine clinical practice were low compared with clinical trial reporting. Providers should feel confident in the safety and administration of IVI during times when follow-up office visits and resources may be limited. When performing an IVI, factors such as a patient's gender, age, number of previous injections, and provider must be taken into account to ensure the best possible outcomes.

Recurrence Rates of Inflammation after Switching from the Originator Infliximab to Biosimilar Infliximab-abda for Noninfectious Uveitis.

PURPOSE: To describe the frequency of ocular flares in patients with noninfectious uveitis who were switched from the originator infliximab to a biosimilar infliximab. DESIGN: Retrospective case series. METHODS: All patients with noninfectious uveitis who were switched from the originator infliximab to biosimilar infliximab-abda for nonmedical reasons were reviewed. Patients were excluded if they had less than 3 months of follow-up on either drug. Data included patient demographics, infliximab dosage information, additional immunosuppression medications, and numbers of and times to flares. The main study outcome was frequency of flares, defined as new or worsening inflammatory activity on examination or imaging. RESULTS: A total of 17 patients met the inclusion criteria. There were no statistical differences between the duration of follow-up while on the originator and the duration while on the biosimilar infliximab (12.0 vs. 10.1 months, respectively; P = .307). Patients experienced more flares per person-years after switching to infliximab-abda (.92), than on the originator infliximab (0.19; P = .028). Four of the 6 patients (66.7%) who experienced flare after switching to infliximab-abda did so within 90 days. Only 1 patient had flares while on originator infliximab went on to develop a single flare on infliximab-abda. The final normalized dosage for patients who flared and remained on infliximab-abda (1.301 mg/kg/week) was higher than that for those who did not flare (1.186 mg/kg/week) but was not statistically significant (P = .417). CONCLUSIONS: Patients who were switched to biosimilar infliximab-abda experience more flares than when previously treated with the originator infliximab. Providers should closely observe patients who switch to biosimilar infliximab, especially within the first 90 days. Patients who do have flares after switching may achieve quiescence with increased biosimilar dosage.

Clinical and Multimodal Imaging Findings in Disseminated Mycobacterium Chimaera.

PURPOSE: To characterize the ophthalmic clinical and multimodal imaging findings of disseminated Mycobacterium chimaera infection after cardiothoracic surgery. DESIGN: Observational case series. PARTICIPANTS: Four patients (8 eyes) with disseminated M. chimaera infection. METHODS: Patients were evaluated with biomicroscopy, OCT and OCT angiography, fundus autofluorescence, and fluorescein and indocyanine green angiography. MAIN OUTCOME MEASURES: Clinical and multimodal imaging findings of patients with disseminated M. chimaera infection. RESULTS: All 4 patients were white men with a mean age of 65.5 years (range, 60-75 years) who had aortic valve or root infection, or both, with M. chimaera diagnosed by culture, gene sequencing, or both. All 4 patients demonstrated bilateral choroidal lesions on funduscopy and evidence of osteomyelitis by imaging, culture analysis, or both at the time of ocular diagnosis. Indocyanine green and OCT angiography revealed numerous additional subclinical choroidal lesions and were used to track disease response to therapy. Fluorescein angiography and fundus autofluorescence were useful in determining lesion age and activity. All patients were treated with 3- or 4-drug antimycobacterial therapy. Three underwent revision of cardiothoracic surgery with removal of infected graft. One patient went on to demonstrate progressive ocular disease that was noted before each of his surgical revisions. Two patients showed improvement in ocular and systemic disease, however one of them developed a choroidal neovascular membrane. The final patient was a single encounter whose clinical and imaging findings showed longstanding inactive disease. CONCLUSIONS: Ophthalmologists should be aware of the systemic and ocular findings of this rare life-threatening disease. Multimodal imaging is useful in corroborating a diagnosis of ocular M. chimaera and particularly in evaluating patient response to therapy, because choroidal activity seems to mimic systemic activity. Treating physicians should be aware of the co-occurrence of choroiditis and osteomyelitis. Choroidal neovascular membrane can also be a late complication of this disease.

Acute retinal necrosis following recombinant subunit varicella-zoster virus vaccine.

PURPOSE: Previously, secondary prevention of herpes zoster required live-attenuated vaccination, which is contraindicated in immunocompromised populations. More recently, a recombinant subunit vaccine (Shingrix, GlaxoSmithKline, Research Triangle Park, North Carolina) was approved by the Food and Drug Administration. Iatrogenic varicella-zoster virus (VZV) infection is theoretically impossible as it does not contain a live virus. We present a case of acute retinal necrosis (ARN) and disseminated zoster after receiving the recombinant subunit vaccine. OBSERVATIONS: A 65-year-old woman with past medical history of multiple myeloma treated with a previous autologous hematopoietic stem cell transplant and now with daratumumab and pomalidomide developed disseminated zoster and subsequently acute retinal necrosis weeks after receiving the zoster subunit vaccine. Molecular testing confirmed the presence of VZV, and the absence of herpes simplex virus, cytomegalovirus, and toxoplasmosis. The VZV was found to be genotypically wildtype and not related to the Oka strain used in the live-attenuated zoster vaccine. She was treated with systemic valacyclovir and intravitreal foscarnet. CONCLUSIONS AND IMPORTANCE: This is the first report of VZV infection following the zoster subunit vaccine. The Advisory Committee on Immunization Practices (ACIP) has recommended the recombinant subunit vaccine over the live-attenuated vaccine due to its superior efficacy. The off-label use of the subunit vaccine in immunocompromised populations has been supported up to this point by studies demonstrating its relative safety. Though post-vaccination VZV infection or reactivation appears to be rare, clinicians should be aware of this potential complication to the recombinant subunit vaccine.

Visual recovery and vascular reperfusion after vaso-occlusive retinopathy from anti-phospholipid syndrome associated with systemic lupus erythematosus.

PURPOSE: To report a case of visual recovery and vascular reperfusion after vaso-occlusive retinopathy from anti-phospholipid syndrome associated with systemic lupus erythematosus. OBSERVATIONS: A 15-year-old boy with a known diagnosis of systemic lupus erythematosus and a clinically significant anti-phospholipid panel presented with sudden vision loss in the left eye. Examination and ocular imaging revealed signs of vaso-occlusive retinopathy. The patient was immediately started on high dose intravenous steroids, followed by mycophenolate mofetil. He remained on aspirin. After showing no improvement in retinal arteriole and capillary perfusion he was started on therapeutic anti-coagulation with enoxaparin. He regained 20/20 vision. Intravenous fluorescein angiography demonstrated reperfusion of retinal arterioles. Optical coherence tomography angiography showed return of flow in the capillary networks. CONCLUSIONS: We present a case of vaso-occlusive retinopathy in a patient with known systemic lupus erythematosus and a clinically significant anti-phospholipid panel, thus meeting criteria for anti-phospholipid syndrome. He was treated with intravenous methylprednisolone, mycophenolate motefil, aspirin, and enoxaparin. The patient not only had great recovery of visual acuity, but also demonstrated reperfusion of arterioles and reconstitution of flow in the retinal capillary network. These findings suggest that the vaso-occlusive disease is reversible if the diagnosis is made promptly and intensive therapy is initiated. IMPORTANCE: Currently there are no reported cases of vaso-occlusive retinopathy from APLS and SLE with visual recovery, reperfusion, and return of capillary flow.

Long-term Outcomes of Glaucoma Drainage Implants in Uveitic Eyes With Fluocinolone Acetonide Implants.

PRECIS: In eyes with uveitis and a history of fluocinolone acetonide (FA) implantation, glaucoma drainage implants (GDIs) provides excellent long-term control of intraocular pressure (IOP). PURPOSE: The purpose of this study was to evaluate the long-term control of IOP achieved by GDIs in uveitic eyes with glaucoma and a FA implant. PATIENTS AND METHODS: Retrospective case series of 56 eyes from 37 patients with a history of noninfectious posterior uveitis who underwent both FA implantation and GDI surgery at the Cleveland Clinic Cole Eye Institute between April 2001 and April 2017. Patients were excluded if they did not undergo FA implantation before, or concurrently with, a GDI. Data was collected up to 10 years after GDI surgery. Outcome measures included IOP, number of IOP-lowering medications, and surgical success rate at each timepoint. Success was defined as IOP between 6 and 21 mm Hg at the most recent follow-up visit, without need for GDI removal or additional IOP-lowering surgeries, or loss of light perception. RESULTS: The mean follow-up time was 71.0 months (median 72.0 mo). IOP was reduced by a mean of 52.9% (range: 43.1% to 62.3%) and the number of IOP-lowering medications decreased by a mean of 69.0% (range: 56.3% to 93.8%) between 1 and 10 years postoperatively. The percentage of eyes with IOP <21 mm Hg was 32.1% at baseline and ranged between 76.8% and 100% at follow-up. The percentage of eyes with IOP <18 mm Hg was 21.4% at baseline and ranged between 67.9% and 100% at follow-up. The surgical success rate was 71.4% at 10 years. CONCLUSIONS: In eyes with uveitis that undergo FA implantation, concurrent or subsequent GDI surgery offers excellent long-term control of IOP. Postoperative IOP and number of IOP-lowering medications dropped considerably and remained stable.

Ultra-widefield Fluorescein Angiography and OCT Findings in Tubulointerstitial Nephritis and Uveitis Syndrome.

PURPOSE: To report the spectrum of posterior segment findings in tubulointerstitial nephritis and uveitis syndrome (TINU) and discuss the abnormalities that can be seen on imaging. DESIGN: Retrospective, consecutive case series. PARTICIPANTS: Patients with TINU and posterior segment manifestations on examination or imaging. METHODS: Patients with elevated urine beta-2 microglobulin (Uß2M) and a diagnosis of TINU were included if they were evaluated at the Cole Eye Institute and did not have alternative etiologies for uveitis. Electronic medical records were reviewed for abnormal findings on ultra-widefield fluorescein angiography (UWFFA) and OCT. MAIN OUTCOME MEASURES: Presence of peripheral vascular leakage, optic disc leakage, chorioretinal lesions, or leakage within the macula on UWFFA. For OCT findings, patients were categorized as having intraretinal fluid, epiretinal membrane, or optic nerve edema. RESULTS: Twenty eyes from 10 patients (6 female, 4 male) with a bimodal age distribution (10-46 years and 77-83 years) were included. Eighteen of 20 eyes (90%) underwent UWFFA; 13 eyes demonstrated the presence of peripheral vascular leakage, 5 eyes showed optic disc leakage, and 6 eyes had leakage within the macula. All eyes underwent OCT imaging; 7 eyes demonstrated intraretinal fluid, 4 eyes were found to have an epiretinal membrane, and 1 eye had optic nerve edema. Six eyes lacked anterior uveitis on initial or follow-up examination but had abnormal findings on UWFFA or OCT. CONCLUSIONS: Tubulointerstitial nephritis and uveitis syndrome is under-recognized in the clinical setting. It can manifest in patients of all ages, and posterior segment involvement is not uncommon. Abnormalities may be seen on posterior segment examination or imaging in the absence of anterior segment inflammation. Tubulointerstitial nephritis and uveitis syndrome should be considered in the differential diagnosis for patients presenting with bilateral uveitis without evidence of infection or other clear etiology for intraocular inflammation.

A Case Report of Endogenous Endophthalmitis in the Setting of Nocardia kroppenstedtii Infection.

A woman with a past medical history of radiation and chemotherapy for multiple malignancies presented with bilateral, multifocal, elevated chorioretinal lesions associated with vitritis. The differential diagnosis included endogenous endophthalmitis and metastatic disease. Her extensive medical history and ill appearance at presentation in concert with vitritis and subretinal lesions was concerning for endogenous endophthalmitis. Although her vitreous sample was negative, her blood cultures were positive for Nocardia kropenstedtii and Nocardia farcinica. She was started on broad-spectrum antibiotic coverage but had brain lesions on presentation and ultimately passed away. This is the first reported case of Nocardia endophthalmitis in the setting of systemic N. kroppenstedtii infection. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:53-55.].

ULTRA-WIDEFIELD MULTIMODAL IMAGING OF PRIMARY VITREORETINAL LYMPHOMA.

PURPOSE: To determine the features of primary vitreoretinal lymphoma on multimodal ultra-widefield imaging and correlate these findings to clinical outcomes. METHODS: We report a retrospective, observational case series of 43 eyes of 23 patients with biopsy-proven B-cell primary vitreoretinal lymphoma. Fundus photography, fluorescein angiography (FA), optical coherence tomography, fundus autofluorescence, and indocyanine green angiography images were reviewed. Medical records were assessed for the central nervous system involvement and visual acuity outcomes at 6 and 12 months after presentation. RESULTS: Common fundus photography findings were sub-retinal pigment epithelium lesions and vitritis alone. Common ultra-widefield FA findings were vascular leakage and scleral staining. Retinal optical coherence tomography features overlying sub-retinal pigment epithelium lesions or within the macula predicted fluorescence patterns. The presence of retinal fluid or disorganization associated with hyperfluorescence and late leakage. Normal retinal structures associated with hypofluorescence of sub-retinal pigment epithelium lesions or macular leopard spotting on FA and fundus autofluorescence. Peripheral abnormalities noted on ultra-widefield fundus photography, FA, and indocyanine green angiography were more frequent than posterior pole abnormalities. No imaging characteristics predicted time to the central nervous system progression. CONCLUSION: Ultra-widefield imaging was more informative than posterior pole imaging in fundus photography, FA, and indocyanine green angiography. Common findings on multimodal ultra-widefield imaging may lead to early diagnostic vitrectomy and may reduce the delay in primary vitreoretinal lymphoma diagnosis.

Characteristics of inflammatory eye disease associated with hidradenitis suppurativa.

OBJECTIVE: Hidradenitis suppurativa (HS) is an inflammatory skin condition that can cause profound morbidity. Patients can present with recurrent nodules, sinus tract formation, abscesses, and/or scarring, mainly affecting the intertriginous areas. Case reports have documented the coexistence of HS and inflammatory eye disease (IED). Herein, we aimed to assess the types of IED associated with HS and the outcomes of IED treatment in patients with HS. METHODS: All the cases with a diagnosis of HS and any IED were identified. Patients with episcleritis and sicca were excluded, and only those with chronic IED were included. An independent ophthalmologist reviewed notes to ensure accuracy of IED diagnosis. Demographics, IED patterns, comorbidities, treatments, and outcomes were retrieved from patient charts. RESULTS: Twenty patients [16 females (80%); 12 African Americans (60%)] were included in this study after the exclusion of 436 cases due to lack of data. The mean age at the diagnosis of HS and IED was 42.1 and 43.6 years, respectively. Thirteen patients had uveitis (65%), six had scleritis (30%), and one had peripheral ulcerative keratitis. Thirteen out of 20 patients (65%) had multiple autoimmune and/or inflammatory comorbidities, including inflammatory bowel disease, rheumatoid arthritis, ankylosing spondylitis, multiple sclerosis, and lupus. Seven patients (35%) did not have any comorbid inflammatory/autoimmune conditions. CONCLUSION: One-third of the patients with HS and IED did not have any autoimmune or inflammatory comorbidity that could explain the eye involvement. The potential association between HS and IED might be a manifestation of a common immune dysregulation phenomenon. Furthermore, the management of IED required an escalation of therapy to systemic immunosuppressive agents in 70% of patients with HS.

Treatment of Acute Zonal Occult Outer Retinopathy With Intravitreal Steroids.

BACKGROUND AND OBJECTIVE: To report on the use of intravitreal steroids in the management of acute zonal occult outer retinopathy (AZOOR), a rare disorder affecting the outer retina. PATIENTS AND METHODS: Retrospective case series of nine eyes of five patients with AZOOR who received intravitreal triamcinolone acetonide (IVTA), dexamethasone intravitreal implant, and/or fluocinolone acetonide implant. Treatment response was determined by reported symptoms and multimodal imaging findings. Patients were observed for at least 1 year following intravitreal steroid treatment (range: 14 months to 63 months). RESULTS: Seven eyes received IVTA, six eyes received the dexamethasone intravitreal implant, and one eye received the fluocinolone acetonide implant. All patients experienced disease stability or improvement based on symptomatic response and multimodal imaging findings after intravitreal steroids. One eye developed central serous retinopathy, and another eye a choroidal neovascular membrane. Five of nine eyes experienced ocular hypertension. All phakic eyes developed cataracts. CONCLUSION: Intravitreal steroids effectively achieved disease stability in patients with AZOOR. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:504-509.].

Treatment Duration and Side Effect Profile of Long-Term Use of Intravitreal Preservative-Free Triamcinolone Acetonide in Uveitis.

PURPOSE: Noninfectious uveitis has been treated historically with corticosteroid therapy in varying doses and routes. Triesence, a preservative-free sterile formulation of triamcinolone acetonide, has been used in a wide spectrum of ocular pathologies, but there have been few large studies validating its dosing or detailing long-term side effects in uveitic disease. The primary aim of this study was to describe the relative duration of action and side effects of 2 doses of preservative-free intravitreal triamcinolone acetonide (PF-IVTA) in uveitis. DESIGN: Retrospective, comparative consecutive case series. METHODS: Charts of all patients receiving PF-IVTA (2 mg or 4 mg) in a defined time period (2012-2014) at the Cole Eye Institute were examined for patient demographics, time to treatment failure (TTF), use of systemic immunosuppression, use of intraocular pressure-lowering therapies, date of cataract surgery and glaucoma filtration surgery, and adverse events. RESULTS: The final data set examined 514 injections in 214 eyes. Mean duration of follow-up was 1.5 years. There was similar demographic distribution between eyes that received 2 mg PF-IVTA only and eyes that received a combination of 4 + 2 mg PF-IVTA. No statistically significant difference in TTF between injection dosages was observed. There was a higher incidence of glaucoma filtering surgery and cataract surgery in eyes that received 4 + 2 mg PF-IVTA as well as a shorter time to glaucoma surgery, when compared to eyes that received 2 mg PF-IVTA alone. CONCLUSIONS: This retrospective study supports that 2 mg PF-IVTA displayed noninferior treatment duration to 4 mg PF-IVTA, and may carry a significantly lower side-effect profile of cataract development and glaucoma filtering surgery.

Herpes zoster keratitis development after acute retinal necrosis.

PURPOSE: To report a case of herpes zoster keratitis in a patient undergoing treatment for herpetic acute retinal necrosis. METHODS: Case report. RESULTS: A 71 year old male presented with acute retinal necrosis of the left eye due to herpes zoster and was treated with intravitreal foscarnet and oral valcyclovir. He developed a retinal detachment and underwent surgical repair. After four weeks, he developed an ipsilateral herpetic zoster keratitis demonstrated by Rose-Bengal staining that was responsive to topical ganciclovir gel. CONCLUSIONS: This case report describes the unusual development of herpes zoster keratitis after the development of unilateral acute retinal necrosis (ARN) in a patient on antiviral treatment.