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Purpose: The purpose of this study was to report an unusual case of pseudoxanthoma elasticum presenting with an inflammatory phenotype associated with atypical and rapidly progressive subretinal fibrosis. Methods: This was an observational case report. Results: A patient with a history of pseudoxanthoma elasticum presented with rapidly progressive subretinal fibrosis, particularly in the left eye, over the course of one year. The patient was noted at presentation to have intraocular inflammation, outer retinal attenuation, multifocal choroiditis-like lesions, and intraretinal fluid (in the absence of obvious clinical or angiographic signs of exudative choroidal neovascular membranes). An ocular inflammatory phenotype was diagnosed, and the patient was treated with a combination of local steroids and systemic corticosteroids/immunomodulatory agents. After initiation of these agents, the patient demonstrated functional and structural improvement, with partial outer retinal reconstitution, decreased intraretinal fluid, and lack of further progression of subretinal fibrosis. Conclusion: This report describes an inflammatory phenotype of pseudoxanthoma elasticum associated with severe and atypical subretinal fibrosis. This case expands upon the currently known spectrum of inflammatory phenotypes associated with pseudoxanthoma elasticum. Treatment with corticosteroids or immunomodulatory treatment should be considered in similar cases. [ABSTRACT FROM AUTHOR]

This case series describes two patients who presented at a tertiary clinical center (NEI) with tattoo-related posterior segment uveitis with Vogt–Koyanagi–Harada features. These findings imply that reactions secondary to tattoo ink may have a causal relationship to the onset of posterior segment inflammation in certain predisposed individuals. Purpose: To present two patients with treatment-naïve posterior segment uveitis with Vogt–Koyanagi–Harada-like features associated with tattoo-related inflammatory skin changes. Methods: Retrospective report of two cases. Results: Using clinical history and multimodal imaging, a diagnosis of posterior segment uveitis with Vogt–Koyanagi–Harada-like features was made in association with tattoo skin changes in both patients. Conclusion: Physicians should be aware that tattoo-associated posterior segment uveitis with Vogt–Koyanagi–Harada-like features can occur. [ABSTRACT FROM AUTHOR]

Purpose: To describe Waldenström's macroglobulinemia (WM) as a masquerade syndrome. Methods: Case report. Results: A 59-year-old human leukocyte antigen (HLA)-A29-negative white male presented with a 7-year history of floaters, progressive vision loss, and poor contrast sensitivity along with choroidal lesions suggestive of birdshot chorioretinopathy (BCR). Fluorescein angiography, fundus autofluorescence, and indocyanine green angiography showed multiple areas of hyperfluorescence and hypofluorescence. Electroretinography showed reduced cone and rod responses. Comprehensive workup for infectious, paraneoplastic, and other causes including sarcoidosis was negative at the time. The patient was treated with multiple immunomodulatory agents without any significant improvement. Two years after initial presentation, the patient developed normocytic anemia and high levels of inflammatory markers. Further workup yielded a diagnosis of WM. His choroidal lesions were significantly reduced after treatment with rituximab and bendamustine. Conclusion: We report a case of WM masquerading as BCR. Other indolent diseases should be considered in the differential diagnosis for HLA-A29-negative patients presenting with birdshot-like lesions, especially if they are clinically unresponsive to multiple systemic immunosuppressive agents. [ABSTRACT FROM AUTHOR]

The article evaluates the effectiveness of fundus autofluorescence (FAF) in diagnosing and managing uveitis. It explains the principles of FAF and describes the image features observed for uveitis. It highlights the ability of FAF in detecting serpiginous choroidopathy, acute posterior multifocal placoid pigment epitheliopathy and multiple evanescent white-dot syndrome.

Purpose: To evaluate the surgical outcome and adverse events associated with implantation of the implantable contact lens (ICL) for the correction of high myopia or high hyperopia. Setting: Helsinki University Eye Hospital, Helsinki, Finland. Methods: In this nonrandomized prospective clinical trial, the ICL V4 was implanted in 26 eyes of 13 patients with normal enrollment criteria (younger than 45 years, a clinically clear crystalline lens) (Group 1) and the V2, V3, or V4 were implanted in 38 eyes of 22 patients with heterogeneous criteria (older than 45 years or opacities in the crystalline lens) (Group 2). The main outcome measures were lens opacity classification system (LOCS II) assessment and transparency analysis of the lens. Results: In Group 1, microdensitometric assessment showed no decrease in crystalline lens transparency and no statistically significant increase in crystalline lens density at any follow-up examination (P>.05). The incidence of anterior subcapsular (AS) opacities was 7.7%, and no eye developed clinically significant cataract during the mean follow-up of 13.2 months ± 12.3 (SD). No myopic eye lost a line of best corrected visual acuity (BCVA); 50.0% of hyperopic eyes lost 1 line of BCVA. Sixty-eight percent of myopic eyes and 75.0% of hyperopic eyes were within ±1.0 diopter of the predicted refraction. In Group 2, the incidence of AS opacities was 47.4% and 10 eyes (26.3%) developed clinically significant cataract during the mean follow-up of 30.9 ± 18.0 months. The visual and refractive results were similar to those in Group 1 at 1 month, but due to progressive AS opacities in the crystalline lens, 23.4% of eyes lost BCVA lines during the follow-up. Intraoperative complications were few and insignificant. Conclusions: Implantation of the ICL for correction of high myopia or high hyperopia appears to be a safe procedure with good visual and refractive results immediately. There was no decrease in the transparency or increase in the density of the crystalline lens with the latest generation of the ICL device in the normal group, indicating enhanced safety. Progressive lens opacification in the heterogeneous group shows there are risk factors for cataract formation; ie, type V3 ICL, preexisting lens opacities, and older patient age. Anterior subcapsular opacities are most likely the result of trauma. [Copyright &y& Elsevier]

Purpose: To evaluate safety and efficacy of Array® SA40N multifocal intraocular lens (IOL) (AMO) implantation in cataract surgery. Setting: Helsinki University Eye Hospital, Helsinki, Finland. Methods: In this prospective randomized comparative trial, 80 patients scheduled for cataract surgery were selected based on preoperative counseling and randomized to have multifocal or monofocal IOL implantation. Fifty-three eyes of 35 patients received a multifocal IOL and 67 eyes of 40 patients, a monofocal IOL. The incidence of complications and visual outcome in the multifocal and monofocal IOL groups were compared. Quality of vision was measured by comparing the severity of visual symptoms (glare, halos, and cataract symptoms score), changes in functional impairment measured by a 7-item visual function test (VF-7), changes in global measures of vision (trouble and satisfaction with vision), and range of accommodation and contrast sensitivity. Results: Intraoperative and postoperative complications and adverse events were few and required no further surgical intervention. Both distance and near visual acuities were significantly better in the multifocal group than in the monofocal group; the difference was most prominent in distance corrected near acuity (P<.001). Thirty-five eyes (67.3%) in the multifocal group and 10 eyes (14.9%) in the monofocal group achieved a distance corrected near acuity of J6 (20/40) or better; 30 eyes (56.6%) and 19 eyes (28.4%), respectively, achieved a best corrected distance acuity of 20/20 or better. Glare symptoms decreased postoperatively in both groups but were slightly more common in the multifocal group. In contrast, halos were significantly more common at 1 month in the multifocal group (P<.001). Contrast sensitivity values were slightly lower with multifocal IOLs at almost all spatial frequencies, but the difference was not significant. The change in the quality of life postoperatively, measured with the VF-7, was significant and identical in both groups. Conclusions: Pseudophakic eyes with multifocal IOLs had better distance and near acuity and range of accommodation than eyes with a monofocal IOL. Slightly lower contrast sensitivity and increased perception of halos by subjects with the multifocal IOL appear to be an acceptable compromise to enhanced near and distance vision. [Copyright &y& Elsevier]

Purpose: To investigate the utility of optical coherence tomography angiography (OCTA) for the detection of inflammatory choroidal neovascularization (iCNV) and monitoring their response to treatment., Methods: A retrospective review of patients with a diagnosis of uveitis and associated iCNV with active exudation was performed. Active iCNV was determined by spectral domain OCT and/or fluorescein angiogram. Spectral domain OCTA outer retina to choriocapillaris slabs was evaluated for the presence of iCNV. Follow-up OCTA images were qualitatively assessed to determine whether regression of iCNV occurred after treatment., Results: Thirteen eyes of 12 patients were included. The etiologies of uveitis include punctate inner choroidopathy (n = 4), multifocal choroiditis (n = 2), presumed sarcoid uveitis (n = 2), tuberculous choroiditis (n = 1), birdshot chorioretinopathy (n = 1), syphilitic uveitis (n = 1), serpiginous choroiditis (n = 1), and idiopathic panuveitis (n = 1). Inflammatory choroidal neovascularization was detected on en face OCTA in 10 of 13 eyes (76.9%). After iCNV treatment, en face OCTA demonstrated complete regression of iCNV in 5 of 10 eyes (50%), partial regression in 2 of 10 eyes (20%), and no regression in 3 of 10 eyes (30%)., Conclusions: Optical coherence tomography angiography is an effective modality for detecting iCNV and could provide detailed visualization regarding location, morphologic structure, and flow of the iCNV and its response to therapy.

Purpose: To describe patients with intermediate uveitis complicated by vasoproliferative tumors (VPTs)., Methods: Data were collected at seven Uveitis/Ocular Oncology centers on demographic, ophthalmic findings at baseline and at follow-up, and on imaging. The therapeutic intervention, final visual acuity, and duration of follow-up were recorded., Results: A total of 36 eyes from 34 patients (12 men, 22 women; mean age 35.3 ± 14.2 years) were included in this study. Visual acuity at presentation ranged from 20/40 to counting fingers. At the time of VPT diagnosis, intermediate uveitis was active in all eyes. The mean VPT thickness was 3.06 ± 0.86 mm. Local treatment to the VPT was provide in 22 eyes (61.1%) and no local treatment to the VPT in 14 eyes (38.9%). After the VPT was detected, systemic or local treatment for the inflammation was initiated and on follow-up FAs 94.4% of the eyes showed resolution of the vascular leakage. During follow-up of 35.8 months, the 22 VPTs treated locally had a reduction in the tumor thickness to 1.25 mm, whereas the 14 VPTs untreated remained stable (final mean tumor thickness 2.65 mm)., Conclusion: The presence of active intermediate uveitis accompanied by VPTs suggests the need for an aggressive uveitis treatment.

Purpose: To investigate whether wide-field multispectral scanning laser ophthalmoscopy could assist in determining depth of chorioretinal pathology in posterior uveitis., Methods: Cross-sectional retrospective review of patients with birdshot chorioretinopathy (BCR; 42 eyes of 21 patients) or active primary vitreoretinal lymphoma (PVRL; 18 eyes of 10 patients) who had multispectral wide-field scanning laser ophthalmoscopy (Optos) images. Images acquired with 532 nm and 635 nm lasers were analyzed separately using Optos V Vantage Pro Review software., Results: All 42 eyes with birdshot chorioretinopathy and 8/18 eyes with active primary vitreoretinal lymphoma had lesions on 635 nm imaging, while 26/42 of the birdshot chorioretinopathy eyes and 18/18 eyes with active primary vitreoretinal lymphoma had lesions on 532 nm imaging. The difference between the 2 groups on both 635 nm and 532 nm was statistically significant (P < 0.05)., Conclusion: Retinal and choroidal lesions in patients with posterior uveitis can be differentially visualized with Optos 532 nm and 635 nm lasers, respectively, allowing determination of depth of pathology.

Purpose: To report acute/subacute vision loss and paracentral scotomata in patients with idiopathic multifocal choroiditis/punctate inner choroidopathy due to large zones of acute photoreceptor attenuation surrounding the chorioretinal lesions., Methods: Multimodal imaging case series., Results: Six women and 2 men were included (mean age, 31.5 ± 5.8 years). Vision ranged from 20/20-1 to hand motion (mean, 20/364). Spectral domain optical coherence tomography demonstrated extensive attenuation of the external limiting membrane, ellipsoid and interdigitation zones, adjacent to the visible multifocal choroiditis/punctate inner choroidopathy lesions. The corresponding areas were hyperautofluorescent on fundus autofluorescence and were associated with corresponding visual field defects. Full-field electroretinogram (available in three cases) showed markedly decreased cone/rod response, and multifocal electroretinogram revealed reduced amplitudes and increased implicit times in two cases. Three patients received no treatment, the remaining were treated with oral corticosteroids (n = 4), oral acyclovir/valacyclovir (n = 2), intravitreal/posterior subtenon triamcinolone acetate (n = 3), and anti-vascular endothelial growth factor (n = 2). Visual recovery occurred in only three cases of whom two were treated. Varying morphological recovery was found in six cases, associated with decrease in hyperautofluorescence on fundus autofluorescence., Conclusion: Multifocal choroiditis/punctate inner choroidopathy can present with transient or permanent central photoreceptor attenuation/loss. This presentation is likely a variant of multifocal choroiditis/punctate inner choroidopathy with chorioretinal atrophy. Associated changes are best evaluated using multimodal imaging.

Purpose: To evaluate fundus autofluorescence (FAF) patterns in patients with primary intraocular (vitreoretinal) lymphoma., Methods: Records of all patients with primary intraocular lymphoma who underwent FAF imaging at the National Eye Institute were reviewed. Fundus autofluorescence patterns were evaluated with respect to clinical disease status and the findings on fluorescein angiography and spectral-domain optical coherence tomography., Results: There were 18 eyes (10 patients) with primary intraocular lymphoma that underwent FAF imaging. Abnormal autofluorescence in the form of granular hyperautofluorescence and hypoautofluorescence was seen in 11 eyes (61%), and blockage by mass lesion was seen in 2 eyes (11%). All eyes with granular pattern on FAF had active primary intraocular lymphoma at the time of imaging, but there were 5 eyes with unremarkable FAF, which were found to have active lymphoma. The most common pattern on fluorescein angiography was hypofluorescent round spots with a "leopard spot" appearance (43%). These hypofluorescent spots on fluorescein angiography correlated with hyperautofluorescent spots on FAF in 5 eyes (36%) (inversion of FAF). Nodular hyperreflective spots at the level of retinal pigment epithelium on optical coherence tomography were noted in 43% of eyes. The hyperautofluorescent spots on FAF correlated with nodular hyperreflective spots on optical coherence tomography in 6 eyes (43%)., Conclusion: Granularity on FAF was associated with active lymphoma in majority of the cases. An inversion of FAF (hyperautofluorescent spots on FAF corresponding to hypofluorescent spots on fluorescein angiography) was observed in less than half of the eyes.

Purpose of Review: Autoimmune retinopathy (AIR) is an immune-mediated disorder characterized by progressive visual loss, abnormal electroretinographic and visual field findings in the presence of circulating anti-retinal antibodies. This review highlights advances made toward understanding the pathophysiology, clinical manifestations, and trends in the management of AIR., Recent Findings: The pathophysiology of AIR is likely antibody-mediated. AIR serum autoantibodies are variable in their size and retinal tissue they target and can also be present in healthy controls and multiple autoimmune diseases. Rarely, AIR may be associated with dysregulated self-tolerance mechanisms in the thymus. Despite progress in research, our understanding of AIR remains incomplete. Lack of standardized methods for anti-retinal antibody testing continues to challenge the interpretation of seropositivity. Conventional immunosuppressives have been further studied, and promising immunomodulatory therapies, such as targeted B-cell therapy, have been introduced. Newer imaging modalities such as fundus autofluorescence and spectral domain optical coherence tomography may be helpful in diagnosis, monitoring progression of disease and response to treatment., Summary: AIR is a rare but vision-threatening disease whose pathogenesis is poorly defined. Lack of standardized clinical or laboratory criteria further complicates the diagnosis and management. Despite recent progress, further basic science research into the autoimmune process is needed. Prospective controlled clinical trials with immunomodulatory therapy can help define future treatment paradigms.

Background: Age-related macular degeneration remains the leading cause of irreversible blindness in the United States and the developed world. Intravitreal injections of anti–vascular endothelial growth factor (VEGF) medications have become standard of care for the treatment of the wet form of the disease. Recent reports have demonstrated an association with various immune factors. We aimed to investigate the effect of immunosuppressive therapy in the clinical course of the wet form of the disease. We compared anti-VEGF therapy plus one of three systemic immunosuppressive therapies versus anti-VEGF therapy alone for recurrent choroidal neovascularization associated with age-related macular degeneration., Methods: This was a pilot, Phase I/II, prospective, randomized, unmasked, single-center trial. Patients with subretinal exudation secondary to recurrent choroidal neovascularization associated with age-related macular degeneration were included in the study. Patients were randomized to 1 of 3 systemic arms immunosuppressive agents (daclizumab, rapamycin, or infliximab) for 6 months plus intraocular anti-VEGF therapy if indicated, compared with a group who received only anti-VEGF therapy if indicated., Results: The number of anti-VEGF injections per group, visual acuity, retinal thickness, and safety measures were assessed in all groups. Thirteen patients were randomized; comparing anti-VEGF injections before and during the study, a decrease in the number of injections from 0.73 injections per month to 0.42 for daclizumab and from 0.67 to 0.34 for sirolimus was seen, while no apparent decrease was seen for either infliximab or observation. Visual acuities were maintained in all groups., Conclusion: These preliminary data suggest that some immunosuppressive agents given systemically can alter the clinical course of the wet form of the disease and support the notion that more definitive clinical trials of immune mediation of age-related macular degeneration are indicated.

Cytotoxic T Lymphocyte-associated antigen 4 (CTLA-4) is an important costimultory receptor expressed on activated T cells. CTLA-4 blockade using a monoclonal antibody (mAb) in conjunction with tumor vaccines has improved tumor responses in animal models and enhanced numerous models of T cell-associated autoimmune diseases. Two patients with stage IV metastatic melanoma vaccinated with the gp 100 melanocyte/melanoma differentiation antigen either before or during anti-CTLA-4 mAb therapy developed uveitis. This is the first report of autoimmune disease involving the eye in patients treated with anti-CTLA-4 mAb. This suggests that CTLA-4 is an important regulatory molecule for maintenance of tolerance to melanosomal antigens and prevention of uveitis.