PURPOSE:The term white dot syndromes (WDS) has historically grouped multiple non-infectious posterior uveitis (NIPU) entities based on a similar funduscopic appearance of "white dots." Despite decades of use, the clinical relevance of this umbrella terminology has been questioned. This perspective critically examines whether WDS remains a valid conceptual and diagnostic construct in the era of advanced retinal and choroidal imaging. DESIGN:Perspective review. METHODS:Critical interpretation of the available literature on imaging and current pathophysiological evidence, combined with observations collected using cutting edge imaging technology (structural high-resolution optical coherence tomography (OCT), OCT angiography, and indocyanine green [ICG] angiography [ICGA]) for 6 of the NIPU classically considered as WDS: multiple evanescent white dot syndrome (MEWDS), multifocal choroiditis with panuveitis (MFCPU), punctate inner choroiditis (PIC), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis (SC), and birdshot chorioretinitis (BSCR). RESULTS:Although these diseases share some overlapping clinical features, multimodal imaging reveals profound differences, with each entity having distinct anatomic features on multimodal imaging. OCT angiography (OCTA) demonstrate distinct patterns of tissue involvement-from photoreceptor/retinal pigment epithelium (RPE) injury in MEWDS, to Bruch's membrane disruption in MFCPU and PIC, to profound choriocapillaris ischemia in APMPPE and SC, and deep stromal choroidal infiltration in BSCR. ICGA further differentiates these entities by choroidal perfusion characteristics, distinguishing true vascular non-perfusion from other inflammatory reactions leading to tissue damage. Imaging-based hypotheses of immunopathogenesis suggest that these entities may arise from different immunopathogenic pathways-transient outer retinal inflammation (MEWDS), possible antigenic exposure from structural disruptions (MFCPU/PIC), primary inflammatory inner choroidal vascular occlusive process (APMPPE), a possible autoimmune or autoinflammatory choroidal ischemic mechanism (SC), and a likely Human Leukocyte Antigen (HLA)-A29-associated autoimmune response affecting the inner retina and choroid (BSCR) CONCLUSIONS: The label WDS, originally based on appearance alone, does not take into consideration major biological and prognostic differences among these NIPU. Current imaging-guided hypotheses of immunopathogenesis suggest that these conditions should no longer be grouped under a single classification. A paradigm shift toward disease-specific terminology is warranted to improve diagnostic precision, guide management, and reflect presumed pathophysiological diversity.

PURPOSE:To develop imaging and consensus-based guidelines for the application of multimodal imaging in the diagnosis, assessment of disease activity, and detection of complications in Vogt-Koyanagi-Harada (VKH) disease. DESIGN:Consensus agreement guided by systematic literature review and expert committee deliberation using a nominal group technique (NGT). PARTICIPANTS:International uveitis and retina specialists participating in the Multimodal Imaging in Uveitis (MUV) taskforce. METHODS:A panel of experts independently reviewed published literature and representative cases of active, resolved, and late-stage VKH disease, using multimodal imaging modalities: color fundus photography (CFP), OCT, fundus autofluorescence (FAF), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and OCT angiography (OCTA). Structured NGT sessions were conducted to define consensus-based imaging descriptors of active and resolved VKH disease, plus VKH complications including retinal pigment epithelium disruption, choroidal neovascularization (CNV), subretinal fibrosis, and other sequelae. MAIN OUTCOME MEASURES:Identification of reproducible multimodal imaging features of VKH and imaging criteria for determining disease activity and complications. RESULTS:The experts agreed that active VKH diagnosis is supported by characteristic imaging findings in the early stages, including bilateral multifocal serous retinal detachments and optic disc hyperemia on CFP, multiloculated subretinal fluid and choroidal thickening on OCT, and round-to-oval hypofluorescent dots on ICGA. Multimodal imaging was considered a critical adjunct to the clinical, neurologic, and integumentary findings incorporated in existing diagnostic systems for VKH. Beyond diagnosis, these modalities are essential for assessing disease activity. Both OCT and ICGA were considered sensitive in detecting disease activity, and subclinical/occult disease reactivation. Fundus fluorescein angiography is useful for detecting multiple pinpoint hyperfluorescent lesions and optic nerve head staining. In contrast, FAF was considered of limited utility for diagnosis. OCT and OCTA were also regarded as important tools for detecting complications such as subretinal fibrosis and CNV. CONCLUSIONS:Incorporating multimodal imaging findings, particularly CFP, OCT, and ICGA, into the evaluation and classification of VKH enhances diagnostic accuracy, improves assessment of disease activity, and enables earlier detection of vision-threatening complications. These consensus-based recommendations provide a structured framework for optimal multimodal imaging use in VKH and may inform future refinements of diagnostic criteria. FINANCIAL DISCLOSURE(S):Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Purpose To determine the incidence, effect on adalimumab drug levels, and clinical consequences of antiadalimumab antibody formation, and to assess potential risk factors. Design Retrospective clinical cohort study. Participants One hundred twenty-eight patients treated with adalimumab who underwent antiadalimumab antibody monitoring. Methods Beginning October 2023, regularly scheduled antiadalimumab antibody and adalimumab level testing was begun. Using staggered entry analysis, anchored observation to treatment initiation, incidence was calculated. Time-updated models evaluated risk factors for antiadalimumab antibody formation. Main Outcome Incidence of antiadalimumab antibodies. Results Antiadalimumab antibodies developed in 37 of 128 patients for a rate of 0.077 per person-year (PY) (95% confidence interval [CI] 0.055/PY, 0.104/PY). Median serum adalimumab concentrations were significantly lower in antiadalimumab antibody-positive blood samples (2.6 µg/mL; interquartile range 0.8, 7.0) than in antibody-negative samples (10.2 µg/mL; interquartile range 6.9, 15.1), P < .00001. In time-updated analyses, there was a suggestion that concomitant immunosuppression was associated with a reduced risk of antiadalimumab antibodies (odds ratio [OR] 0.64; 95% CI 0.37, 1.10; P = .10) and weekly adalimumab dosing was associated with a reduced risk (OR 0.62; 95% CI 0.42, 0.91; P = .01). Antiadalimumab antibodies were associated with active ocular inflammation (OR 3.68; 95% CI 1.99, 6.82; P < .00001). Conclusions Antiadalimumab antibodies occur commonly among patients treated with long-term adalimumab, with a cumulative incidence of nearly 50% by 8 years of therapy. Antibody formation was associated with lower serum adalimumab levels and active ocular inflammation.

Aims To characterise the incidence of bilateral involvement and choroidal neovascularisation (CNV) in punctate inner choroiditis (PIC).Methods Retrospective, single-centre case series of 52 patients with PIC evaluated at a tertiary referral centre in the USA. Patients meeting Standardization of Uveitis Nomenclature criteria were included. Records were reviewed for laterality, CNV, treatment and visual outcomes. Incidence rates were calculated using person-year and eye-year denominators with staggered entry anchored at PIC diagnosis. Kaplan-Meier methods were used for time-to-event analyses. Time-updated analysis assessed immunosuppression as a risk modifier for active CNV.Results At diagnosis, 31/52 patients (59.6%) had unilateral disease. During follow-up, 11 developed fellow-eye involvement (0.08/person-year; 95% CI 0.04 to 0.13), with the highest incidence within 5 years of diagnosis (0.20/person-year; 95% CI 0.09 to 0.36). None of the four unilateral patients treated with immunosuppression developed fellow-eye disease while receiving therapy. At diagnosis, CNV was present in 34/73 eyes (46.6%). During follow-up, 20 eyes developed incident CNV (0.06/eye-year; 95% CI 0.04 to 0.10), highest within 5 years of diagnosis (0.14/eye-year; 95% CI 0.08 to 0.22). In time-updated analysis controlling for prior CNV, immunosuppression was associated with reduced odds of active CNV (OR, 0.50; 95% CI 0.27 to 0.93). 10 eyes developed visual acuity worse than 20/40, all associated with CNV; 8 improved to better than 20/40 after anti-vascular endothelial growth factor therapy.Conclusion PIC frequently presents unilaterally but carries risk of bilateral progression. CNV is common at presentation and is a cause of vision loss. Immunosuppression is associated with reduced odds of active CNV.

Purpose To develop imaging and consensus-based guidelines for the application of multimodal imaging in ocular toxoplasmosis (OT). Design International expert consensus study using the nominal group technique (NGT) guided by systematic literature review. Participants International uveitis specialists and retina experts participating in the Multimodal Imaging in Uveitis (MUV) taskforce. Methods A subgroup of experts reviewed the published literature on imaging in OT, along with complete multimodal imaging datasets from cases fulfilling the Standardized Uveitis Nomenclature (SUN) criteria. Imaging modalities included color fundus photography (CFP), optical coherence tomography (OCT), fundus autofluorescence (FAF), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and OCT angiography (OCTA). NGT sessions were conducted to identify key imaging features of typical and atypical presentations of active and inactive OT across modalities and to define imaging findings associated with disease-related complications. Consensus-based imaging descriptors and practical imaging guidelines were developed through iterative discussion. The proposed guidelines were voted on by the MUV taskforce. Main Outcome Measures Identification of multimodal imaging features of active and inactive OT and development of imaging guidelines for diagnosis, activity assessment, and detection of complications. Results The experts agreed that classic OT remains primarily a clinical diagnosis. CFP was considered sufficient for diagnosing typical active OT presenting as focal or paucifocal necrotizing retinochoroiditis. OCT provided important information on lesion depth, retinal and choroidal involvement, and vitreoretinal interface abnormalities, and was particularly valuable in atypical presentations and for longitudinal disease monitoring. FAF assisted in staging lesion evolution and identifying MEWDS-like reactions. FA and ICGA were not recommended for routine use but suggested in selected cases to evaluate the presence and extent of complications. OCTA was considered sufficient for detecting choroidal neovascularization in most cases. Multimodal imaging was generally recommended for atypical OT and for assessment of complications. Conclusions The consensus-based imaging guidelines by MUV provide a standardized and pragmatic framework for the use of multimodal imaging in OT. While the diagnosis in typical cases remains largely clinical, as outlined in the SUN classification, OCT and FAF are valuable for monitoring disease activity, and additional imaging modalities support the management of atypical presentations and complications.

PURPOSE:To assess the association of cataract progression and intraocular pressure (IOP) elevation with stable-dosing of topical corticosteroid for chronic anterior uveitis (CAU). METHODS:All patients with CAU from the Systemic Immunosuppressive Therapy for Eye Diseases Cohort treated with topical prednisolone 1% or equipotent equivalent for at least six months were analyzed. Main outcomes were incident cataract formation and IOP elevation above predefined thresholds (≥21 mmHg and ≥30 mmHg). Additional variables with potential to influence the frequency of the main outcomes were also collected. RESULTS:For the incident cataract outcome, 346 patients met the eligibility criteria. Based on differing eligibility criteria, fewer patients were enrolled for the IOP events (n = 260 and 282 for ≥21 and ≥30 mmHg, respectively). In general, the incidence of cataract formation increased with increasing daily drops of stable-dosing of topical corticosteroid; however, there was no excess risk (relative to zero drops) for eyes exposed to ≤1 drop daily of prednisolone 1% or equivalent [aHR, 0.91 (95% CI, 0.29, 2.84)]. Similarly, higher daily topical corticosteroid exposure conferred greater risk of both IOP outcomes, though plateau effects were observed. For eyes receiving ≤1 drop daily of prednisolone 1% or equivalent, there was no excess risk of either IOP outcome [aHR for ≥21 mmHg, 0.77 (95% CI, 0.19, 3.05) and aHR for ≥30 mmHg, 0.92 (95% CI, 0.11, 7.76)]. CONCLUSIONS:Stable-dosing of topical prednisolone 1% or equivalent more than once daily was associated with increased cataract formation and IOP elevation over medium-term follow-up, whereas ≤1 drop/day was not.

Importance:Retinal vasculitis is a sight-threatening condition associated with diverse ocular and systemic diseases. Variability in terminology and definitions across clinical practice and research has limited diagnostic consistency, communication among specialists, and comparability of studies. Objective:To develop standardized, consensus-based definitions for retinal vasculitis and related terms to improve diagnostic clarity and harmonize communication among ophthalmologists and other medical specialties. Design, Setting, and Participants:This was an international modified Delphi consensus study conducted using a structured consensus process guided by a comprehensive literature review, including systematic reviews and meta-analyses. Two rounds of Delphi surveys were performed, and consensus was predefined as at least 75% agreement. Included was an international expert panel of 27 specialists in ophthalmology, rheumatology, pathology, imaging, and research methodology. The specialists had recognized expertise in retinal and systemic vasculitis, retinal imaging, or consensus methodology. Study data were analyzed from March to September 2025. Exposure:Participation in a structured Delphi process evaluating proposed definitions and terminology related to retinal vasculitis and associated vascular inflammatory entities. Main Outcomes and Measures:Consensus definitions for retinal vasculitis and related entities, with agreement using predefined consensus thresholds. Results:A total of 27 international experts participated in the Delphi process. After 5 executive committee members involved in developing preliminary definitions were excluded from voting to minimize bias, a total of 22 independent experts completed both Delphi rounds. All candidate statements exceeded the predefined consensus threshold in the first round (agreement range, 83.3%-95.5%), although some demonstrated variability in the strength of agreement. After refinement of definitions, consensus strengthened in the second round, with agreement levels ranging from 95.2% to 100%. The panel established consensus definitions for 8 terms: retinal vasculitis, vascular leakage, infectious retinal vasculitis, noninfectious retinal vasculitis, retinal perivasculitis, retinal vasculopathy, primary retinal vasculitis, and far-peripheral vascular leakage. These definitions were developed to distinguish vascular leakage from true vasculitis, clarify inflammatory and noninflammatory vascular disorders, and promote consistent terminology across clinical practice and research. Conclusions and Relevance:This international Delphi consensus established standardized nomenclature for retinal vasculitis and related vascular inflammatory entities. Adoption of these definitions may improve diagnostic accuracy, facilitate interpretation of imaging findings, enhance consistency across clinical studies, and support future research efforts, including the development of artificial intelligence-based classification systems for retinal vascular disease.

Objective To evaluate whether common plasma proteomic pathways connect host genetic risk factors to incident age-related macular degeneration (AMD) in persons with AIDS. Design Nested case-control study. Participants Subset of persons enrolled in the Longitudinal Study of Ocular Complications of AIDS. Methods Baseline cryopreserved plasma specimens were assayed for inflammatory and cardiovascular proteins using the Olink Inflammation Explore Panels 1 to 2 and the Cardiometabolic Explore Panels 1 to 2. Age-related macular degeneration‑associated genetic variants were assessed using Applied Biosystems Taqman probes. Baseline proteomic profiles for 26 persons who subsequently developed incident intermediate-stage AMD after 5 to 10 years of follow-up and 49 controls without AMD matched for age, natal sex, race/ethnicity, and follow-up duration were compared. False discovery rate correction was performed with the Storey Q method, and both unsupervised gene ontology pathway enrichment and dedicated pathway analyses were performed. Age-related macular degeneration‑associated plasma protein levels were compared between AMD high-risk and AMD low-risk genotypes. Main Outcome Measures Incident intermediate-stage AMD. Results 371 (26%) of 1448 evaluable plasma proteins were associated with incident AMD at the false discovery rate Q < 0.05 threshold. Hallmark pathways significantly enriched in incident AMD included inflammation, complement, and fatty acid metabolism pathways. Complement factor H, a complement activation regulating protein with a genetic association with AMD, levels appeared to be associated with AMD (adjusted odds ratio: 0.12 per 2-fold increase, P = 0.055). Several related proteins in the Hallmark complement pathway were strongly associated with AMD, 16 including CD46, platelet-derived growth factor β, chemokine ligand 1, and CCL5 (all Q < 0.05), which have been linked to AMD risk in genetic studies. Among cholesterol homeostasis-related genes, lipoprotein lipase was associated with a decreased risk of AMD (adjusted odds ratio: 0.18 per 2-fold increase, Q = 0.008). Participants with AMD high-risk genotypes had elevated levels of shared proteins in inflammatory, complement, and fatty acid metabolism pathways. Conclusions Levels of several plasma proteins linked to the complement pathway and cholesterol homeostasis, which have been linked to AMD risk in genetic studies, are associated with incident AMD in people with AIDS. Plasma levels of shared inflammatory proteins linked to several different genetic AMD risk factors predicted AMD risk, suggesting common immunologic mechanisms linking genetic risk factors for AMD. Financial Disclosure(s) Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Purpose To develop imaging and consensus-based guidelines for the application of multimodal imaging in the clinical diagnosis, monitoring and detection of complications in Behçet disease (BD) uveitis. Design International expert consensus agreement using the nominal group technique (NGT) guided by systematic literature review. Participants International uveitis and retina experts participating in the Multimodal Imaging in Uveitis (MUV) taskforce. Methods A committee of experts reviewed the published literature on imaging in BD uveitis, along with representative multimodal imaging datasets of active, resolved and late-stage uveitis in BD. All cases in these datasets met the Standardized Uveitis Nomenclature (SUN) diagnostic criteria. Imaging modalities included color fundus photography (CFP), fundus fluorescein angiography (FFA), optical coherence tomography (OCT), fundus autofluorescence (FAF), indocyanine green angiography (ICGA), and OCT angiography (OCTA). NGT sessions were conducted to define consensus-based key imaging descriptors of active and resolved BD uveitis, along with the complications and sequelae. Main Outcome Measures Identification of reproducible multimodal imaging features of active and resolved BD uveitis. Results The experts agreed that CFP, FFA and OCT are the most relevant imaging modalities in the management of BD uveitis, with particular emphasis on ultra-widefield imaging. Characteristic findings on CFP include vitreous haze, retinal infiltrates, optic nerve head inflammation, and prominent retinal vasculitis with possible occlusion. FFA was deemed critical in assessing vascular, macular and optic nerve head leakage, thereby indicating disease activity. OCT is helpful in detecting and characterizing cystoid macular edema, partial thickness inner retinitis (smudge-sign), subretinal fluid, and overlying vitreous condensation. FFA and OCT assist in demonstrating disease resolution, and detection of late changes such as retinal non-perfusion, neovascularization, epiretinal membrane formation and retinal atrophy. The experts concluded that FAF, ICGA and OCTA have limited role in disease. Based on these findings, consensus-based statements were generated and voted upon by the MUV taskforce. Conclusion Incorporation of consensus-based imaging guidelines by MUV, particularly CFP, FFA and OCT, enhances diagnostic evaluation, improves assessment of disease activity, assessment of treatment response, and detection of complications in BD uveitis. These recommendations provide a structured framework for optimal multimodal imaging use in BD uveitis and aid future refinements of diagnostic criteria.

PURPOSE:To develop imaging-based statements and consensus recommendations for the application of multimodal imaging in tubercular choroiditis (TBC). DESIGN:International expert committee-led consensus agreement using a modified nominal group technique with further refinement from literature review and full task force voting. PARTICIPANTS:International expert committee of uveitis and retina specialists from the Multimodal Imaging in Uveitis (MUV) task force. METHODS:The MUV task force convened a group of international experts to develop a set of standardized imaging criteria for TBC. Cases with focus on tubercular serpiginous-like choroiditis (TB SLC) and tuberculomas/tubercles were provided by expert members to develop a review case portfolio of both active and inactive disease with longitudinal follow-up. Cases included color fundus photography, fundus autofluorescence, fundus fluorescein angiography, indocyanine green angiography, OCT, and OCT angiography. The case portfolio was reviewed by all members, and through a structured nominal group technique and guided by a literature review, the group refined descriptive statements characterizing active, evolving, and inactive lesions until consensus was achieved. MAIN OUTCOME MEASURES:Imaging statements and consensus guidelines describing features of TB SLC and tuberculoma/tubercle in relation to diagnosis, monitoring, and detection of disease complications. RESULTS:The case portfolio included 31 cases, and the group achieved consensus on defining distinct imaging features of TB SLC and choroidal tuberculomas/tubercles. For TB SLC, key features of active lesions included yellow-white multifocal or placoid lesions with hyperautofluorescent edges on fundus autofluorescence, outer retinal hyperreflectivity, and focal choroidal thickening on OCT. Both indocyanine green angiography and OCT angiography highlight lesions that are often more extensive than those seen clinically. Choroidal tuberculomas were defined as solitary, lobulated masses often associated with subretinal fluid and retinal neovascularization, whereas tubercles appear as multiple small lesions associated with disseminated tuberculosis. Consensus-based guidelines were established to use these multimodal imaging features for diagnosis, monitoring response to treatment, and detecting complications. CONCLUSIONS:The MUV imaging guidelines for TBC extend existing diagnostic frameworks by systematically integrating multimodal imaging features for disease characterization and monitoring. These consensus-based recommendations enhance phenotypic classification, improve assessment of disease activity, and provide clinically relevant endpoints for evaluating treatment response. FINANCIAL DISCLOSURE(S):Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

PURPOSE:To develop imaging-based measures for disease assessment in noninfectious posterior uveitis (NIPU). DESIGN:A mixed-methods design, beginning with a review of previously developed imaging recommendations formulated by separate subcommittees of the multimodal imaging in uveitis (MUV) initiative, followed by a structured consensus process using the nominal group technique (NGT), facilitated by an independent expert committee. METHODS:An expert committee reviewed and extracted all consensus-based imaging recommendations from the MUV subcommittee manuscripts focused on five major NIPU entities. The primary objective was to categorize imaging features as suggestive of active disease (SAD), suggestive of inactive disease (SID), or equivocal. This process was conducted using the NGT to reach consensus-based imaging measures. These recommendations were further voted upon by members of the full task force. RESULTS:A total of 49 imaging statements were deliberated using two rounds of NGT and independent voting. For the five included diseases, a total of 21 statements qualified as features of SAD, whereas 12 statements were classified as SID. The remaining 16 statements were categorized as equivocal features that need further investigation to determine whether the disease is active. CONCLUSIONS:This study builds upon the multinational efforts of the MUV initiative to extend the standardization of uveitis nomenclature (SUN) work through the integration of additional multimodal imaging information. Defining clear imaging-based outcome measures for NIPU, it establishes a structured framework supporting objective disease assessment. These standardized imaging measures are expected to enhance the utility of multimodal imaging in both routine uveitis care and future clinical trials.

PURPOSE:To develop imaging- and consensus-based guidelines for the application of multimodal imaging in cytomegalovirus retinitis (CMVR). DESIGN:Consensus agreement guided by a systematic literature review and expert committee deliberation using the nominal group technique (NGT). PARTICIPANTS:International uveitis specialists and retina experts participating in the Multimodal Imaging in Uveitis (MUV) task force. METHODS:Experts independently reviewed published literature and representative cases of active and inactive CMVR using color fundus photography (CFP), OCT, fundus autofluorescence, fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and OCT angiography. Through structured NGT sessions, the committee developed consensus-based descriptors of active and inactive CMVR, key imaging biomarkers of disease activity, and characteristic complications. The proposed guidelines were subsequently voted on by the full MUV task force. MAIN OUTCOME MEASURES:Identification of reproducible multimodal imaging features of CMVR; definition of modality-specific biomarkers of disease activity and healing; consensus on the preferred imaging modalities for diagnosis, monitoring, and detection of complications. RESULTS:The experts agreed that CFP remains the most essential baseline and follow-up imaging modality for documenting the pattern, extent, and borders of the lesion, as well as the response to treatment. Ultra-widefield CFP was particularly valued for its ability to detect peripheral/satellite lesions and complications such as early retinal breaks. OCT is helpful in identifying inner retinal necrosis, characterizing retinal layer involvement, and development of complications such as cystoid macular edema (CME) and epiretinal membrane. Fundus autofluorescence assists in delineating the advancing edge of active retinitis and identifies healed lesions by their sharply demarcated hypoautofluorescent appearance. Fundus fluorescein angiography may help in identifying arteriolar occlusion, CME, and optic nerve head leakage. OCT angiography and ICGA have limited roles in diagnosing CMVR or assessing disease activity. CONCLUSIONS:Clinical examination/CFP remains the primary method for evaluating CMVR, in addition to other clinical tools such as diagnostic laboratory testing. OCT and FFA serve as complementary tools for detecting ocular complications, especially in immune-recovery uveitis. These consensus-based guidelines provide a framework for optimal imaging selection in CMVR and support future refinements of standardized diagnostic criteria. FINANCIAL DISCLOSURE(S):Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

PURPOSE:To identify the incidence of cataract and the outcomes of cataract surgery in eyes with ocular cicatricial pemphigoid (OCP). METHODS:Phakic eyes were identified from the Systemic Immunosuppressive Therapy for Eye Diseases Cohort Study and followed for the incidence of visually significant cataract defined as: newly reduced visual acuity 20/50 or worse attributed to cataract; and/or incident cataract surgery. Secondarily, all eyes with OCP that underwent cataract surgery and had a year of follow up thereafter, were included in an analysis of visual outcome. RESULTS:Three hundred fifty-five phakic eyes (200 patients) with OCP were at risk. Eighty eyes developed visually significant cataract over 1064 eye years (incidence rate = 7.5%/eye-year, 95% confidence interval [CI] = 5.6 to 10.1). Higher age was associated with increased incidence of cataract (adjusted hazard ratio [aHR] = 4.47; 95% CI, 1.95-10.23 for age 60-75 inclusive and aHR = 8.37; 95% CI, 3.60-19.42 for age > 75, each compared with age <60 years). Seventy-nine eyes of 61 patients were monitored for > = 1 year following cataract surgery. Cataract surgery was associated with an improvement of vision around 4 lines, which was sustained through at least 48 months. Poorer pre-operative visual acuity was associated with poorer long-term visual outcome. CONCLUSIONS:The incidence of cataract was high in this older population. No factors predictive of cataract such as duration of OCP or use of corticosteroids were identified. Visual acuity improved after surgery by a median of 4 lines' gain at one year; poorer long-term outcome among those with initially poorer visual acuity may be secondary to corneal scarring.

PURPOSE:To evaluate the incidence of failure of trabeculectomy versus tube shunt (TS) glaucoma surgery in eyes of patients with uveitis. DESIGN:Multicenter retrospective cohort study. PARTICIPANTS:Among 356 eyes of 288 patients with noninfectious inflammatory eye disease undergoing first incisional glaucoma surgery using one of the techniques, 244 eyes had TSs, and 112 eyes had trabeculectomy augmented with mitomycin-C (Trab-MMC). METHODS:A standardized chart review was used to collect clinical data over time retrospectively. Cox regression analyses with adjustment for propensity score and intereye correlations were performed to compare the incidence of failure of glaucoma surgery between TS and Trab-MMC. MAIN OUTCOME MEASURES:Failure of glaucoma surgery of the first 5 years postoperatively, defined as the following: (1) intraocular pressure (IOP) ≤ 5 or > 21 mmHg at 2 consecutive visits at least 90 days apart beginning 3 months after surgery; or (2) reoperation; or (3) complete blindness (no light perception). RESULTS:The median age was 40.3 years (interquartile range [IQR], 13.4-57.3 years) in the TS group and 44.2 years (IQR, 29.0-58.9 years) in the Trab-MMC group. The median preglaucoma surgery IOP was 30.0 mmHg (IQR, 21-35.5 mmHg) in the TS group and 30.5 mmHg (IQR, 20-38 mmHg) in the Trab-MMC group. Anterior uveitis was the most common location of primary inflammation in both the TS (52.5%) and Trab-MMC 55.4%) groups. Failure was observed in the TS group in 23.5%, 27.1%, and 30.8% cumulatively through 12, 24, and 36 months, respectively, versus 16.1%, 25.6%, and 30.0%, respectively, in the Trab-MMC group. In the propensity score-adjusted Cox regression analysis, there was no significant difference in failure incidence rate between the TS and Trab-MMC groups (adjusted hazard ratio, 1.08; 95% confidence interval, 0.65-1.78; P = 0.77). Success without the requirement for IOP-lowering medicines was observed more frequently in the Trab-MMC group. CONCLUSIONS:Tube shunt and Trab-MMC fail frequently with similar incidences when done as the first glaucoma surgery among eyes with uveitis over 5 years of follow-up, but there were more complete successes in the Trab-MMC group than in the TS group at 12, 24, and 36 months. FINANCIAL DISCLOSURE(S):Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

PURPOSE:To evaluate clinical and treatment outcomes in patients with peripheral ulcerative keratitis (PUK). DESIGN:Retrospective, case series SUBJECTS: Patients diagnosed with PUK at the Wilmer Eye Institute between January 2003 and October 2022. METHODS:Data collected included demographics, presence of systemic disease, disease laterality, duration of disease, PUK activity, presence of corneal perforation, and treatments. Outcomes of interest included: disease control, corticosteroid-sparing success, corticosteroid-discontinuation success, sustained drug-free remission, disease reactivation, occurrence of perforation, and need for surgery. RESULTS:Fifty-seven patients with PUK were identified. The median age was 53 years, with 46% of patients being Black. Most patients (56%) had an associated systemic diagnosis. The median duration of symptoms prior to presentation was 3 months and 42% of patients presented with bilateral disease. Of the 81 affected eyes, 7 had perforated prior to presentation. During a median follow-up of 3 years, 76% of patients received oral prednisone and 80% received at least 1 immunosuppressive drug. Disease control was achieved in all patients within a median of 1.3 months. The rates of corticosteroid-sparing success and corticosteroid discontinuation were 0.44 per patient-year (/PY) and 0.27/PY, respectively. Sustained drug-free remission was achieved in only 6% of patients. During follow-up, the rate of corneal perforation was 0.009/EY. The rate of disease reactivation was 0.07/EY, with a median time to reactivation of ∼2 years. CONCLUSIONS:Over a moderate amount of follow-up, systemic therapy achieved disease control, corticosteroid-sparing and corticosteroid discontinuation. However, sustained drug-free remission was infrequent in our cohort.

Purpose: Evaluation of longer-term effectiveness of 3 intravitreal therapies (methotrexate, ranibizumab, or dexamethasone implant) for participants enrolled in the randomized comparative effectiveness trial the Macular Edema Ranibizumab versus Intravitreal Anti-inflammatory Therapy (MERIT) Trial followed up for 24 weeks. Design: Multicenter randomized controlled clinical trial with masked evaluation of retinal thickness and visual acuity. Participants: Patients with persistent or recurrent uveitic macular edema. Methods: Participants from 33 centers were randomized 1:1:1 (stratified by presence or absence of concomitant systemic immunosuppression for uveitis) to receive a sequence of intravitreal treatments with dexamethasone implant, methotrexate, or ranibizumab. Participants with bilateral macular edema received the same treatment bilaterally. During 24 weeks of follow-up, nonassigned treatments were permitted beginning from 12 weeks for those meeting re-treatment criteria. Main Outcome Measures: Central subfield thickness (CST) change from baseline OCT measurement was the main outcome. Secondary outcomes included change in mean standard letters of baseline best-corrected visual acuity (BCVA). Analyses were conducted according to 2 principles: (1) as assigned, in which outcomes were analyzed according to their original randomized treatment, and (2) a supplementary censored analysis, in which data were excluded after an eye received a nonassigned treatment. Results: Among 194 enrolled participants (225 eligible eyes), 177 participants (207 eyes) completed 24 weeks of follow-up. Eyes assigned to methotrexate (55%) and ranibizumab (37%) more frequently received nonassigned treatments (88% dexamethasone implant or intravitreal corticosteroid injection) compared with eyes assigned to dexamethasone (7%). In the as-assigned analysis, dexamethasone showed superior improvement in macular edema compared with ranibizumab (CST, 34% vs. 19%; P = 0.01), but not compared with methotrexate (CST, 31%; P = 0.59) after being superior to both other regimens at 12 weeks. However, in the censored analysis, only dexamethasone was associated with improvements in macular edema (CST, 34% vs 8% [P < 0.001] and 5% [P < 0.001]) and BCVA improvement of > 5 letters compared with methotrexate and ranibizumab, respectively. Dexamethasone more often was associated with intraocular pressure elevations of >= 24 mmHg (32%) and of >= 30 mmHg (10%). Conclusions: Dexamethasone was more effective than methotrexate and ranibizumab for the treatment of persistent or recurrent uveitic macular edema through 24 weeks, with manageable side effects.

PURPOSE:To develop consensus-based imaging guidelines for diagnosing and monitoring birdshot chorioretinopathy (BSCR). DESIGN:Consensus-based approach guided by literature and an expert committee using a nominal group technique (NGT). METHODS:An expert committee of 5 international uveitis specialists reviewed 15 well-documented representative BSCR cases with comprehensive imaging data. Cases with active and inactive disease were included. Imaging, including color fundus photography (CFP), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), optical coherence tomography (OCT), fundus autofluorescence (FAF), and OCT angiography (OCTA) were reviewed. Using a structured NGT approach, consensus-based recommendations were developed for specific disease characteristics, biomarkers of activity, and complications. The recommendations were voted upon by members of the full task force. RESULTS:For the diagnosis of BSCR, CFP, FFA, and ICGA were identified as the key imaging modalities. ICGA was identified as a key imaging modality for assessing the presence of choroidal lesions. FFA was deemed crucial for monitoring retinal vascular leakage and assessing the treatment response. OCT, while not essential for diagnosis, was valuable for detecting complications such as cystoid macular edema and retinal thinning. The committee did not reach a consensus on the role of FAF and OCTA for the diagnosis or monitoring of BSCR. CONCLUSIONS:The MUV consensus-based imaging guidelines for BSCR expand the Standardization of Uveitis Nomenclature (SUN) classification criteria by reaffirming the critical role of ICGA and providing a standardized guidelines for using other imaging modalities in the diagnosis and monitoring of BSCR. These guidelines are expected to facilitate monitoring of disease activity and complications using multimodal imaging.

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Objective To evaluate the incidence of visually significant posterior capsule opacification (PCO with visual acuity <= 20/50) and the incidence of Nd:YAG laser capsulotomy in the year following cataract surgery for uveitic eyes. Method Patients were identified from the Systemic Immunosuppressive Therapy for Eye Diseases (SITE) Cohort Study using a standardized chart review process. Results Among 1,855 uveitic eyes of 1,370 patients who had undergone cataract surgery, visually significant PCO occurred in 297 eyes (16%), and YAG laser capsulotomy was done in 407 eyes (22%) within the first year following surgery. Higher odds of developing 20/50 visual acuity attributed to PCO were noted in children and young adults compared with adults older than 65 years of age (overall p = 0.03). Poorer preoperative visual acuity (overall p = 0.0069) and postoperative inflammation (odds ratio [OR] = 1.83; 95% CI, 1.37-2.45; p < 0.0001) were associated with PCO incidence. In multivariable analysis, risk factors for YAG laser capsulotomy were younger age groups compared with those older than 65 years of age at the time of surgery (adjusted OR [aOR] = 1.90-2.24; 95% CI, 1.90-2.24; overall p = 0.0007), female sex (aOR = 1.37; 95% CI, 1.03-1.82; p = 0.03), postoperative active inflammation (aOR = 165; 95% CI, 1.27-2.16; overall p < 0.0001), extracapsular cataract extraction compared with phacoemulsification (aOR = 1.70; 95% CI, 1.17-2.47; overall p < 0.0001), and insertion of an intraocular lens (aOR = 4.60; 95% CI, -2.29-9.25; p < 0.0001). Black race was associated with lower YAG laser capsulotomy incidence than Whites (aOR = 0.36; 95% CI, 0.24-0.52; overall p < 0.0001). Conclusions Vision-reducing (<= 20/50) PCO is common, occurring in about one sixth of uveitic eyes within 1 year of cataract surgery; a higher number (22%) of eyes underwent YAG laser capsulotomy within the first year. Age and postoperative inflammation following cataract surgery are the variables most associated with the incidence of visually significant PCO and YAG laser capsulotomy.

PURPOSE:The Multimodal Imaging in Uveitis (MUV) project is a comprehensive initiative aimed at developing guidelines for the use of multimodal imaging (MMI) in diagnosing and managing noninfectious posterior uveitis (NIPU). This project seeks to develop standardized guidelines and a minimal imaging set leading to evidence and consensus-based imaging guidelines that are applicable across diverse clinical settings. This manuscript describes the overall goals and methodology of the project. DESIGN:Descriptive study. METHODS:The MUV project was structured into 7 phases: (1) a global survey to assess current practices and the need for standardized MMI criteria, (2) study design planning to define research questions and establish expert committees, (3) systematic review of the literature, (4) evidence and consensus-building on imaging guidelines through the Nominal Group Technique (NGT), (5) agreement with proposed guidelines by task force and development of consensus statements for use of MMI in the diagnosis and monitoring of NIPU, (6) standardizing the endpoints of inflammation on imaging in NIPU and (7) prospective validation of MMI criteria, using formal consensus techniques, to achieve supermajority agreement. RESULTS:The initial survey revealed that nearly 90% of uveitis and retina specialists already incorporate MMI into their diagnostic process for NIPU, with strong support for standardized practices. The NGT phase formulated and achieved statements on imaging guidelines of MMI, findings that were further substantiated by the systematic review. The final guidelines, proposed by the NGT and approved by the task force, offer a standardized framework for utilizing MMI in NIPU. This was a key aspect of this patient-focused update. CONCLUSION:The MUV project introduces a formalized set of guidelines for MMI use in NIPU, enhancing and extending the Standardization of Uveitis Nomenclature (SUN) classification framework. The comprehensive guidelines developed through this initiative will standardize the use of MMI in clinical practice, which should lead to optimal use of imaging for more accurate diagnoses, better monitoring of disease activity, and improved management of complications.