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Purpose: To evaluate the influence of immunomodulatory therapy (IMT) on visual and treatment outcomes of inflammatory choroidal neovascularization (iCNV) in patients affected by multifocal choroiditis (MFC), and to compare them to patients treated with steroids as needed., Design: Multicenter retrospective matched cohort study., Methods: Patients affected by MFC with iCNV were divided into a IMT group and a "steroids as needed" group and matched according to the time between diagnosis and beginning of systemic treatment. Visual acuity (VA), number of anti-vascular endothelial growth factor (VEGF) intravitreal injections, and number of iCNV reactivations during 2 years of follow-up after treatment initiation were compared between the 2 groups., Results: A total of 66 eyes of 58 patients were included, equally divided into the 2 groups. Patients in the IMT group had a lower relative risk (RR) of iCNV reactivation (0.64, P = .04) and of anti-VEGF intravitreal injection retreatment (0.59, P = .02). Relapses of MFC-related inflammation were independently associated with a higher RRs of iCNV reactivation (1.22, P = .003). Final VA was higher in the IMT compared to the steroids as needed group (mean [SD], 69.1 [15.1] vs 77.1 [8.9] letters, P = .01), and IMT was associated with greater VA gains over time (+2.5 letters per year, P = .04)., Conclusions: IMT was associated with better visual and treatment outcomes in MFC complicated by iCNV compared to steroids as needed. The better outcomes of the IMT group and the association between MFC-related inflammation and iCNV reactivations highlight the need for tighter control of inflammation to prevent iCNV relapses and visual loss., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: To identify characteristics on multimodal imaging (MMI) in idiopathic multifocal choroiditis (MFC) that can identify inflammatory activity and distinguish choroidal neovascularization (CNV) activity from inflammatory activity., Design: Prospective cohort study., Methods: MMI consisted of spectral-domain optical coherence tomography (angiography) (SD-OCT(A)), fundus autofluorescence, fundus photography, infrared imaging, fluorescein angiography (FA), and indocyanine green angiography (ICGA). MMI characteristics obtained during active and inactive disease were compared within the same lesion. Secondly, MMI characteristics were compared between active inflammatory lesions with and without CNV activity., Results: Fifty patients (110 lesions) were included. In 96 lesions without CNV activity, the mean focal choroidal thickness was increased during the active disease (205 µm) compared to the inactive disease (180 µm) (P ≤ .001). Lesions with inflammatory activity typically demonstrated moderately reflective material located in the sub-retinal pigment epithelium (RPE) and/or in the outer retina with disruption of the ellipsoid zone. During the inactive stage of the disease, the material disappeared or became hyperreflective and indistinguishable from the RPE. During the active stage of the disease, the area of hypoperfusion in the choriocapillaris significantly increased as visualized on both ICGA and SD-OCTA. CNV activity in 14 lesions was associated with subretinal material with a mixed reflectivity and hypotransmission of light to the choroid on SD-OCT and leakage on FA. SD-OCTA identified vascular structures in all active CNV lesions and in 24% of lesions without CNV activity (showing old, quiescent CNV membranes)., Conclusion: Inflammatory activity in idiopathic MFC was associated with several MMI characteristics, including focally increased choroidal thickness. These characteristics can guide clinicians in the challenging process of the evaluation of disease activity in idiopathic MFC patients., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to determine classification criteria for punctate inner choroiditis (PIC)., Design: Machine learning of cases with PIC and 8 other posterior uveitides., Methods: Cases of posterior uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on diagnosis by using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used in the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the posterior uveitides. The resulting criteria were evaluated in the validation set., Results: A total of 1,068 cases of posterior uveitides, including 144 cases of PIC, were evaluated by machine learning. Key criteria for PIC included: 1) "punctate"-appearing choroidal spots <250 µm in diameter; 2) absent to minimal anterior chamber and vitreous inflammation; and 3) involvement of the posterior pole with or without mid-periphery. Overall accuracy for posterior uveitides was 93.9% in the training set and 98.0% (95% confidence interval: 94.3-99.3) in the validation set. The misclassification rates for PIC were 15% in the training set and 9% in the validation set., Conclusions: The criteria for PIC had a reasonably low misclassification rate and appeared to perform sufficiently well for use in clinical and translational research., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: To analyze baseline clinical and imaging risk factors associated with poor outcome in patients with tubercular serpiginous-like choroiditis (TB SLC)., Design: Retrospective clinical study., Methods: Charts and fundus photographs of consecutive patients with active TB SLC seen at a single tertiary referral center with 6 months follow-up after initiation of treatment were reviewed. Logistic mixed models were performed to determine the clinical and imaging factors associated with the response to therapy, including the opacity of choroiditis graded according to a 3-point scale., Results: This study included 203 eyes of 183 patients with active TB SLC. Poor initial best-corrected visual acuity (BCVA) and foveal and optic disc involvement were associated with poor response to therapy at 6 months (odds ratio [OR] 4.489, 95% confidence interval [CI]: 1.92-10.47; P = .001; OR 2.892, 95% CI: 1.23-6.81; P = .015; OR 11.633, 95% CI: 3.17-42.71; P < .001, respectively). The high opacity grades (2 and 3) were also associated with poor outcomes OR 9.541; 95% CI: 2.94-30.91; P = .001). Poor baseline BCVA and high grade of opacity of the lesions were the composite risk factors for paradoxical worsening of TB SLC (OR 7.555, 95% CI: 1.78-32.02; P = 0.006; OR 7.434, 95% CI: 1.34-41.18; P =0.021, respectively)., Conclusions: TB SLC with higher grades of lesion opacity at baseline may be associated with greater risk of poor therapeutic response and paradoxical worsening. Grading of baseline lesion opacity may be used in future prospective studies to predict the biological behavior of the lesions and may serve as a guide to therapeutic interventions., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: Provide baseline and preliminary follow-up results in a 5-year longitudinal study of Blau syndrome., Design: Multicenter, prospective interventional case series., Methods: Baseline data from 50 patients from 25 centers worldwide, and follow-up data for patients followed 1, 2, or 3 years at the end of study enrollment. Ophthalmic data were collected at baseline and yearly visits by means of a standardized collection form., Results: Median age at onset of eye disease was 60 months and duration of eye disease at baseline 145 months. At baseline 38 patients (78%) had uveitis, which was bilateral in 37 (97%). Eight patients (21%) had moderate to severe visual impairment. Panuveitis was found in 38 eyes (51%), with characteristic multifocal choroidal infiltrates in 29 eyes (39%). Optic disc pallor in 9 eyes (12%) and peripapillary nodules in 9 eyes (12%) were the commonest signs of optic nerve involvement. Active anterior chamber inflammation was noted in 30 eyes (40%) at baseline and in 16 (34%), 17 (57%), and 11 (61%) eyes at 1, 2, and 3 years, respectively. Panuveitis was associated with longer disease duration. At baseline, 56 eyes (75%) were on topical corticosteroids. Twenty-six patients (68%) received a combination of systemic corticosteroids and immunomodulatory therapy., Conclusions: Blau uveitis is characterized by progressive panuveitis with multifocal choroiditis, resulting in severe ocular morbidity despite continuous systemic and local immunomodulatory therapy. The frequency and severity of Blau uveitis highlight the need for close ophthalmologic surveillance as well as a search for more effective therapies., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: To describe the features of an artifact on near-infrared reflectance and MultiColor imaging, termed "ghost maculopathy," and to illustrate how it may masquerade as true chorioretinal pathology., Design: This was a retrospective, observational case series., Methods: The authors studied 144 eyes of 72 consecutive patients in a vitreoretinal clinical practice, reviewing multimodal imaging including color and red-free fundus photography, fundus autofluorescence (FAF), near-infrared reflectance, MultiColor imaging, and spectral-domain optical coherence tomography (SD OCT)., Results: In 36 of 144 eyes (25%), there was an appearance of a hyper-reflective spot on near-infrared reflectance and MultiColor imaging, located at the macula, nasal or superonasal to the fovea, which did not correspond to any apparent lesion on color and red-free fundus photography, FAF, or SD OCT. This spot was termed the "ghost image" in this phenomenon of "ghost maculopathy." The ghost image was present consistently on near-infrared reflectance and MultiColor imaging in all 36 eyes at every imaging encounter, showing minimal and subtle variability in its shape and location within each eye; however, it showed large interindividual variability in size, shape, location, and reflectivity between different eyes. Nine eyes were found to have a similar hyper-reflective spot resembling that in ghost maculopathy, but corresponding SD OCT images were consistent with diagnoses of choroidal nevus, age-related macular degeneration, and multifocal choroiditis. All eyes with ghost maculopathy were found to be pseudophakic with a posterior chamber intraocular lens., Conclusion: Ghost maculopathy is the phenomenon of an imaging artifact appearing at the macula on near-infrared reflectance and MultiColor imaging that occurs predominantly in pseudophakic patients and may be mistaken for true chorioretinal pathology. Awareness of this artifact is prudent to avoid misinterpretation of clinical findings and possible unnecessary over-investigation., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: To evaluate clinical manifestations of patients with uveitis and scleritis of unknown origin and positive QuantiFERON-TB Gold In-Tube test (quantiferon) in a country not endemic for tuberculosis., Design: Multicenter retrospective cohort study., Methods: Retrospective review of the clinical, laboratory, and imaging data of 77 patients. Main outcome measures consisted of ocular and systemic features as well as results of laboratory examinations., Results: Out of all, 60 of 71 (85%) were living for at least 6 months in tuberculosis-endemic regions. Location of uveitis was variable; posterior uveitis (29/77; 38%) was the most frequent. Two clinical entities were commonly noted: retinal occlusive vasculitis (21/77; 27%) and serpiginoid choroiditis (11/77; 14%). Antituberculosis treatment was completed in 32 patients; 29 of them (91%) achieved complete remission. Mean quantiferon level was 7.5 U/mL; 71% had values above 2 U/mL and 41% above 10 U/mL. We observed no associations between quantiferon levels and clinical and/or imaging features. Previous tuberculosis infection was diagnosed in 5 of 77 patients (6.5%), while hilar/mediastinal lymphadenopathy was found in 25 of 76 patients (33%). Of these, 12 were consistent with the diagnosis of sarcoidosis, 9 were typical for (prior) tuberculosis, and 4 were compatible with both diagnoses., Conclusions: Ocular features of patients with idiopathic uveitis and positive quantiferon were diverse, but retinal occlusive vasculitis and serpiginoid choroiditis were common. The quantiferon levels were usually highly elevated and 33% of patients exhibited lymphadenopathy, suggesting frequently the diagnosis of sarcoidosis. Ocular inflammation reacted favorably to antituberculosis treatment, although only a small minority had documented (prior) tuberculosis., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: To determine ocular signs predictive of tubercular uveitis., Design: Retrospective, nonrandomized, comparative interventional case study., Methods: Three hundred eighty-six patients with active uveitis were treated at a tertiary care single-center uveitis practice. Uveitis was presumed to be tubercular in patients who showed evidence of latent or manifest tuberculosis without any other known cause and who did not show recurrence of uveitis after 12 months of antitubercular therapy. One hundred eighty-two patients who thus obtained clinical diagnoses of presumed tubercular uveitis were enrolled in group A. Two hundred four patients with uveitis resulting from a nontubercular cause were enrolled in group B. Patients were monitored for the presence of types of keratic precipitates (mutton fat or fine), posterior synechiae (broad based or filiform), iris nodules, snowballs, snow banking, vasculitis (with or without choroiditis), serpiginous-like choroiditis, and other types of posterior uveitis (choroidal abscess, retinochoroiditis, or exudative retinal detachment) which were compared between the 2 groups. Statistical analysis was carried out at a 5% level of significance. The main outcome measures were clinical signs significantly associated with tubercular uveitis., Results: Broad-based posterior synechiae, retinal vasculitis with or without choroiditis, and serpiginous-like choroiditis were seen significantly more commonly in patients with tubercular uveitis. Filiform posterior synechiae were more frequent in eyes with nontubercular uveitis., Conclusions: Broad-based posterior synechiae, retinal vasculitis with or without choroiditis, and serpiginous-like choroiditis in patients with latent or manifest tuberculosis in tuberculosis-endemic areas are suggestive of a tubercular cause of uveitis and merit specific treatment., (Copyright 2010 Elsevier Inc. All rights reserved.)

Purpose: To determine whether clinically isolated anterior segment recurrence is associated with concomitant subclinical choroidal inflammation in Vogt-Koyanagi-Harada (VKH) disease., Design: Interventional, retrospective case series., Methods: Nine patients with recurrent VKH disease who satisfied the following criteria: 1) presence of anterior chamber (AC) inflammation of at least 1+ cells after a period of quiescence of three months or more, 2) no posterior segment involvement, and 3) indocyanine green angiography (ICGA) performed during the time of anterior segment recurrence at the Singapore National Eye Centre. ICGA was performed. Therapy was administered based on clinical features. The main outcome measures were the ICGA features., Results: Twelve eyes of nine patients were included in the study. All eyes had mild AC inflammation of 1 to 2+ cells, except for one eye that had 3+ cells. ICGA showed early large vessel hyperfluorescence, becoming fuzzy in character in the intermediate phase in 11 of 12 eyes with AC inflammation. In four eyes, few hypofluorescent dots were seen in the intermediate phase that remained hypofluorescent in the late phases. Late diffuse hyperfluorescence was observed in five eyes. All patients received high-dose oral prednisolone and topical prednisolone acetate. Four patients required additional steroid-sparing immunosuppressants. All patients attained clinical quiescence within two weeks to two months. Repeat ICGA showed absence of active leakage, significant decrease in choroidal vascular leakage, or no change in the number and density of hypofluorescent spots., Conclusions: Isolated anterior segment recurrence in VKH disease may occur concomitantly with subclinical choroidal inflammation and requires systemic therapy.

Purpose: To report an uncommon case of multifocal choroiditis as the result of disseminated Cryptococcus neoformans infection in a patient who is HIV-positive., Design: Interventional case report., Methods: A 27-year-old HIV-positive woman with fever, headache, and vomiting was examined. Lumbar puncture was performed and revealed C neoformans infection. Her condition evolved with sudden bilateral blindness and deafness. Ophthalmologic examination revealed multiple yellowish choroidal lesions in the posterior pole of both eyes., Results: Postmortem examination showed disseminated C neoformans infection. Histologic examination of the eyes confirmed the presence of C neoformans in the choroiditis., Conclusion: Multifocal choroiditis in C neoformans infection is a rare ophthalmic manifestation. The recognition of this condition by ophthalmologists can help physicians to diagnose a disseminated and fatal disease.

Five patients (all female, 14 to 34 years old) had an unusual form of multifocal choroiditis resulting in progressive subretinal fibrosis. This condition differed from other multifocal choroidal diseases such as acute multifocal placoid pigment epitheliopathy, birdshot retinochoroidopathy, and punctate inner choroiditis. Instead of forming atrophic or pigmented chorioretinal scars, the acute lesion healed with the formation of discrete, sharply angulated subretinal scars. In the macula the lesions coalesced, forming broad zones of subretinal fibrosis. Serous and hemorrhagic macular detachment may occur during the course of the disease with fluorescein angiographic features suggesting subretinal neovascularization. Subretinal neovascularization could not, however, explain the widespread scarring. Although progressive subretinal fibrosis may be considered an unusual outcome for a variety of other diseases, we believe that it is a distinct clinical entity distinguishable from other multifocal choroidal diseases. Retinal pigment epithelial hyperplasia may cause the condition.

A father and son with pigmented paravenous retinochoroidal atrophy demonstrated the classical fundus appearance of bilateral sharply circumscribed patches of retinochoroidal atrophy and pigmentation along the retinal veins. Our patients, and the preponderance of males in previously reported cases of this disease, suggest the possibility of Y-chromosome mediated male-to-male transmission. Electrophysiologic findings in our patients included elevated dark-adaptation thresholds, slightly reduced electroretinographic amplitudes, slightly prolonged a- and b-wave implicit times, and (in Case 1) abnormal electro-oculographic ratios. These findings indicate a diffuse tapetoretinal degeneration, and suggest that pigmented paravenous retinochoroidal atrophy is another incomplete form of retinitis pigmentosa.

Retinal pigment epithelial detachment and optic disk neovascularization developed in two men, 51 and 49 years old, who had serpiginous choroiditis. Fluorescein angiograms demonstrated loss of pigment epithelium and choriocapillaris in the late stages in both cases. Both men sustained permanent visual loss. In one case the final visual acuity of the affected eye was 20/200. In the second case the final visual acuity was R.E.: counting fingers at 4 feet and L.E.: 20/70.

Serpiginous and geographic choroiditis, one and the same disease, is characterized by episodic involvement of the pigment epithelium and choroid. Each new lesion passes through an acute phase into an atrophic and scarring one. We observed 20 patients, some with long-term follow-up; some had eventual widespread involvement of both posterior poles. There was no basis for the disease, and treatment did not affect its course. In the patients with long-term disease there was widespread atrophy of the choroid and pigment epithelium and variable amounts of pigment clumping and subretinal fibrous tissue deposition. In nine patients discrete peripheral patches of atrophy were also found. In some eyes the atrophy at the posterior pole had become so confluent that the condition could have been mistaken superficially for a primary choroidal dystrophy. The uninvolved areas appeared to be normal. The electroretinogram and electrooculogram were only affected when extensive disease was present.

We studied 40 patients with the presumed ocular histoplasmosis triad for ten to 15 years. Another patient developed the choroidal features of the syndrome in previously normal eyes. The basic lesion is a nodular choroidal infiltrate that evolves from clinically and angiographically normal retina. It is initially hypofluorescent and has an overlying serous detachment. It evolves through episodes of serous detachment and hyperfluorescence into a choroidal scar with breaks in Bruch's membrane and occasional subretinal neovascularization. Seven of 42 eyes (16.6%) developed new choroidal infiltrates in choroid and retina that were clinically and angiographically normal more than ten years ago. After ten years about 50% of eyes with central scars will have visual acuity changes of 3 or more lines because of progressive remodeling and extension of the disciform scar. Nine of 40 fellow eyes (22.5%) sustained a disciform process during the entire follow-up period. One patient developed typical choroidal lesions in previously normal fundi. Results of extensive physical and laboratory tests for histoplasmosis and other diseases were normal.