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Objectives: We aimed to outline the demographic data, clinical spectrum, and treatment approach of sarcoidosis in a large group of patients and sought to figure out the variations of early-onset (EOS) and late-onset paediatric sarcoidosis (LOS)., Methods: The study followed a retrospective-descriptive design, with the analysis of medical records of cases diagnosed as paediatric sarcoidosis., Results: Fifty-two patients were included in the study. The median age at disease onset and follow-up duration were 83 (28.2-119) and 24 (6-48) months, respectively. Ten (19.2%) cases had EOS (before 5th birthday) and 42 (80.7%) cases had LOS. The most common clinical findings at the time of the disease onset were ocular symptoms (40.4%) followed by joint manifestation (25%), dermatological symptoms (13.5%), and features related to multi-organ involvement (11.5%). Anterior uveitis was the most common (55%) one among ocular manifestations. Patients with EOS displayed joint, eye, and dermatological findings more commonly than patients with LOS. The recurrence rate of disease in patients with EOS (5.7%) and LOS (21.1%) were not statistically different (P = .7)., Conclusions: Patients with EOS and LOS may present with variable clinical features and studies addressing paediatric sarcoidosis cases in collaboration between disciplines will enhance the awareness of this rare disease among physicians and assist early diagnosis with lesser complications., (© Japan College of Rheumatology 2023. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Aims: To investigate the correlation between widefield fundus fluorescein angiography (WF-FA) and anterior chamber laser flare photometry (LFP-flare) in Behçet uveitis (BU)., Methods: Patients with BU who underwent Heidelberg WF-FA between March 2016 and March 2018 were included. Demographics, ocular findings, LFP-flare, central macular thickness were retrospectively analyzed., Results: Eighty-six patients (162 eyes) were included. There was a high correlation between WF-FA leakage score and LFP-flare (rho: 0.691, p < .001). For a flare value of 5.75 ph/ms or higher, the sensitivity for predicting angiographic leakage was 70% and the specificity was 94% (AUC: 0.897, p < .001). Flare values that were equal or above 7.6 ph/ms were found to be 100% specific for leakage in the WF-FA., Conclusion: A strong correlation was found between WF-FA leakage and LFP-flare in patients with BU. High LFP-flare levels indicate subclinical posterior segment inflammation in clinically inactive BU.

Purpose : To develop an algorithm for the diagnosis of Behçet's disease (BD) uveitis based on ocular findings. Methods : Following an initial survey among uveitis experts, we collected multi-center retrospective data on 211 patients with BD uveitis and 207 patients with other uveitides, and identified ocular findings with a high diagnostic odds ratio (DOR). Subsequently, we collected multi-center prospective data on 127 patients with BD uveitis and 322 controls and developed a diagnostic algorithm using Classification and Regression Tree (CART) analysis and expert opinion. Results : We identified 10 items with DOR >5. The items that provided the highest accuracy in CART analysis included superficial retinal infiltrate, signs of occlusive retinal vasculitis, and diffuse retinal capillary leakage as well as the absence of granulomatous anterior uveitis or choroiditis in patients with vitritis. Conclusion : This study provides a diagnostic tree for BD uveitis that needs to be validated in future studies.

Purpose: To investigate the effect of interferon alfa-2a on T regulatory (Treg) cells, T helper 17 (Th17) cells, and expression of Toll-like receptors (TLRs) in Behçet disease (BD) patients with uveitis., Methods: Twenty-seven patients who received interferon alfa-2a for active BD uveitis despite conventional immunomodulatory therapies and healthy controls were enrolled. Peripheral blood Treg and Th17 cell frequencies were determined by flow cytometry as gated cells for CD3+CD4+Foxp3+ and CD3+CD4+IL17A+, respectively. Th17 RAR-related orphan receptor (ROR)γt mRNA expression was verified by real-time PCR (RT-PCR). Treg and Th17 cell cytokines were detected by ELISA in the supernatant of short-term cell cultures. RT-PCR was used to assess expression of TLR-2, TLR-3, TLR-4, TLR-8, and TLR-9 using cDNA prepared from CD4+ T cells and monocytes., Results: Treg and Th17 cell frequencies and Th17 RORγt expression were significantly elevated, and IL-10 concentration in Treg cell supernatants was significantly lower in BD patients than in controls. Th17 IL-17, IL-6, IL-21, IL-22, IL-23, IFN-γ, and TNF-α concentrations were significantly higher and all TLR expressions were significantly elevated in patients. Interferon alfa-2a led to a significant reversal in Treg and Th17 cell frequencies, Th17 RORγt expression, Treg and Th17 cell cytokine production, and TLR expression by CD4+ T cells and monocytes., Conclusions: Despite a relative increase in Treg cells, impaired IL-10 production suggests that Treg dysfunction may play a role in induction of BD uveitis. Favorable effects of interferon alfa-2a may be associated with recovery of Treg cell function, suppression of Th17 cells, and reduced expression of TLRs on CD4+ T cells and monocytes.

Purpose: To screen for psychological disorders in patients with active uveitis., Methods: Patients were screened for depression (BDI-II), state anxiety (STAI-I), VR-QOL (NEI-VFQ-25), and HR-QOL (SF-36). Association of depression and anxiety with sociodemographic and clinical parameters and with VR-QOL and HR-QOL were analyzed. Multivariate linear regression models were constructed for NEI-VFQ-25 and SF-36 subscales., Results: Of 99 patients, 37.3% screened positive for depression and 52.5% for anxiety. Depressed patients had lower visual acuity in the better seeing eye (p = 0.013) and more frequently panuveitis (p = 0.018). Anxious patients were younger (p = 0.009), had earlier onset of uveitis (p = 0.015), and had more frequently panuveitis (p = 0.016). Bivariate comparisons showed significant associations between psychological disorders and VR-QOL and HR-QOL. Significant bivariate associations were mostly lost in multivariate analyses for anxiety, but were preserved for depression., Conclusions: A positive screening test for depression and anxiety is common in patients with uveitis. Low vision and panuveitis are associated with depression. Depression is associated with impairment of VR-QOL and HR-QOL.

Behçet disease is a chronic relapsing multisystem inflammatory disorder. Ocular involvement is characterized by a bilateral recurrent non-granulomatous panuveitis and retinal vasculitis. Posterior segment findings vary during the course of the disease, in parallel with the relapsing and remitting intraocular inflammation. Structural alterations occur with increased disease duration. Fluorescein angiography is the gold standard in revealing the extent and severity as well as the leaky and/or occlusive nature of retinal vasculitis. Multimodal imaging using color fundus photography, fluorescein angiography, and optical coherence tomography is essential in visualizing diagnostic features, detecting structural changes, and monitoring disease activity and response to treatment in patients with Behçet uveitis.

Purpose: To report the outcomes of tocilizumab treatment for refractory ocular inflammatory diseases., Methods: A retrospective case series of 17 patients (28 eyes) diagnosed with recalcitrant ocular inflammatory diseases including uveitis (10 cases), scleritis (six cases) and orbital pseudotumour (one case), who received tocilizumab between April 2010 and March 2015. All patients were initiated with treatment of 4 mg/kg or 8 mg/kg tocilizumab. The primary outcome was absence of inflammation and achievement of steroid sparing at 6 and 9 months. Secondary outcomes were change in visual acuity and major adverse effects of tocilizumab causing discontinuation of the treatment., Results: Mean age at initiation of tocilizumab was 41 ± 16 years. Prior to tocilizumab treatment, all patients underwent unsuccessful conventional immunosuppressive therapy while 94% of patients (16/17) failed treatment with various biological agents. After tocilizumab administration, control of inflammation and steroid sparing were achieved in 63% and 71% of uveitis patients at 6 and 9 months, while 50% of scleritis patients achieved the primary outcome at 6 and 9 months. Mean duration of tocilizumab therapy was 12.6 ± 10.0 (range, 2-35) months. Three of four patients who had a follow-up of at least 18 (range, 18-35) months experienced quiescent inflammation for up to 32 months of tocilizumab use until last visit. Four patients (24%) discontinued tocilizumab due to serious side effects including neutropenia, unacceptable dizziness and nausea, severe angioedema and severe abdominal pain., Conclusion: Our series demonstrated moderate efficacy of tocilizumab in recalcitrant uveitis and scleritis. Serious adverse effects were not uncommon., (© 2016 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To describe clinical manifestations, diagnostic approaches, therapy, and outcomes of biopsy-proven intraocular lymphoma.Methods: Review of tertiary referral center records between 2005 and 2015.Results: A total of 51 eyes of 26 patients were included; mean age of onset was 60.42 years. Common ocular complaints included floaters (42%) and blurred vision (35%); 62% of patients had ocular and central nervous system involvement; 11% had systemic lymphoma; and 27% had only ocular involvement. Vitreous analysis was positive for malignant cells in 77% of patients on initial biopsy, and in 100% of patients on repeat biopsy. In total, 20/26 patients received systemic and topical treatment before IOL diagnosis was made; 25 patients received intravitreal methotrexate and/or rituximab; one patient received intracameral rituximab. All patients achieved remission by their final visit.Conclusions: Intraocular lymphoma often masquerades as intraocular inflammation, resulting in delayed or misdiagnosis with subsequent inappropriate management. Optimal therapy is a challenge for oncologists and ophthalmologists. [ABSTRACT FROM AUTHOR]

Purpose: To report the efficacy and safety of interferon (IFN) α-2a in patients with cystoid macular edema (CME) associated with presumed ocular tuberculosis (TB).Methods: We reviewed the clinical records of 5 patients with presumed ocular TB who had been treated with IFN α-2a for recurrent CME during or after completion of anti-tubercular therapy. IFN α-2a was administered at an initial dose of 3 million IU per day and then tapered after the initial response. Treatment efficacy was assessed by central macular thickness (CMT) measurement using spectral-domain optical coherence tomography and visual acuity.Results: Three patients were men, and 2 were women. Patients were aged between 38 and 66 years. Mean CMT was 483 ± 178.6 μm at baseline, 302.3 ± 56 μm at 1 week, 312.3 ± 49.5 μm at 1 month, and 286.2 ± 31.9 μm at 3 months. Mean LogMAR visual acuity was 0.6 ± 0.4 at baseline, 0.4 ± 0.3 μm at 1 week, 0.3 ± 0.3 at 1 month, and 0.3 ± 0.3 at 3 months. The treatment was interrupted for 10 days because of neutropenia after 2 weeks in 1 patient and discontinued in another after 10 days because of intolerance. Total treatment duration was 3-24 months in the remaining 4 patients.Conclusion: The present small case series suggests that IFN α-2a may be an effective and safe therapeutic option for CME that is associated with presumed ocular TB. [ABSTRACT FROM AUTHOR]

Purpose: To describe the clinical and visual outcomes of juvenile idiopathic arthritis (JIA)-associated uveitis in adults and to examine risk factors for ongoing inflammation in adulthood. Methods: Medical records were reviewed for patients with JIA-associated uveitis who were >16 years old at the final visit (the last visit prior to data collection). Results: In total, 135 eyes of 77 patients (70 female, 7 male) were included. The mean age of patients at the final visit was 29.72 ± 11.27 years. The number of eyes with visual acuity of ≤20/50 and ≤20/200 at the final visit was 37 (28 %) and 20 (15 %), respectively; at least one ocular complication was present in 72 % of eyes. Band keratopathy was the most frequent complication (42 %), followed by cataract (25 %), posterior synechiae (22 %), maculopathy (22 %), ocular hypertension (13 %), and hypotony (5 %). At the final visit, patients who were >16 years of age at presentation to the Massachusetts Eye Research and Surgery Institution had more ocular complications and a greater degree of vision loss than patients who were ≤16 years of age. Ongoing inflammation at the final visit was noted in 40 patients (52 %). The presence of posterior synechiae, hypotony, cataract at presentation, and a history of cataract surgery prior to presentation were predictive of ongoing inflammation in adulthood in univariate analysis. The presence of hypotony and posterior synechiae at the initial visit were predictive factors in multivariate analysis. Conclusions: JIA-associated uveitis may be associated with ongoing inflammation, ocular complications, and severe visual impairment in adulthood. The presence of posterior synechiae and hypotony at the initial visit is predictive of ongoing inflammation. [ABSTRACT FROM AUTHOR]

Pediatric uveitis may be a serious health problem because of the lifetime burden of vision loss due to severe complications if the problem is not adequately treated. Juvenile idiopathic arthritis (JIA)-associated uveitis is characterized by insidious onset and potentially blinding chronic anterior uveitis. Periodic ophthalmologic screening is of utmost importance for early diagnosis of uveitis. Early diagnosis and proper immunomodulatory treatment are essential for good visual prognosis. The goal of treatment is to achieve enduring drug-free remission. The choice of therapeutic regimen needs to be tailored to each individual case. One must keep in mind that patients under immunomodulatory treatment should be monitored closely due to possible side effects. Local and systemic corticosteroids have long been the mainstay of therapy; however, long-term corticosteroid therapy should be avoided due to serious side effects. Steroid-sparing agents in the treatment of JIA-associated uveitis include antimetabolites and biologic agents in refractory cases. Among the various immunomodulatory agents, methotrexate is generally the first choice, as it has a well-established safety and efficacy profile in pediatric cases and does not appear to increase the risk of cancer. Other classic immunomodulators that may also be used in combination with methotrexate include azathioprine, mycophenolate mofetil, and cyclosporin A. Biologic agents, primarily tumor necrosis factor alpha inhibitors including infliximab or adalimumab, should be considered in cases of treatment failure with classic immunomodulatory agents. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the effect of phacoemulsification with intraocular lens (IOL) implantation on intraocular inflammation using laser flare photometer in eyes with uveitis. Materials and Methods: Sixty-six eyes of 55 consecutive patients followed-up at Istanbul University, Istanbul School of Medicine, Department of Ophthalmology, Uveitis Service undergoing phacoemulsification with posterior chamber intraocular lens implantation between December 2009 and April 2013 were included in this retrospective study. Clinical records of patients were analyzed for demographic data, pre-operative and post-operative best-corrected visual acuity (BCVA), intraocular pressure, and quantitative measurement of inflammation with laser flare photometry. Baseline evaluations were performed 1 day before surgery and follow-up examinations took place at 1, 7, 30, and 90 days after surgery. Main outcome measures were BCVA and quantitative measurement of intraocular inflammation with laser flare photometry. Results: Best-corrected visual acuity improved in 60 eyes and visual loss did not occur in any eye. Median intraocular flare value the day before surgery was 8.2 (range: 3.1-82.2) photons/ms. Postoperatively, median flare value initially increased to 16.8 (range: 0-158) photons/ms on day 1 which was a statistically significant increase compared to preoperative values. Thereafter decreased to 10.40 (range: 0-110) photons/ms on day 7, 11.25 (range: 4.4-145) photons/ms on day 30, and 8.1 (range: 0-84.6) photons/ms on day 90. There was no statistically significant difference between preoperative values and day 90. Conclusion: In uveitic eyes with well-controlled ocular inflammation before surgery, phacoemulsification with IOL implantation appears safe and effective as assessed by changes in the BCVA and laser flare photometry, an objective method to measure intraocular inflammation. [ABSTRACT FROM AUTHOR]