Fluorescein Angiography Score Research Articles

Outcomes of Intravenous Tocilizumab Treatment for Refractory Pars Planitis

ABSTRACT Purpose To evaluate outcomes of intravenous (IV) tocilizumab (TCZ) in patients with pars planitis refractory to conventional immunomodulatory therapy and anti-tumor necrosis factor (TNF) alpha agents. Methods Medical records of eight patients diagnosed with pars planitis and treated with monthly 4 or 8 mg/kg IV TCZ were reviewed. The primary objective was to initiate and sustain remission continuously for three consecutive months. Secondary outcome measures were changes in best corrected visual acuity (BCVA), degree of anterior chamber (AC) inflammation, vitreous cell, vitreous haze, presence of vitreous or pars plana exudates, peripheral vasculitis, fluorescein angiography (FA) score and central subfieldthickness (CST) on macular optical coherence tomography (OCT). Results Fourteen eyes of eight patients were treated with IV TCZ. Seven patients were women. The average age was 31.35 ± 16.42 years. In 6 (75%) out of 8 patients, IV TCZ, either as monotherapy or in combination with another conventional immunomodulatory agent, induced and sustained remission. The average FA score reduced from 11.15 ± 3.52 at the baseline visit to 6.50 ± 2.12 at the one-year follow-up visit (p-value < 0.05). None of the patients experienced any side effects of IV TCZ. Conclusion IV Tocilizumab (TCZ) may represent an effective and safe treatment option for patients diagnosed with pars planitis resistant to conventional immunomodulatory therapy and anti-TNF alpha agents.

The Efficacy of Adalimumab in Children with Chronic Non-infectious Posterior Uveitis and Panuveitis: A Retrospective Cohort Study.

This study aimed to assess the efficacy and safety of adalimumab in pediatric patients with chronic non-infectious posterior uveitis and panuveitis (not associated with juvenile idiopathic arthritis). The medical records of children (< 18years old) with chronic non-infectious posterior uveitis and panuveitis were collected and analyzed in this retrospective cohort study. Children were allocated to a conventional adalimumab-free treatment (CT) or adalimumab (ADA) group based on whether they additionally received adalimumab. In total, 69 children (138 eyes) were included, with 21 (42 eyes) and 48 (96 eyes) in the CT and ADA groups, respectively. During the average follow-up period of 24months, the improvement in all ocular parameters (best-corrected visual acuity, intraocular inflammation, fluorescein angiography score) was better in the ADA group than in the CT group, except for changes in central macular thickness, which did not significantly differ between the groups. The mean time of first alleviation, which was after 1.03 ± 0.12months of therapy, was earlier in the ADA group than in the CT group (2.30 ± 0.46months). In the ADA group, 90.6% of children had remission within 3months, and 47.9% had no relapse during follow-up. Cough and cold were the most common adverse events in the ADA group; however, the number of adverse events was similar between both the groups. Adalimumab was effective in the treatment of chronic noninfectious posterior uveitis and panuveitis in pediatric patients, and disease inactivity was accomplished in the majority of the patients, thereby improving visual outcomes and maintaining disease stability. Adverse events were limited and tolerable.

Non-invasive biomarkers for close activity monitoring in birdshot chorioretinitis: Optical coherence tomography angiography and beyond.

The purpose of the study was to identify non-invasive imaging biomarkers potentially useful for close activity monitoring in birdshot chorioretinitis (BSCR). Cross-sectional study of BSCR eyes included as per Levinson's and/or SUN criteria. Eyes were blindly classified into active or inactive groups per clinical inflammatory parameters, ultra-widefield (UWF) pseudocolour images, UWF fluorescein angiography (FA) and macular optical coherence tomography (OCT) cube. Qualitative and quantitative OCT and OCT-angiography (OCT-A) parameters at the fundus, superonasal and inferonasal fields were compared between active and inactive eyes. Thirty consecutive BSCR patients (60 eyes) were analysed. 28 eyes (46.66%) were from women and the overall mean age was 59.7 ± 12.3 years. Active eyes showed an abnormal retinal thickening at inferonasal field (nasal retinal thickness) and a higher averaged thickened retinal index (ATRI) (72.36 active vs. 20.12 inactive, p < 0.0001). A significant moderate correlation was observed between ATRI and FA scores (r = 0.259, p = 0.022). Macular vascular loops were more frequent in the superficial vascular plexus of OCT-A in the active eyes (p = 0.028). The vascular perfusion index tended to be higher in all subfields of active eyes but did not reach statistical significance. Multimodal imaging could be key to discerning activity in BSCR eyes. Higher ATRI and the presence of vascular loops in the superficial plexus are potential non-invasive activity biomarkers for the close monitoring of BSCR.

Intravitreal dexamethasone implants facilitate the management of refractory Behçet's uveitis with vasculitis

To investigate the efficacy and safety of dexamethasone (DEX) implant, Ozurdex ®, as an adjunctive treatment for refractory Behçet's uveitis (BU), a total of 61 patients (80 eyes) were included in this cross-sectional study and divided into the non-DEX and DEX groups. After >12 months of treatment, the improvement in the fluorescein angiography score and vitritis score was significantly higher in the DEX group than in the non-DEX group. Although the posterior capsule opacification score was exacerbated, the rate of low-dose systemic glucocorticoid was higher and the relapse times were fewer in the DEX group. Therefore, Ozurdex® is an effective and safe option for patients with BU that are refractory to systemic immunosuppressant treatments by controlling vasculitis, stabilizing vitreous inflammation, preventing recurrence, and reducing daily glucocorticoid doses.

Efficacy and Safety of Tocilizumab in the Management of Non-Infectious Uveitis Failed with Conventional Immunomodulatory and Anti-TNFα Therapies

ABSTRACT Purpose To determine the outcomes of intravenous (IV) tocilizumab (TCZ) in patients with non-infectious uveitis who failed with conventional immunomodulatory and anti-TNFα therapies. Methods Records of seven patients with non-infectious uveitis treated with monthly IV TCZ (4–10 mg/kg) or biweekly IV TCZ (8 mg/kg) were reviewed. Outcome measures were changes in visual acuity, anterior chamber cell and flare grade, vitreous haze, central subfield thickness (CST), and fluorescein angiography (FA) score. Results Ten eyes of seven patients received TCZ therapy. Median age of patients was 14 (range, 7–24) years. Median duration of TCZ therapy was 15 (range, 5–32) months. Mean CST reduced from 373 ± 101.0 μm to 298.2 ± 40.3 μm. Mean FA score reduced from 12.5 ± 4.3 to 3.6 ± 2.6. One patient developed elevated liver transaminases. Conclusion IV TCZ is a potentially effective and safe therapeutic option for the management of refractory non-infectious uveitis.

Six-month outcomes of infliximab and tocilizumab therapy in non-infectious retinal vasculitis.

To evaluate the efficacy of infliximab (IFX, 5-10 mg/kg) (Group 1) and tocilizumab (TCZ, 4-8 mg/kg) (Group 2) infusions in non-infectious retinal vasculitis (RV) using Angiographic Scoring for the Uveitis Working Group fluorescein angiography (FA) scoring system. Records of 14 patients (24 eyes) in Group 1 and 8 patients (11 eyes) in Group 2 were retrospectively evaluated to assess visual acuity (VA), anterior chamber cell and flare, vitreous haze, central subfield thickness (CST), and FA scoring at baseline and 6 months of follow-up. The measurements were employed to grade in each group. In Group 1 and 2, respectively, there was no underlying disease in 9 (60%) and 3 (42.9%) patients. Three (42.9%) patients in Group 2 had juvenile idiopathic arthritis (JIA) as the most common identified cause. Mean improvement in VA (log MAR) and CST were 0.04 ± 0.14 and 40.3 ± 78.5 µm in Group 1; 0.04 ± 0.09 and 47.3 ± 82.3 µm in Group 2, respectively. Mean FA scores were significantly reduced from 12.4 ± 5.2 and 11.6 ± 4.4 at baseline to 6.4 ± 5.0 and 5.8 ± 3.9 at 6-month in Group 1 and 2, respectively. In Group 2, 9 eyes of 6 patients (75%) had the history of IFX use prior to TCZ initiation. There was no significant safety concern requiring treatment discontinuation during the follow-up in either group. IFX and TCZ infusions showed statistically significant improvement of non-infectious RV as shown by ASUWOG FA Scoring System. TCZ, as well as IFX, appeared to be effective treatment options for non-infectious RV.

Characterization of Fluorescein Angiography Features in Pediatric Patients with Uveitis

ABSTRACT Purpose To characterize fluorescein angiography (FA) features in pediatric uveitis. Methods A retrospective cross-sectional study of pediatric uveitis patients in Atlanta, GA, who had FA imaging between May 2009 and August 2020. Results A total of 137 eyes of 88 patients were evaluated. The mean FA score in anterior uveitis was lower than that in other categories (p = .0093). The mean FA score in the clinically active group was higher than that in the clinically inactive group (p = .0057). The mean FA score in eyes 20/40 or better was lower than that in other categories (p = .0292). Worse visual acuity was associated with retinal vascular staining/leakage, retinal staining/pooling, and neovascularization elsewhere. Non-infectious uveitis more often demonstrated optic disc hyperfluorescence, retinal vascular staining/leakage, capillary leakage, and pinpoint leaks, while infectious uveitis more often demonstrated retinal staining/pooling. Conclusion A standardized quantitative FA scoring system can be useful in the characterization of uveitis in pediatric patients.

Characterization and validation of a chronic retinal neovascularization rabbit model by evaluating the efficacy of anti-angiogenic and anti-inflammatory drugs

To establish a rabbit model with chronic condition of retinal neovascularization (RNV) induced by intravitreal (IVT) injection of DL-2-aminoadipic acid (DL-AAA), a retinal glial (Müller) cell toxin, extensive characterization of DL-AAA induced angiographic features and the suitability of the model to evaluate anti-angiogenic and anti-inflammatory therapies for ocular vascular diseases. DL-AAA (80 mmol/L) was administered IVT into both eyes of Dutch Belted rabbit. Post DL-AAA delivery, clinical ophthalmic examinations were performed weekly following modified McDonald-Shadduck Scoring System. Color fundus photography, fluorescein angiography (FA), and optical coherence tomography (OCT) procedures were performed every 2 or 4wk until stable retinal vascular leakage was observed. Once stable retinal leakage (12wk post DL-AAA administration) was established, anti-vascular endothelial growth factor (VEGF) (bevacizumab, ranibizumab and aflibercept) and anti-inflammatory (triamcinolone, TAA) drugs were tested for their efficacy after IVT administration. Fluorescein angiograms were scored before and after treatment following a novel grading system, developed for the DL-AAA rabbit model. Post DL-AAA administration, eyes were presented with moderate to severe retinal/choroidal inflammation which was accompanied by intense vitreous flare and presence of inflammatory cells in the vitreous humor. Retinal hemorrhage was restricted to the tips of neo-retinal vessels. FA revealed maximum retinal vascular leakage at 2wk after DL-AAA injection and then persisted as evidenced by stable mean FA scores in weeks 8 and 12. Retinal vascular angiographic and tomographic features were stable and consistent up to 36mo among two different staggers induced for RNV at two different occasions. Day 7, mean FA scores showed that 1 µg/eye of bevacizumab, ranibizumab, aflibercept and 2 µg/eye of TAA suppress 65%, 90%, 100% and 50% retinal vascular leakage, respectively. Day 30, bevacizumab and TAA continued to show 66% and 44% suppression while ranibizumab effect was becoming less effective (68%). In contrast, aflibercept was still able to fully (100%) suppress vascular leakage on day 30. On day 60, bevacizumab, ranibizumab and TAA showed suppression of 7%, 12%, and 9% retinal vascular leakage, respectively, however, aflibercept continued to be more effective showing 50% suppression of vascular leakage. The DL-AAA rabbit model mimics RNV angiographic features like RNV and chronic retinal leakage. Based on these features the DL-AAA rabbit model provides an invaluable tool that could be used to test the therapeutic efficacy and duration of action of novel anti-angiogenic formulations, alone or in combination with anti-inflammatory compounds.

The Efficacy of Adalimumab as an Initial Treatment in Patients with Behçet’s Retinal Vasculitis

Background: No study has evaluated the effectiveness of Adalimumab (ADA) as first-line in treatment-naïve patients with retinal vasculitis due to Behçet’s Uveitis (BU). Objective: To compare the efficacy of ADA plus conventional therapy and conventional therapy alone as initial treatments in naïve BU patients characterized by retinal vasculitis. Methods: Medical records of BU patients characterized by retinal vasculitis treated with conventional therapy (CT, refers to glucocorticoid and immunosuppressive agents) alone or ADA plus conventional therapy with at least 6 months of follow-up between February 2015 and June 2020 were analyzed. Only patients who were first diagnosed with BU without previous systemic treatment were reviewed. The retinal vasculitis score based on fluorescein angiography (FA), best-corrected visual acuity, glucocorticoid-sparing effect, the number of relapses and ocular complications were evaluated. Results: A total of 45 patients (87 eyes) were included. Twenty-four patients (55.33%) in the CT group were treated with conventional therapy and 21 patients (46.67%) in the ADA group were treated with ADA plus conventional therapy. The inflammatory parameters improved in both groups. FA scores showed significantly greater improvement in ADA group than CT group (p < 0.001). The median number of relapses was significantly lower, and the duration of remission was longer in ADA group than CT group (p < 0.001). At the last visit, a significantly better BCVA improvement (p = 0.024), better inflammation control (anterior chamber inflammation p = 0.017 and vitritis p < 0.001) and lower daily glucocorticoid dosage (p = 0.005) were identified in patients received ADA therapy. In CT group, 1 patient suffered hepatitis B and tuberculosis, 1 had growth retardation, 1 patient had with osteoporosis, then followed by other mild AEs (mostly respiratory upper tract infections); while in ADA group, 1 patient experienced a mild pneumonia (n = 1) while milder AEs were represented mostly by respiratory upper tract infections followed by gastrointestinal discomfort. Conclusion: ADA plus conventional therapy achieved superiority over conventional therapy as initial treatment in naïve BU patients with retinal vasculitis.

Clinical Follow-up of Patients with Behçet Uveitis after Discontinuation of Infliximab Therapy

ABSTRACT Purpose To evaluate the clinical course of patients with Behçet uveitis after discontinuation of infliximab (IFX) therapy. Methods Medical records of eight patients who discontinued treatment between 2010 and 2018 were retrospectively analyzed. The main outcome measures were frequency of uveitis attacks per year, best-corrected visual acuity (BCVA), aqueous flare, foveal thickness and fluorescein angiography (FA) scores before initiation, during treatment and after 6, 12, and 24 months of cessation of the IFX therapy. Results The mean follow-up after withdrawal of infusions was 38.6 ± 20.4 (12–90) months. Frequency of uveitis attacks, BCVA, aqueous flare, foveal thickness and FA scores were improved significantly after treatment (p < .05). In terms of these parameters, there was no significant difference between the periods of during treatment and after 6, 12, and 24 months of cessation of the IFX therapy. Conclusion IFX therapy might be discontinued safely with an effective inflammation control in patients with Behçet uveitis.

Quantitative measurements with optical coherence tomography angiography in Behçet uveitis.

The aim of this study was to evaluate the quantitative measurements obtained by optical coherence tomography angiography in patients with Behçet uveitis. A total of 22 consecutive patients with Behçet uveitis and 19 age-matched healthy individuals were included in the study. Quantitative analysis of optical coherence tomography angiography images was performed. Comparisons between the patients and the control groups and correlation analysis between optical coherence tomography angiography results and age, visual acuity, duration of uveitis, central macular thickness, and fluorescein angiography scores in the patient group was performed. The mean superficial foveal avascular zone area was 283.040 ± 113.003 µm2 in the patient group and 254.268 ± 75.813 µm2 in the control group (p = 0.821). The mean deep foveal avascular zone area was 317.657 ± 116.225 µm2 in the patient group and 332.954 ± 94.783 µm2 in the control group (p = 0.775). The foveal avascular zone area could not be calculated in eight eyes with macular atrophy in the patient group because of unclear foveal avascular zone borders. Both superficial and deep capillary vessel density in all regions were lower in the patient group than in the control. Superior, inferior, and temporal capillary vessel density were correlated with fluorescein angiography score (Pearson's r = -0.380, 0.392, and -0.384, p < 0.05 in superficial plexus; r= -0.357, -0.401, and -0.321, p < 0.05 in deep plexus, respectively). The foveal avascular zone area was correlated with central macular thickness in both superficial and deep plexus (r= -0.387 and -0.331, p < 0.05, respectively). Recurrent uveitis attacks affecting the macula are associated with a decrease in capillary vessel density and expansion in the foveal avascular zone area in Behçet disease. The qualitative evaluation of the optical coherence tomography angiography findings may be more valuable in patients with macular atrophy.

Screening and Follow-Up of Acute ROP: Reproducibility of Fluorescein Angiography

Binocular indirect ophthalmoscopy (BIO) is fundamental for screening of retinopathy of prematurity (ROP). Digital retinal imaging devices with fluorescein angiography (FA) proved to be useful in screening and management of ROP. FA provides valuable additional information that is not detectable through ophthalmoscopy. FA images are relatively easy to interpret even by personnel without specific experience in ROP. The aim of this study is to evaluate reproducibility of FA for the screening and follow-up of ROP. A total of 106 pairs of FA images of 30 eyes of 15 premature infants with stage II ROP were evaluated by 5 ophthalmologists: 2 experts, 2 non-experts, and 1 expert in reading FA in adult patients. Each operator gave a score to each of following parameters: leakage, ischemic areas, peripheral plus disease and vascular anomalies. The images were reviewed twice. Intra- and inter-concordance between the readers of the FA findings was evaluated by the means of Cohen's kappa coefficient (κ). The intra-operator concordance was very good (κ > 0.81) for all FA findings. Inter-operator concordance was good (κ> 0.41) for all operators and all FA findings. Global concordance was: substantial (intra-inter readers: κ > 0.61) for leakage, ischemic areas, and plus disease; almost perfect (κ> 0.81) for vascular anomalies; and moderate (κ = 0.41-0.60) for continuity/discontinuity of the ischemic areas. Total FA score was directly correlated to the percentage of treatment: a score ≥ 7 was correlated with 100% treatment and a score ≤ 3 with no treatment. Treatment timing was inversely correlated to FA score: a score ≥ 8 was correlated with a timely treatment (≤ 6days), and a score ≤ 7 was correlated with a delayed treatment (< 10days). This study showed that FA represents a reproducible imaging technique. It is useful for detecting ROP progression, and to define the treatment timing and type.

The Relationship between Fluorescein Angiography Leakage after Infliximab Therapy and Relapse of Ocular Inflammatory Attacks in Ocular Behçet’s Disease Patients

ABSTRACT Purpose: To clarify the relationship between fluorescein angiography (FA) leakage after infliximab therapy and ocular attack relapse in patients with ocular Behçet’s disease (BD). Methods: Patients with ocular BD were divided into two groups based on the presence (Group 1) or absence (Group 2) of ocular attacks after IFX therapy. FA leakage was evaluated by FA score in each of the optic discs, macula, large retinal vessels, and capillary vessels. Receiver operating characteristic (ROC) curve analysis was performed to evaluate the relationship between FA score after IFX therapy and ocular attack relapse. Results: The areas under the curves obtained from the ROC curve of optic disc score and capillary vessels score after IFX therapy were 0.867 (95% confidence interval [CI]: 0.788–0.946) and 0.788 (95% CI: 0.649–0.927), respectively. Conclusions: FA leakage in the optic disc and capillary vessels after IFX therapy was strongly related to ocular attack relapse.

Prolonged Cone b-Wave on Electroretinography Is Associated with Severity of Inflammation in Noninfectious Uveitis

This study sought to investigate retinal function in patients with noninfectious uveitis by using full-field electroretinography (ERG) and correlate the ERG to disease duration and severity of inflammation. Prospective cohort study. Patients (n= 200) with noninfectious uveitis and a disease duration of <1 year (group A [n= 80]) and those with a disease duration of >5 years (group B [n= 120]) were included. ERGs of the total 355 uveitis eyes were measured according to an extended International Society for Clinical Electrophysiology of Vision protocol. ERG abnormalities were related to demographics and uveitis characteristics, including anatomical classification, duration of uveitis, severity of inflammation, best corrected visual acuity (BCVA), cystoid macular edema (CME), and the highest fluorescein angiography (FA) score during the course of the disease. ERGs showed abnormalities in 235 eyes (66.2%). The most frequent and pronounced ERG abnormality was a prolonged implicit time of the cone b-wave (151 eyes [42.5%]), which was associated with vitritis (P= .005); cells in the anterior chamber (P=.007); the highest fluorescein angiography score (P= .011); age (P < 0.001); and pupil diameter (P < 0.001). BCVA was relatively good (0.05 logMAR [interquartile range {IQR}, 0.011, 0.22]) and not associated with this ERG abnormality. There were no differences between the ERG abnormalities in group A and those in group B and no significant associations between ERG abnormalities and anatomical classification or specific diagnoses. ERG results are frequently affected in cases of noninfectious uveitis of all anatomic subtypes, including anterior uveitis without apparent inflammation of the posterior segment. ERG abnormalities appear tobeassociated with the severity of inflammation fromboth the present and the past and therefore may be seen even when signs of retinal inflammation have disappeared.

The Results of Interferon-Alpha Treatment in Behçet Uveitis

ABSTRACTPurpose: To assess the efficacy and tolerability of interferon alpha (IFN-α) treatment in patients with refractory Behçet uveitis (BU).Methods: The patients with BU who were treated with subcutaneous IFN-α were divided into groups according to treatment response. Demographic features, best corrected visual acuity (BCVA), central macular thickness (CMT) value, and fluorescein angiography (FA) scores were compared between the groups.Results: Twenty-one (84%) of the 25 patients responded to IFN-α treatment. Active inflammation resolved at the first month’s visit in all patients. The improvement in mean BCVA and CMT achieved at the first month’s visit in all groups; however, the improvement in mean CMT in the non-responder group could not be maintained at the last visit. Main FA score significantly decreased in both the complete and partial remission group; however, it decreased minimally in the non-responder group.Conclusion: IFN-α seems an effective and safe treatment option in the management of refractory BU.

Sensitivity of indocyanine green angiography compared to fluorescein angiography and enhanced depth imaging optical coherence tomography during tapering and fine-tuning of therapy in primary stromal choroiditis: A case series

PurposeTo investigate indocyanine green angiography (ICGA), fluorescein angiography (FA), and enhanced depth imaging optical coherence tomography measured choroidal thickness (EDI-OCT-CT) in the follow-up of inflammatory activity in stromal choroiditis [Vogt-Koyanagi-Harada disease (VKH) and birdshot retinochoroiditis (BRC)] under treatment in order to monitor tapering of therapy or readjustment of therapy in case of subclinical disease recurrence. MethodsPatients with initial onset disease and/or treatment-naive stromal choroiditis (VKH & BRC) at entry, quiet under therapy, and having had a follow-up of at least four years monitored with dual FA and ICGA and EDI-OCT-CT measurements were analyzed retrospectively. ICGA and FA scores and EDI-OCT-CT values were correlated with therapy, and significant changes of each modality were correlated with disease evolution. ResultsOf the 31 VKH and 29 BRC patients seen from 1995 to 2017 in our center, four patients (2 VKH and 2 BRC patients) fulfilled the inclusion criteria. During tapering, two patients (both VKH) showed no significant ICGA, FA, and EDI-OCT-CT changes (mean follow-up time 5.6 years) and allowed for safe tapering. In the other two (BRC) patients (mean follow-up time 6.25 years), a total of seven significant subclinical changes were demonstrated by ICGA alone after therapy modifications due to side-effects or during attempted tapering of therapy, while FA and EDI-OCT-CT remained unchanged. ConclusionsICGA was the most sensitive monitoring modality of stromal choroiditis, able to identify subclinical recurrences following change of therapy and inversely treatment responses after readjusted therapy, events otherwise missed by FA and EDI-OCT. ICGA proved efficient for safe therapy tapering or for timely adjustment of therapy in stromal choroiditis when necessary.

QUANTITATIVE ANALYSIS OF STRUCTURAL ALTERATIONS IN THE CHOROID OF PATIENTS WITH ACTIVE BEHÇET UVEITIS.

To quantitatively analyze in vivo morphology of subfoveal choroid during an acute attack of Behçet uveitis. In this prospective study, 28 patients with Behçet uveitis of ≤4-year duration, and 28 control subjects underwent enhanced depth imaging optical coherence tomography. A novel custom software was used to calculate choroidal stroma-to-choroidal vessel lumen ratio. Subfoveal choroidal thickness was measured at fovea and 750 μm nasal, temporal, superior, and inferior to fovea. Patients underwent fluorescein angiography and indocyanine green angiography. Receiver operating characteristic curve and area under the curve were computed for central foveal thickness. The eye with a higher Behçet disease ocular attack score 24 was studied. The main outcome measures were choroidal stroma-to-choroidal vessel lumen ratio and choroidal thickness. The mean total Behçet disease ocular attack score 24, fluorescein angiography, and indocyanine green angiography scores were 7.42 ± 4.10, 17.42 ± 6.03, and 0.66 ± 0.73, respectively. Choroidal stroma-to-choroidal vessel lumen ratio was significantly higher in patients (0.413 ± 0.056 vs. 0.351 ± 0.063, P = 0.003). There were no significant differences in subfoveal choroidal thickness between patients and control subjects. Choroidal stroma-to-choroidal vessel lumen ratio correlated with retinal vascular staining and leakage score of fluorescein angiography (r = 0.300, P = 0.036). Central foveal thickness was significantly increased in patients (352.750 ± 107.134 μm vs. 263.500 ± 20.819 μm, P < 0.001). Central foveal thickness showed significant correlations with logarithm of minimum angle of resolution vision, Behçet disease ocular attack score 24, total fluorescein angiography score, retinal vascular staining and/or leakage and capillary leakage scores of fluorescein angiography, and total indocyanine green angiography score. At 275 μm cutoff, diagnostic sensitivity and specificity of central foveal thickness for acute Behçet uveitis were 89% and 72%, respectively (area under the curve = 0.902; 95% CI = 0.826-0.978, P < 0.001). There was choroidal stromal expansion which was not associated with thickening of the choroid. Central foveal thickness may be used as a noninvasive measure to assess inflammatory activity in early Behçet uveitis.

Evaluation of efficacy of infliximab for retinal vasculitis and extraocular symptoms in Behçet disease.

We evaluated ocular symptoms and activity of retinal vasculitis of Behçet disease before and after infliximab therapy, using Behçet disease ocular attack score 24 (BOS24) and fluorescein angiography (FA) score. We also analyzed the efficacy of infliximab for ocular and extraocular symptoms. Retrospective study. Using medical records, we analyzed FA and BOS24 to evaluate the association between the efficacy of infliximab therapy and FA as well as BOS24 scores. Further, we evaluated the association between FA scores and extraocular symptoms. After 2years of infliximab therapy, 6-month BOS24 was significantly reduced compared to that before treatment (PreBOS24-6M). After 4years of infliximab therapy, 6-month BOS24 was also significantly reduced compared to preBOS24-6M. After 2years of infliximab therapy, FA score median (interquartile range) decreased significantly compared to that before treatment [FA-2Y vs. preFA: 0 (0-0) vs.15.5 (12-24); P < 0.0001; n = 38]. The FA-4Y score in subjects followed for at least 4years was also significantly lower than the preFA score [0 (0-0) vs.16.5 (12-24.5); P < 0.0001; n = 28]. Among 38 patients, extraocular symptoms were resolved following treatment in 29 cases (76.3%). No significant correlation was observed between the improvement in FA-2Y and FA-4Y scores and the occurrence and persistence of extraocular symptoms (P = 0.33; n = 38 or P = 0.4; n = 28). Infliximab therapy is effective for the treatment of ocular and extraocular involvement of Behçet disease. BOS24 and FA scores are useful for evaluating the efficacy of infliximab therapy.

Tocilizumab for the Treatment of Behçet Uveitis that Failed Interferon Alpha and Anti-Tumor Necrosis Factor-Alpha Therapy

ABSTRACTPurpose: To report the results of tocilizumab (TCZ) treatment in patients with Behçet uveitis (BU) who had failed conventional, interferon alpha, and anti-Tumor necrosis factor-alpha therapy.Methods: We reviewed the records of five patients with BU treated with monthly infusions of TCZ 8 mg/kg. Outcome measures were visual acuity, anterior chamber cells, laser flare meter (LFM) readings, vitreous haze, central macular thickness (CMT), and fluorescein angiography (FA) score.Results: The clinical inactivity of BU and 20/50 or better vision were achieved in three female and two male patients treated with TCZ for 5–19 months. The mean LFM reading was reduced from 15.4 ± 2.7 to 5.0 ± 0.9 ph/ms; the mean CMT from 324.7 ± 36.6 µm to 280.2 ± 34.1 µm; and the mean FA score from 20.6 ± 5.4 to 9.3 ± 4.5 µm at the last visit. The only side effect was a slight elevation of the total cholesterol level in one patient.Conclusions: Tocilizumab may be a safe and effective therapeutic option for refractory BU.

Short-term results of endovascular surgery with tissue plasminogen activator injection for central retinal vein occlusion.

To examine the effects of retinal endovascular surgery (REVS) with tissue plasminogen activator injection into the retinal vein in central retinal vein occlusion (CRVO) eyes. Sixteen consecutive CRVO patients with macular edema and decreased visual acuity who were referred to Toyama University Hospital between March 2014 and February 2016 were included in this study. Changes in visual acuity (VA) and central retinal thickness (CRT) were evaluated up to 6months after REVS. Staining and leakage of the retinal veins in fluorescein angiography (FA) was graded in nine patients. Ten of 16 eyes were determined to be non-ischemic while the remaining six were ischemic. The mean logarithm of the minimum angle of resolution (logMAR) of VA was significantly improved from 0.98±0.58 (mean±standard deviation) at baseline to 0.78±0.61 at 3months (p=0.002), and 0.64±0.60 at 6months (p=0.003) after REVS. At 6 months, VA was improved in eight eyes (50%), while the other eight (50%) showed no change; none showed worsening. In the 10 eyes with non-ischemic CRVO, the mean VA was significantly improved at 6months (p=0.002), whereas no improvement was found in the six eyes with ischemic CRVO, . In all eyes, the mean CRT was significantly improved from 804±343μm at baseline to 506±304μm at 2months (p=0.014), 332±229μm at 3months (p=0.0001), and 305±235μm at 6months (p=0.00001). The postoperative complications observed were prolonged vitreous hemorrhage in one eye and neovascular glaucoma in two eyes. For postoperative recurrence of macular edema, sub-tenon injection of triamcinolone acetonide was given to five eyes, and intravitreal injection of an anti-VEGF agent was given to five eyes. Pan-retinal photocoagulation was performed on six eyes with ischemic type CRVO. The FA score was significantly improved after REVS (p=0.018). REVS using a specially made micro-needle may be a surgical treatment option for non-ischemic CRVO, but it did not seem effective for ischemic CRVO.