Retinal thickness profiles in patients with Behçet uveitis during remission / Perfis de espessura da retina em pacientes com uveíte de Behçet durante remissão

ABSTRACT Purpose: The purpose of this study was to evaluate the intraretinal layer thickness in the macular region and its correlation with the duration of uveitis and visual acuity in patients with Behçet uveitis. Methods: In this cross-sectional study, we included 93 eyes of 57 patients with Behçet uveitis and 100 eyes of 50 healthy individuals admitted to a tertiary center from January to September 2017. We performed macular measurements in all subjects via spectral domain-optical coherence tomography (SD-OCT) and divided the retina into layers using automated segmentation software on the SD-OCT device. We then compared layer thicknesses between the patient and control groups and evaluated the correlation between OCT parameters and the duration of uveitis and visual acuity in the patient group. Results: Our records show a mean age of 37.9 ± 10.8 (18-64) years and 37.7 ± 12.2 (21-61) years in the patient and control groups (p=0.821), respectively. Meanwhile, data reveal a mean duration of uveitis of 6.9 ± 4.7 (1-20) years. We found a reduction in the total outer layer thickness in the patient group (p<0.001). However, we did not find a statistically significant difference in the inner retinal layers except in the inner nuclear layer. The duration of uveitis negatively correlated with the outer retinal layer's thickness (correlation coefficient = -0.250). On the other hand, visual acuity positively correlated with the central macular, the total inner layer, and the outer retinal layer thicknesses (correlation coefficients: 0.194, 0.154, and 0.364, respectively). However, the inner nuclear layer negatively correlated with visual acuity. Conclusions: Using retinal segmentation via SD-OCT for follow-ups can help estimate visual loss in patients with Behçet uveitis, which can cause significant changes in intraretinal layers in the macular region.

RESUMO Objetivo: Avaliar a espessura das camadas intraretinianas na região macular e sua relação com a duração da uveíte e acuidade visual em pacientes com uveíte de Behçet. Métodos: Este estudo transversal incluiu 93 olhos de 57 pacientes com uveíte de Behçet e 100 olhos de 50 indivíduos saudáveis que foram admitidos em um hospital terciário entre janeiro de 2017 e setembro de 2017. As medições maculares foram realizadas com tomografia de coerência óptica de domínio espectral (SD-OCT) em todos os pacientes. A retina foi dividida em camadas usando software de segmentação automatizado no dispositivo SD-OCT. As espessuras da camada foram comparadas entre os pacientes e os grupos controle. No grupo de pacientes, foi avaliada a correlação entre os parâmetros obtidos na OCT e a duração da uveíte e acuidade visual. Resultados: A média de idade foi de 37,9 ± 10,8 (18-64) no grupo de pacientes e 37,7 ± 12,2 (21-61) no grupo controle (p=0,821). A duração média da uveíte foi de 6,9 ± 4,7 (1-20) anos. A espessura total das camadas externas no grupo de pacientes foi reduzida (p<0,001). Uma diferença estatisticamente significativa não foi encontrada nas camadas internas da retina, exceto na camada nuclear interna. Uma correlação negativa foi detectada entre a duração da uveíte e a espessura da camada externa da retina (coeficiente de correlação = -0,250). Uma correlação positiva significativa foi detectada entre a acuidade visual e a espessura macular central bem como a espessura total das camadas internas e externas da retina (coeficientes de correlação 0,194; 0,154 e 0,364, respectivamente). A camada nuclear interna foi negativamente correlacionada com a acuidade visual. Conclusões: A uveíte de Behçet pode causar alterações significativas nas camadas intraretinianas na região macular. A segmentação da retina com SD-OCT pode ser útil para acompanhamentos e para estimar a perda visual em pacientes com uveíte de Behçet.

Sarcoid-like Uveitis with or without Tubulointerstitial Nephritis during COVID-19.

PURPOSE: To report sarcoid-like uveitis with or without tubulointerstitial nephritis (TIN) during coronavirus disease 2019 (COVID-19) and to discuss diagnostic evaluation and treatment. METHODS: Detailed information on the subject's demographics, medical history, ophthalmic examination findings, and laboratory results were obtained from medical records. Fluorescein angiography (FA) and indocyanine green angiography (ICGA) images were evaluated. RESULTS: All seven patients aged between 8 and 17 had bilateral granulomatous panuveitis. TIN preceded in four patients. Thorax computed tomography of patient 1 was found to be compatible with COVID-19, patients 2 and 3 were in contact with Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) positive parents, patients 4 and 5 were found to be SARS-CoV-2 PCR positive, and patients 6 and 7 were positive for SARS-CoV-2 IgG antibodies. ICGA revealed hypofluorescent dots in six patients. Intraocular inflammation was controlled with corticosteroid and/or immunomodulatory therapy. Visual acuity was maintained or improved in all. CONCLUSION: SARS-CoV-2 infection may be related to sarcoid-like uveitis and acute tubulointerstitial nephritis.

Retinal thickness profiles in patients with Behçet uveitis during remission.

PURPOSE: The purpose of this study was to evaluate the intraretinal layer thickness in the macular region and its correlation with the duration of uveitis and visual acuity in patients with Behçet uveitis. METHODS: In this cross-sectional study, we included 93 eyes of 57 patients with Behçet uveitis and 100 eyes of 50 healthy individuals admitted to a tertiary center from January to September 2017. We performed macular measurements in all subjects via spectral domain-optical coherence tomography (SD-OCT) and divided the retina into layers using automated segmentation software on the SD-OCT device. We then compared layer thicknesses between the patient and control groups and evaluated the correlation between OCT parameters and the duration of uveitis and visual acuity in the patient group. RESULTS: Our records show a mean age of 37.9 ± 10.8 (18-64) years and 37.7 ± 12.2 (21-61) years in the patient and control groups (p=0.821), respectively. Meanwhile, data reveal a mean duration of uveitis of 6.9 ± 4.7 (1-20) years. We found a reduction in the total outer layer thickness in the patient group (p<0.001). However, we did not find a statistically significant difference in the inner retinal layers except in the inner nuclear layer. The duration of uveitis negatively correlated with the outer retinal layer's thickness (correlation coefficient = -0.250). On the other hand, visual acuity positively correlated with the central macular, the total inner layer, and the outer retinal layer thicknesses (correlation coefficients: 0.194, 0.154, and 0.364, respectively). However, the inner nuclear layer negatively correlated with visual acuity. CONCLUSIONS: Using retinal segmentation via SD-OCT for follow-ups can help estimate visual loss in patients with Behçet uveitis, which can cause significant changes in intraretinal layers in the macular region.

Familial Mediterranean fever associated frosted branch angiitis, retinal vasculitis and vascular occlusion.

OBJECTIVES: To analyse the entity of retinal vasculitis, including frosted branch angiitis (FBA), or retina vascular occlusion in patients with familial Mediterranean fever (FMF). METHODS: Retrospective collaborative case series using invitation by email to uveitis specialists around the Mediterranean basin. This series was combined with a literature review. Exclusion criteria included infectious diseases, Behçet's disease or other autoimmune diseases. RESULTS: A total of 16 patients (21 eyes) had FMF and retinal vasculitis (FBA 11 patients, mild retinal vasculitis 5 patients). The mean age at onset of vasculitis was 29.5 ± 13.4 (range 9-62) with a female to male ratio of 9 to 7. In 19 eyes treated with various forms of corticosteroid and/or immunosuppression, the mean initial spectacle-corrected visual acuity improved from 6/194 to 6/10.5 at the last mean follow-up of 29.0 ± 34.9 months (p < 0.001). The most common FEVR mutations were M680I and M694V. In addition, retinal vascular occlusions included one case of central retinal artery occlusion and one case of branch retinal artery occlusion. CONCLUSION: FBA and milder forms of retinal vasculitis are associated with FMF. Therapy involves an increase in colchicine dosage in early cases, a long period of oral corticosteroid, intravitreal dexamethasone implant or periocular corticosteroid in select cases, and combination therapy with systemic immunosuppression in severe cases. FMF needs to be included in the differential diagnosis of retinal vasculitis.

The Effect of Vision-Related Quality of Life on Depression and Anxiety in Patients with Behçet Uveitis.

OBJECTIVES: To evaluate the effect of vision-related quality of life on depression and anxiety in patients with Behçet uveitis. MATERIALS AND METHODS: The Beck Depression Inventory (BDI), the State-Trait Anxiety Inventory (STAI) I-II, and the Visual Functioning Questionnaire (VFQ)-25 were used to evaluate 105 patients being followed for Behçet uveitis. Sociodemographic data and VFQ-25 scores were compared between the groups with and without depression and anxiety. Regression analysis was performed to determine the relationship between the variables. RESULTS: Forty-eight (82.8%) men and 10 (17.2%) women who completed the questionnaires were included in the study. The mean age of the patients was 37.76±11.14 (18-65) years and the mean duration of uveitis was 8.57±7.43 (1-27) years. The mean VFQ-25 composite, BDI, STAI-I, and STAI-II scores were 74.90±18.50 (18.79-97.04), 10.76±8.90 (0-43), 42.52±6.23 (25-55), and 46.53±6.80 (27-58), respectively. Of 58 patients, 31% had depressive symptoms and 58.6% had anxiety symptoms. VFQ-25 composite score was lower in the depressive group than in the group with no depression (p=0.030), while there was no significant difference in this score between the groups with and without anxiety. Regression analysis revealed a negative relationship between total VFQ-25 composite score and depression. CONCLUSION: In our study, high rates of depression and anxiety were detected in patients with Behçet uveitis. Patient-reported visual functioning was associated with depression. In patients with Behçet uveitis, it is important to evaluate vision-related quality of life as well as visual acuity.

Quantitative measurements with optical coherence tomography angiography in Behçet uveitis.

PURPOSE: The aim of this study was to evaluate the quantitative measurements obtained by optical coherence tomography angiography in patients with Behçet uveitis. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Pediatric Uveitis in a Referral Center in North Part of Turkey.

Purpose: To analyze the demographic characteristics, clinical features, ocular complications, and visual outcome of pediatric patients with uveitis.Methods: Retrospective evaluation of medical records.Results: The study included 156 eyes of 93 patients. Fifty-three patients were female and 40 were male. Mean age at onset of the uveitis was 9.54 ± 4.29 years. The mean follow-up period was 29.88 ± 28.97 months. Anterior uveitis (49.5%) was the most common anatomic type followed by panuveitis (21.5%), intermediate uveitis (18.3%), and posterior uveitis (10.7%). Juvenile idiopathic arthritis (JIA) was the most common leading systemic disease (18.3%) followed by Behçet disease (11.8%). It was detected at least one complication in 53 (34.0%) eyes at presentation. Mean LogMAR visual acuity was found <0.3 in 136 (87.2%) eyes at final examination.Conclusion: The most common localization was the anterior segment and the most common etiologic relationship was JIA. Visual outcome could be satisfactory with early and appropriate treatment.

Changes in corneal thickness, upper and lower tear film in seasonal allergic conjunctivitis by steroid treatment: anterior segment optical coherence tomography study.

PURPOSE: To evaluate the effects of loteprednol etabonate on cornea and tear film in patients with seasonal allergic conjunctivitis by anterior segment optical coherence tomography (OCT). METHODS: The right eyes of 52 subjects with seasonal allergic conjunctivitis were evaluated in the study. Central corneal thickness (CCT), the thicknesses of central corneal epithelium (CET) and central non-epithelial cornea (CnET), and lower and upper tear meniscus areas were measured by spectral-domain OCT and ocular surface disease index (OSDI) were obtained at baseline. After topical use of loteprednol etabonate 0.5% eye drop three times a day for 1 week, the measurements were repeated. Paired T test was used for analysis. RESULTS: Mean CCT and mean thicknesses of CET and CnET at baseline were 528.19 ± 34.47 µm, 64.10 ± 5.30 µm and 462.84 ± 33.43 µm, respectively, and these were 526.31 ± 33.37 µm, 64.65 ± 4.84 µm and 459.84 ± 32.11 µm after the treatment, respectively. OSDI score was 21.55 ± 4.40 at baseline and 13.13 ± 2.39 after 1 week. The lower and upper tear meniscus areas at baseline were 0.026 ± 0.017 mm2 and 0.017 ± 0.007 mm2, respectively, and they were 0.017 ± 0.009 mm2 and 0.016 ± 0.007 mm2, respectively, after the treatment. There is statistically significant difference between before and after values of CCT, CnET, lower tear meniscus area and OSDI score (p < 0.05). CONCLUSION: There is no change in corneal epithelial thickness and upper tear meniscus area after 1-week treatment with loteprednol etabonate in patients with seasonal allergic conjunctivitis, but it may cause decrease in non-epithelial corneal thickness and lower tear meniscus area.

Sarcoid-like Granulomatous Intraocular Inflammation Caused by Vemurafenib Treatment for Metastatic Melanoma

Vemurafenib is a potent inhibitor of genetically activated BRAF, which is responsible for tumoral proliferation in cutaneous melanoma. A 56-year-old man receiving vemurafenib therapy presented with uveitis. Over the course of the disease, he developed bilateral, granulomatous uveitis with multiple peripheral chorioretinal lesions. Serum angiotensin-converting enzyme levels increased. The patient was diagnosed with probable ocular sarcoidosis related to vemurafenib and was treated with an intravitreal dexamethasone implant. This case is the first report that shows the clinical and angiographic features of a patient with vemurafenib-related sarcoid-like granulomatous uveitis.

The Results of Interferon-Alpha Treatment in Behçet Uveitis.

Purpose: 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.

Comparison of central corneal thickness measurements using different imaging devices and ultrasound pachymetry.

PURPOSE: To compare central corneal thickness (CCT) measurements obtained by the AL-Scan, Lenstar LS900, Galilei, and ultrasound pachymetry (UP) in normal and cataractous eyes. METHODS: Eighty eyes of healthy subjects were included in the study. Each subject was assessed by four different methods of measurements using the AL-Scan, Lenstar LS900, Galilei, and UP by a single examiner. To assess the intraobserver repeatability, three consecutive measurements were taken for the AL-Scan. RESULTS: The mean CCT [± standard deviation (SD)] for the AL-Scan, Lenstar LS900, Galilei, and UP were 554.6 ± 30.9 µm, 542.9 ± 31.3 µm, 570.7 ± 30 µm, and 552.7 ± 32.8 µm, respectively. The differences between pairs of mean CCT for the methods are statistically significant for the pairs of Galilei-UP, AL-Scan-Galilei, and Lenstar LS900-Galilei. Bland-Altman plots showed that AL-Scan-UP have the closest agreement, followed by Lenstar-UP and AL-Scan-Lenstar. Galilei was found to have the poorest agreement with the other three methods. The intraobserver repeatability of the AL-Scan was very good with an intraclass correlation coefficient (ICC) of 0.980. CONCLUSION: We found that CCT measurements between the AL-Scan-UP, Lenstar LS900-UP, and AL-Scan-Lenstar LS900 showed very strong correlation and comparable agreement. AL-Scan-UP showed the closest agreement and these devices can be used interchangeably in clinical practice. Galilei significantly showed higher value of CCT compared to other methods. It was also observed that the Al-Scan had excellent intraobserver repeatability.

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

PURPOSE: 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.

Vitreomacular Interface Disorders in Behçet's Uveitis.

OBJECTIVES: To determine the prevalence of vitreomacular interface (VMI) disorders in patients with Behçet's uveitis and assess the relationship between VMI disorders and clinical characteristics. MATERIALS AND METHODS: The macular optical coherence tomography (OCT) images of 160 eyes of 96 patients with Behçet's uveitis who were being followed in the Uvea-Behçet's clinic were assessed retrospectively for VMI disorders including posterior vitreous detachment (PVD), epiretinal membrane (ERM), vitreomacular adhesion (VMA), vitreomacular traction (VMT), full-thickness macular hole (FTMH), lamellar hole (LH) and pseudohole. RESULTS: Twenty-four patients (25%) with unilateral and 72 patients with bilateral uveitis were included in the study. Six (30%) of 20 eyes with anterior uveitis and 57 (40.7%) of 140 eyes with posterior uveitis, in total 63 (39.4%) eyes of 160 eyes had at least one VMI disorder. PVD was detected in 4 eyes (2.5%), ERM in 48 eyes (30%), VMA in 12 eyes (7.5%), and LH in 1 eye (0.6%). None of the eyes had VMT, FTMH, or pseudohole. ERM was detected in 13 eyes (8.1%) on fundus examination and 48 (30%) eyes by OCT (p=0.001). VMI was detected in 12 (50%) of 24 eyes with prior intraocular surgery and 51 (37.5%) of 136 eyes without. The mean duration of uveitis was 7.3±5.8 years in patients with VMI disorders and 5.8±7.7 years in patients without (p=0.04). There was no relation between VMI disorders and anatomic location of uveitis, history of past ocular surgery, number of ocular/periocular steroid injections, or visual acuity. CONCLUSION: VMI disorders are common in patients with Behçet's uveitis. Their frequency increases with the duration of uveitis. OCT is more sensitive than fundus examination in the detection of VMI disorders.