A Comparison of Adult and Juvenile Behcet Patients and a Look at Clinical Trends: Retrospective Data from a Turkish Follow-Up Cohort Study.

BACKGROUND: Previous reports indicate that juvenile Behçet's disease (BD) may have a different course than adult BD. However, as a direct comparison with adult Behçet patients has only been made in a limited number of studies, the issue is still controversial. OBJECTIVES: The primary aim of our study was to compare clinical manifestations in a large cohort of juvenile and adult Behçet patients registered in a single centre. The secondary aim of our study was to compare the data of newly diagnosed patients registered between 1998 and 2020 with the data of those registered between 1976 and 1997. METHODS: Data were collected retrospectively from medical records of patients registered between 1998 and 2020. Juvenile BD was defined as fulfilment of International Criteria for Behçet's Disease at or before 16 years of age. RESULTS: A similar course of disease was noted in juvenile and adult Behçet patients with no significant difference in the frequency of mucocutaneous findings, major organ involvement, and positivity of the pathergy test. A comparison of the periods, 1976-1997 and 1998-2020, revealed no significant difference in the prevalence of mucocutaneous lesions and major organ involvement. CONCLUSIONS: Our results indicate that juvenile and adult Behçet patients have a similar course with a similar frequency of clinical manifestations. Contrary to reports suggesting an overall tendency to milder disease over time, no decrease in the risk of major organ involvements was observed. A significant trend towards a decline in pathergy test positivity was noted.

Ocular inflammatory diseases in children with familial Mediterranean fever: a true association or a coincidence?

PURPOSE: To describe the characteristics of patients with familial Mediterranean fever (FMF) with concurrent ocular inflammatory disease (OID) and to analyze possible relations between them. METHODS: Clinical data were extracted from electronic medical records. Additionally, the medical literature on OIDs reported in patients with FMF was reviewed. RESULTS: Among 512 pediatric patients with FMF, five cases were found to have OIDs: bilateral anterior chronic uveitis, bilateral panuveitis, recurrent optic neuritis (RON), recurrent orbital myositis (ROM), and acquired Brown's syndrome. The first cases of ROM and acquired Brown's syndrome in FMF have been described in the literature. All cases presented with early-onset typical FMF attacks, carried at least one M694V mutation, and experienced OID while on colchicine. CONCLUSION: Increased frequency of OIDs in FMF as per the pediatric population and relapsing and chronic course of OIDs occasionally with concurrent FMF attacks suggest that this inflammatory syndrome, especially those carrying M694V mutations, may be a predisposing factor for OIDs.

Comparison of the efficacy of nepafenac 0.1% in quiescent Behçet's uveitis and non-uveitic healthy patients after phacoemulsification surgery.

PURPOSE: To compare the efficacy of nepafenac on anterior chamber flare and intraocular pressure(IOP) in healthy (group 1) and uveitic eyes (group 2) undergoing cataract surgery. METHODS: A retrospective, consecutive case series study. RESULTS: Among 54 the patients, 14 had a history of uveitis. The groups were similar in age and gender. There were significant changes in flare values in both groups. When the temporal changes of flare values were compared, there was no difference between the two groups. There were no significant changes in IOP values in both group. When the temporal changes of IOP values were compared, there was no difference between the groups. CONCLUSIONS: Nepafenac 0.1% has been shown to be effective in suppressing inflammation after cataract surgery in uveitic eyes as well as in healthy eyes. In addition, it has been observed that it does not increase intraocular pressure in both healthy and uveitic eyes and it is safe to use with this regard.

Ocular Inflammatory Events Following COVID-19 Vaccination in the Paediatric Population: A Multinational Case Series.

BACKGROUND: Ocular inflammatory events following COVID-19 vaccination have been reported in the adult population. METHODS: Multinational case series of patients under the age of 18 diagnosed with ocular inflammatory events within 28 days of COVID-19 vaccination. RESULTS: Twenty individuals were included. The most common event was anterior uveitis (n = 8, 40.0%), followed by intermediate uveitis (7 patients, 35%), panuveitis (4 patients, 20%), and posterior uveitis (1 patient, 5%). The event was noticed in the first week after vaccination in 11 patients (55.0%). Twelve patients (60.0%) had a previous history of intraocular inflammatory event. Patients were managed with topical corticosteroids (n = 19, 95.0%), oral corticosteroids (n = 10, 50.0%), or increased dose of immunosuppressive treatment (n = 6, 30.0%). Thirteen patients (65.0%) had a complete resolution of the ocular event without complications. All patients had a final visual acuity unaffected or less than three lines of loss. CONCLUSION: Ocular inflammatory events may happen in the paediatric population following COVID-19 vaccination. Most events were successfully treated, and all showed a good visual outcome.

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

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.

Atypical Chronic Central Serous Chorioretinopathy Mimicking Vogt-Koyanagi-Harada Disease: Full Therapeutic Response to Half-Fluence Photodynamic Therapy

The aim of this case report is to describe a case of atypical central serous chorioretinopathy (CSCR) definitively diagnosed after 8 years. A 44-year-old woman presented with reduced visual acuity in her left eye. Her visual acuity was light perception with projection in the right eye and 0.15 in the left. She described a similar decline in vision in her right eye 8 years ago. At that time, she had exudative retinal detachment and was treated with systemic immunosuppressive therapy for a presumed diagnosis of Vogt-Koyanagi-Harada disease. Despite resolution of the exudative retinal detachment, macular scarring developed. Eight years later, she developed inferior exudative retinal detachment in the left eye. A diagnosis of atypical CSCR was made with the help of multimodal imaging and her left eye was successfully treated with eplerenone and half-fluence photodynamic therapy (hf-PDT). In conclusion, early diagnosis and treatment of atypical CSCR may prevent subretinal fibrosis formation and permanent vision loss. Hf-PDT and eplerenone are successful treatment options for atypical CSCR.

Chickenpox Associated Retinitis and Occlusive Vasculitis in a Vaccinated Child: A Case Report and Literature Review.

Either retinitis and occlusive vasculitis are rare but vision threatening ocular complications of chickenpox in children. In this case report a 13-year-old girl who developed chickenpox 2 days before complaining with visual loss in her right eye is presented. She was vaccinated one dose of varicella zoster virus (VZV) vaccine when she was 12 months old. Best corrected visual acuity was counting fingers at 1.5 m in right eye. A subtle anterior segment inflammation and mild vitritis were observed. Fundoscopic examination of right eye showed ischemia in paracentral macula and white foci of retinitis along the superotemporal branch of retinal vessels. She was hospitalized and intravenous acyclovir treatment at 3 × 10 mg/kg daily dose was started. Serum IgM and IgG for VZV were positive. Aqueous humor PCR test was also reported positive for VZV DNA. Oral methylprednisolone was added at a dose of 64 mg/day at the 3rd day acyclovir treatment. Macular edema developed at 4th week of treatment and bevacizumab was administered intravitreally. After 3 injections retinal edema subsided completely. At 6-month follow-up retinal ischemia in superotemporal periphery was observed and photocoagulation was added to treatment.

The Use of Optical Coherence Tomography Angiography in the Diagnosis of Inflammatory Type 1 Choroidal Neovascularization Secondary to Tuberculosis: A Case Report.

Purpose: To report a case of choroidal neovascularization (CNV), which was not obvious with dye angiography but was detected with optical coherence tomography angiography (OCTA).Methods: A 50-year-old female presented with decreased vision in her right eye. Funduscopic examination revealed a swollen hyperemic disc, peripapillary exudation, and choroidal infiltration.Results: Optical coherence tomography revealed intraretinal cysts, subretinal fluid in the macular region, and subretinal hyperreflective material in the papillomacular area. Fluorescein angiography revealed early hypofluorescence in the areas of choroidal infiltrations, and indocyanine green angiography (ICGA) also showed hypocyanescence corresponding to these infiltrations and revealed a faint hypocyanescence in the papillomacular region, further corresponding to the subretinal hyperreflective material on OCT scan. Two weeks after the initiation of systemic therapy with the diagnosis of tuberculosis, OCTA scans detected Type 1 CNV in the peripapillary area.Conclusion: OCTA may be superior to ICGA in the detection of CNV secondary to inflammatory situations.

Comparison of Spectral Domain Optical Coherence Tomography (SD OCT) Findings with Laser Flare Photometry (LFP) Measurements in Behçet's Uveitis.

Purpose: This study aims to compare the spectral domain optical coherence tomography (SD OCT) findings and laser flare photometry (LFP) measurements in Behçet's disease (BD).Methods: This study was composed of 4 groups as follows: "Group 1: Healthy volunteers, Group 2: Volunteers with BD and no eye involvement, Group 3: Patients with active uveitis with BD, Group 4: Patients with BD in quiescent phase of uveitis". Laser Flare Photometry (Kowa FM-600, Kowa Acculas, San Jose, CA, USA), SD-OCT and Enhanced Depth Imaging protocol (EDI) (Spectralis®, Heidelberg Engineering Inc., Heidelberg, Germany) and Fundus Florescein Angiography (FFA) (Heidelberg Retina Angiograph 2®; Heidelberg Engineering, Heidelberg, Germany) were performed for all patients.Results: Twenty-four eyes of 12 healthy volunteers (Group 1); 61 eyes without ocular involvement of 31 volunteers with BD (Group 2); 29 active uveitic eyes of 17 patients with BD (Group 3); 35 eyes with quiescent uveitis in 18 patients with BD (Group 4) participated in the evaluation. There was a significant difference between visual acuity, submacular choroidal thickness (SCT), and flare values comparison among the groups(p<0.001,p<0.002, and p<0.001). When compared considering the SCT, choroidal thickness in Group 3 was higher than in the eyes of Group 1 and Group 2 (p:0.005, p:0.006). Central macular thickness (CMT) and disease duration were negatively correlated (rho:-0.453, p<0.001), while CMT and flare values were positively correlated (rho:0.267,p:0.006).Conclusion: Flare is a reliable indicator of the inflammation in Behçet's uveitis. Its concurrent use with OCT to evaluate choroid and macula enables the assessment of inflammation.

Comparison of the Treatment Results for Behçet Uveitis in Patients Treated with Infliximab and Interferon.

Purpose: To compare the efficacy and safety of infliximab and interferon in patients with refractory Behçet uveitis.Methods: Medical records of 20 patients treated with infliximab (IFX) and 33 patients treated with interferon (IFN) were retrospectively analyzed by evaluating the number of uveitis attacks, visual acuity and ocular inflammation at 6 and 12 months after initiation of treatment.Results: Mean duration of treatment was 22 ± 18 months in IFX group and 30.3 ± 22.5 months in IFN group. Sixteen patients of IFX group (80%) and 28 patients of IFN group (85%) achieved remission. In both groups, the mean best-corrected visual acuity and all inflammatory parameters improved. In terms of these parameters, there was no significant difference between the two groups.Conclusion: Infliximab and interferon improve control of ocular inflammation and efficiently decrease the relapse rate. Our results determined no significant difference between two agents in controlling intraocular inflammation.

Cytomegalovirus Endotheliitis After Penetrating Keratoplasty.

Cytomegalovirus (CMV)-related corneal endotheliitis is an inflammation of the corneal endothelium caused by CMV. It may occur de novo or after ocular surgery in otherwise healthy individuals. In patients who have undergone keratoplasty, the differential diagnosis of viral endotheliitis and immune-related graft rejection is challenging due to the similar clinical findings. Here we report a patient who underwent penetrating keratoplasty and was using local and systemic immunosuppressive agents due to previous history of graft rejection. At postoperative year 4, ophthalmologic examination revealed localized corneal edema, coin-shaped keratic precipitates, and increased intraocular pressure, consistent with viral endotheliitis. Polymerase chain reaction revealed CMV-DNA amplification in the aqueous humor sample. Valganciclovir treatment was started and the symptoms improved in 2 months. It should be kept in mind that local or systemic immunosuppressants used after keratoplasty may trigger CMV reactivation. Anti-CMV treatment should be initiated immediately in patients with coin-shaped keratic precipitates.

Culture-Proven Candida Albicans Endogenous Endophthalmitis in a Patient with Onychomycosis.

Purpose: To report a case of Candida albicans endogenous endophthalmitis in an immunocompetent patient with onychomycosis.Methods: Retrospective case report.Results: A 40-year-old man with onychomycosis presented with C. albicans subretinal abscess in the left eye. Systemic and intravitreal injections did not prevent further progression of the infection. The patient underwent pars plana vitrectomy. One month after surgery, the intraocular inflammation gradually subsided. However, his visual acuity stayed at counting fingers as a result of macular scarring.Conclusion: The aim of this case presentation is to emphasize that endogenous fungal endophthalmitis can be seen in an immunocompetent patient. The use of systemic steroids in the past was the main reason for the progression of the disease in this case. In these situations, when the clinical findings suggest a fungal etiology, it should keep in mind that endogenous candida endophthalmitis can be a result of fungal infections from distant sites such as the toenails and systemic steroids should not be started before definite diagnosis.

Topical cyclosporine-A in dry eye associated with chronic graft versus host disease.

We evaluated the efficacy and safety of topical cyclosporine-A in dry eye associated with chronic graft versus host disease (GVHD) in 8 patients. Visual acuity, fluorescein staining of cornea and conjunctiva, tear break-up time, Schirmer test results and subjective complaints were recorded. Topical cyclosporine-A might be an effective and safe treatment option in dry eye related to chronic GVHD.

Optic Nerve Avulsion and Retinal Detachment After Penetrating Ocular Trauma: Case Report.

Optic nerve avulsion is a rare pathology with poor prognosis usually seen after blunt trauma. The optic nerve is separated from the sclera by indirect forces due to the relatively weak structure of the lamina cribrosa area. Here we describe an 11-year-old boy who experienced optic nerve avulsion and retinal detachment after penetrating ocular trauma.

Exudative retinal detachment.

Exudative retinal detachment develops when fluid collects in the subretinal space. The subretinal space between the photoreceptors and the retinal pigment epithelium is the remnant of the embryonic optic vesicle. In the developed eye the subretinal space is of minimal size, but it can reopen under pathological conditions that disrupt the integrity of blood-retinal barrier. Inflammatory, infectious, infiltrative, neoplastic, vascular, and degenerative conditions may be associated with blood-retinal barrier breakdown and the sequential development of exudative retinal detachment. We elaborate on the pathogenesis and the differential diagnosis of exudative retinal detachment and specifically discuss the spectrum of diseases associated with exudative retinal detachment in uveitis clinics.

Validation of the EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis by disease content experts.

The European League Against Rheumatism recommendations for the management of antineutrophil cytoplasmic antibody-associated vasculitis have been recently published. Unique to recommendation development, they were also voted on by members of a learned society. This paper explores the wider validity of the recommendations among people who self-identify as clinicians caring for patients with vasculitis. In addition to the task force, a learned society (European Vasculitis Society-EUVAS) was invited, through online survey, to rate independently the strength of evidence of each recommendation to obtain an indication of the agreement among the final target audience and ultimate end-users of the recommendations. The survey took place in June 2015. Of the 158 EUVAS members surveyed, there were 88 responses (55.7%). There was a large degree of agreement in the voting patterns between EUVAS survey participants and task force members. Notable exceptions were lower grades for the recommendation of the use of rituximab for remission induction in patients with eosinophilic granulomatosis with polyangiitis and for methotrexate and mycophenolate mofetil as remission maintenance agents in patients with granulomatosis with polyangiitis/microscopic polyangiitis by EUVAS members. These results are encouraging and suggest that the voting patterns of the task force are representative of the wider vasculitis community. We recommend future recommendations adopt this approach for data/expert-based treatment guidelines, especially for multisystem diseases.

Bilateral optic neuritis in a patient with Behçet's disease who respond to therapeutic plasma exchange.

Bilateral optic neuritis has been reported very rarely as a manifestation of neuro-Behçet's disease. We present a 50 year old woman who had 20-year history of Behçet's disease presented with acutely blurred vision associated with orbital pain in both eyes. Visual acuity was 0.4 in the right eye and light perception in the left eye; afferent pupillary defect was detected in the left eye. Bilateral swelling of the optic disk was found. The cerebrospinal fluid sample tests were within normal limits. Brain magnetic resonance imaging, magnetic resonance venography and fundus fluorescein angiography were normal. She was diagnosed with bilateral optic neuritis and treated with intravenous methyl prednisolone for 10 days. As there was no response to the treatment, therapeutic plasma exchange was started and the patient's visual acuities improved moderately. We suggest that when high dose steroid is failed to treat ON in BD, treatment with TPE may be considered.

Causes of chemoreduction failure in retinoblastoma and analysis of associated factors leading to eventual treatment with external beam radiotherapy and enucleation.

PURPOSE: To evaluate the causes of chemoreduction failure in retinoblastoma and to analyze the associated factors for eventual treatment with external beam radiotherapy and enucleation. DESIGN: Prospective noncomparative case series. PARTICIPANTS: Seventy-one patients with 105 eyes with intraocular retinoblastoma that underwent chemoreduction therapy between October 1998 and January 2003. INTERVENTION: A 6-treatment cycle of chemoreduction therapy with vincristine, etoposide, and carboplatin was administered at monthly intervals. Unresponsive disease was defined as persistence of retinal tumors, vitreous seeds, or subretinal seeds after the second treatment cycle, with no appreciable sign of regression. Eyes with unresponsive disease were enucleated after the second treatment. Eyes that responded to chemoreduction therapy received focal treatment, including indirect laser photocoagulation, transpupillary thermotherapy, cryotherapy, and ruthenium 106 episcleral plaque radiotherapy after the second chemoreduction treatment, if necessary, to achieve complete tumor regression. Recurrence was defined as the regrowth of retinal tumors, vitreous or subretinal seeds after an initial favorable response, and regression. Recurrent retinal tumor, vitreous seeds, or subretinal seeds were treated with focal treatments and 2 to 3 additional chemoreduction treatments. When these methods failed or were not applicable, external beam radiotherapy and/or enucleation was administered. MAIN OUTCOME MEASURES: The use of external beam radiotherapy and enucleation for chemoreduction failure, which was defined as unresponsive or recurrent disease. RESULTS: The mean follow-up was 25.7 months (range: 6-49). Ten of 105 eyes (9.5%) with unresponsive disease were enucleated after the second treatment. Of the remaining 95 eyes, 42 (44.2%) developed recurrence after chemoreduction. Recurrent disease failing to be treated successfully by other methods was treated with external beam radiotherapy in 26 of 95 eyes (27.4%) and enucleation in 22 of 95 eyes (23.2%). External beam radiotherapy was successful in preventing enucleation in 20 of 26 eyes (76.9%). Overall, the globe salvage rate was 69.5%, ranging from 36.1% for Reese-Ellsworth group V disease to 87.0% for groups I to IV disease. Histopathologically, 29 of 31 enucleated eyes (93.5%) had poorly differentiated or moderately differentiated retinoblastoma. Using multivariate logistic regression analysis, factors predictive of eventual treatment with external beam radiotherapy were female gender (P = 0.010), presence of subretinal seeds (P = 0.023), and a greater number of chemoreduction treatments (P = 0.027). By multivariate analysis, the factors associated with the need for eventual treatment with enucleation were recurrence of retinal tumors (P = 0.004), presence of vitreous seeds (P = 0.008), greater tumor thickness (P = 0.015), presence of subretinal fluid (P = 0.040), and older patient age (P = 0.042). CONCLUSIONS: Chemoreduction failure in this article was defined as unresponsive or, more commonly, recurrent retinoblastoma. Older patient age, greater tumor thickness, presence of vitreous seeds and subretinal fluid at baseline, and retinal tumor recurrence after chemoreduction were factors associated with the need for enucleation.