Clinical features, diagnosis, treatment, and course of ocular sarcoidosis with or without uveitis: A retrospective, comparative study.

OBJECTIVE: To evaluate and compare characteristics, diagnosis, treatment, visual prognosis, and course between ocular sarcoidosis with or without uveitis in a population in Southern France. METHODS: We retrospectively analyzed data from patients with ocular sarcoidosis in a tertiary eye care center in Nice from January 2003 to December 2021. The inclusion criterion was biopsy-proven ocular sarcoidosis according to IWOS criteria as the first clinical manifestation of sarcoidosis. RESULTS: A total of 25 patients were included. Twenty patients had uveitis (70% panuveitis, 20% intermediate uveitis, and 10% anterior uveitis) and five patients had non-uveitic ocular sarcoidosis (one patient with dacryoadenitis, one patient with orbital granuloma, two patients with palpebral granuloma, and one patient with episcleritis). Only the cases with uveitis had bilateral involvement (85% of cases). There was no significant difference in ethnicity, biopsy diagnosis, systemic manifestations, or treatment between the two groups. Final visual outcomes remained good for both groups, with 96% of patients with BCVA>20/50, with no significant difference. Patients with non-uveitic sarcoidosis experienced less recurrence on treatment (P=0.042) and more remission (P=0.038) than patients with uveitis. Eighty percent of patients with uveitis had at least three suggestive clinical intraocular signs meeting IWOS criteria. CONCLUSION: In this population in Southern France, uveitis was the most common presentation of ocular sarcoidosis. The type of ocular sarcoidosis does not appear to be correlated with the type of systemic manifestations, use of systemic therapy, or visual prognosis, but patients with non-uveitic ocular sarcoidosis appear to have a better course with fewer recurrences on treatment and more remission than patients with uveitic ocular sarcoidosis.

Idiopathic uveitis presents different characteristics and outcome from juvenile idiopathic arthritis- related uveitis / Uveíte idiopática apresenta características e desfecho diferentes da uveíte associada à Artrite Idiopática Juvenil

ABSTRACT Objective: To describe the clinical features and outcomes of patients with uveitis associated with juvenile idiopathic arthritis (JIA) and idiopathic uveitis. Methods: This was an observational, retrospective study, conducted in a tertiary center. Patients under 18 years old who experienced at least one episode of uveitis and followed between 2000 and 2019 were included. Results: A total of 82 patients were included, of whom 43 had idiopathic uveitis and 39 had uveitis associated with JIA. Anterior uveitis was the primary site of ocular inflammation (76.8%) and occurred in 24 and 39 patients with idiopathic uveitis and uveitis associated with JIA arthritis, respectively (p=0.02). The complete response to corticotherapy was more frequent in uveitis associated with JIA (p=0.001). Total and partial responses to biological disease modifying antirheumatic drugs were more frequent in uveitis associated with JIA (p=0.025) and idiopathic uveitis (p=0.045), respectively. There were 203 complications: cataracts were more frequently present in idiopathic uveitis (p=0.05), while synechiae was more frequent in uveitis associated with JIA (p=0.02). Conclusion: Idiopathic uveitis and uveitis associated JIA frequently follow a chronic course and an increased risk of visual loss in childhood. The uveitis associated with JIA showed better response to systemic corticotherapy and total response to biologic disease modifying antirheumatic drugs more frequently.

RESUMO Objetivos: Descrever as características clínicas e desfechos dos pacientes com uveíte associada à Artrite Idiopática Juvenil (AIJ) e da Uveíte Idiopática. Métodos: Este foi um estudo retrospectivo observacional conduzido em um centro terciário. Foram incluídos pacientes abaixo dos 18 anos de idade que apresentaram pelo menos um episódio de uveíte e que estiveram em acompanhamento médico entre os anos de 2000 e 2019. Resultados: Foram incluídos 82 pacientes, sendo 43 com uveíte idiopática e 39 com uveíte associada à AIJ. A uveíte anterior foi o sítio primário de acometimento (76,8%) em 24 e 39 pacientes com uveíte idiopática e uveíte associada à AIJ, respectivamente (p=0.02). Resposta total à corticoterapia foi mais frequente na uveíte associada à AIJ (p=0.001). Respostas total e parcial às drogas antirreumáticas modificadoras de doença biológicas foram mais frequentes na uveíte associada à AIJ (p=0.025) e na uveíte idiopática (p=0.045), respectivamente. Foram encontradas 203 complicações: a catarata foi mais frequente na uveíte idiopática (p=0.05), enquanto a sinéquia foi mais frequente na uveíte associada à AIJ (p=0.02). Conclusão: A uveíte idiopática e a uveíte associada à AIJ frequentemente apresentam um curso crônico e um risco elevado de perda visual na infância. A uveíte associada à AIJ apresentou melhor resposta à corticoterapia sistêmica e resposta total às drogas modificadoras de doença reumática biológicas mais frequentemente.

Simultaneous cytomegalovirus uveitis and HIV retinopathy / Uveíte por citomegalovírus e retinopatia por HIV simultâneas

ABSTRACT A 41 year-old man presented with unilateral vision loss for a week and constitutional symptoms for 3 months. Ophthalmic evaluation revealed cotton wool spots on the right eye and widespread retinal necrosis and hemorrhage on the left eye, suggestive of cytomegalovirus uveitis and HIV infection and retinopathy, and confirmed by serology. The patient was treated with ganciclovir and highly active antiretroviral therapy and preserved contralateral vision. Clinicians should be aware of uveitis and retinopathies to prevent irreversible vision loss and systemic conditions.

RESUMO Paciente do sexo masculino, 41 anos, com queixa de perda de acuidade visual unilateral por 1 semana e sintomas constitucionais por 3 meses. Ao exame oftalmológico, apresentava exsudatos algodonosos, em olho direito, e áreas de necrose e hemorragias retinianas, em olho esquerdo, com suspeita de uveíte por citomegalovírus e retinopatia por HIV, confirmadas por sorologias. O paciente foi tratado com ganciclovir e terapia antirretroviral e preservou a visão contralateral. Os oftalmologistas devem estar atentos para casos de uveítes e retinopatias, para prevenirem perda visual irreversível e condições sistêmicas.

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.

Behçet's disease uveitis.

Uveitis in Behçet's disease (BD) is frequent (40% of cases) and is a major cause of morbidity. The age of onset of uveitis is between 20 and 30 years. Ocular involvement includes anterior, posterior or panuveitis. It is non-granulomatous. Uveitis may be the first sign of the disease in 20% of cases or it may appear 2 or 3 years after the first symptoms. Panuveitis is the most common presentation and is more commonly found in men. Bilateralisation usually occurs on average 2 years after the first symptoms. The estimated risk of blindness at 5 years is 10-15%. BD uveitis has several ophthalmological features that distinguish it from other uveitis. The main goals in the management of patients are the rapid resolution of intraocular inflammation, prevention of recurrent attacks, achievement of complete remission, and preservation of vision. Biologic therapies have changed the management of intraocular inflammation. The aim of this review is to provide an update previous article by our team on pathogenesis, diagnostic approaches, identification of factors associated with relapse and the therapeutic strategy of BD uveitis.

French recommendations for the management of non-infectious chronic uveitis.

This French National Diagnostic and Care Protocol (NDPC) includes both pediatric and adult patients with non-infectious chronic uveitis (NICU) or non-infectious recurrent uveitis (NIRU). NICU is defined as uveitis that persists for at least 3 months or with frequent relapses occurring less than 3 months after cessation of treatment. NIRU is repeated episodes of uveitis separated by periods of inactivity of at least 3 months in the absence of treatment. Some of these NICU and NIRU are isolated. Others are associated with diseases that may affect various organs, such as uveitis associated with certain types of juvenile idiopathic arthritis, adult spondyloarthropathies or systemic diseases in children and adults such as Behçet's disease, granulomatoses or multiple sclerosis. The differential diagnoses of pseudo-uveitis, sometimes related to neoplasia, and uveitis of infectious origin are discussed, as well as the different forms of uveitis according to their main anatomical location (anterior, intermediate, posterior or panuveitis). We also describe the symptoms, known physiopathological mechanisms, useful complementary ophthalmological and extra-ophthalmological examinations, therapeutic management, monitoring and useful information on the risks associated with the disease or treatment. Finally, this protocol presents more general information on the care pathway, the professionals involved, patient associations, adaptations in the school or professional environment and other measures that may be implemented to manage the repercussions of these chronic diseases. Because local or systemic corticosteroids are usually necessary, these treatments and the risks associated with their prolonged use are the subject of particular attention and specific recommendations. The same information is provided for systemic immunomodulatory treatments, immunosuppressive drugs, sometimes including anti-TNFα antibodies or other biotherapies. Certain particularly important recommendations for patient management are highlighted in summary tables.

Fecal calprotectin levels in acute anterior uveitis in patients with spondyloarthritis / Níveis de calprotectina fecal na uveite anterior aguda em pacientes com espondiloartrites

ABSTRACT Purpose: This study measured fecal calprotectin levels in a series of patients with anterior uveitis in order to determine whether anterior uveitis patients with associated spondyloarthritis have higher levels of fecal calprotectin than patients with anterior uveitis of other etiologies. A third group of patients with spondyloarthritis without uveitis was also evaluated to understand the role of acute anterior uveitis in increasing fecal calprotectin. Methods: In this cross-sectional study, 28 patients were divided into three groups: (a) Group 1, spondyloarthritis and uveitis (n=9); (b) Group 2, spondyloarthritis without uveitis (n=10); and (c) Group 3, uveitis without spondyloarthritis (n=9). The levels of fecal calprotectin were determined. Results: Groups 1 and 2 showed higher median fecal calprotectin levels (101.0 and 93.0 µg/g, respectively) compared with Group 3 (9.0 µg/g) (p=0.02). However, no relationship between fecal calprotectin levels and the presence of uveitis with spondyloarthritis could be demonstrated. Conclusion: Patients with spondyloarthritis with or without acute anterior uveitis have significantly elevated levels of fecal calprotectin. This test may be useful for differentiating spondyloarthrit-associated uveitis from uveitis of other etiologies.

RESUMO Objetivo: Este estudo avaliou os níveis de calprotectina fecal em uma série de pacientes com uveíte anterior na tentativa de determinar se pacientes com uveíte associada com espondiloartrites apresentam níveis mais elevados desta proteína do que pacientes com uveíte anterior de outras etiologias. Um terceiro grupo com espondiloartrites sem uveíte também foi incluído na avaliação para entendimento do papel da uveíte anterior no aumento da calprotectina fecal. Métodos: Estudo transversal de 28 pacientes divididos em três grupos: (a) com espondiloartrites e uveíte (n=9); (b) com espondiloartrites sem uveíte (n=10) e (c) com uveíte sem espondiloartrites (n=9). A dosagem de calprotectina fecal foi avaliada. Resultados: Pacientes com uveíte anterior associada a espondiloartrites apresentaram valores medianos maiores de calprotectina fecal (101 µg/g) que os valores dos pacientes com uveíte sem espondiloartrites (9 µg/g), pacientes com espondiloartrites sem uveíte que também demonstraram valores maiores (93.0 µg/g) que os dos pacientes com uveíte sem espondiloartrites (p=0,02). Conclusão: Pacientes com espondiloartrites com e sem uveíte anterior aguda demonstraram níveis significativamente elevados de calprotectina fecal. Este teste pode ser útil na diferenciação entre uveítes associadas com espondiloartrites de uveítes de outras etiologias. Entretanto, não foi possível demonstrar associação entre o aumento dos níveis de calprotectina fecal e a presença da uveíte em espondiloartrites.

[Sarcoid uveitis: Ophthalmologist's and internist's viewpoints]. / Uvéites sarcoïdosiques : regards croisés de l'ophtalmologiste et de l'interniste.

Sarcoidosis is one of the leading causes of inflammatory eye disease. All ocular structures can be affected, but uveitis is the main manifestation responsible for vision loss in ocular sarcoidosis. Typical sarcoid anterior uveitis presents with mutton-fat keratic precipitates, iris nodules, and posterior synechiae. Posterior involvement includes vitritis, vasculitis, and choroidal lesions. Cystoid macular edema is the most important and sight-threatening consequence of sarcoid uveitis. Patients with clinically isolated uveitis at diagnosis rarely develop other organ involvement. Even though, ocular sarcoidosis can have a severe impact on visual prognosis, early diagnosis and a wider range of available therapies (including intravitreal implants) have lessened the functional impact of the disease, particularly in the last decade. Corticosteroids are the cornerstone of treatment for sarcoidosis, but up to 30% of patients achieve remission with requiring high-dose systemic steroids. In these cases, the use of steroid-sparing immunosuppressive therapy (such as methotrexate) is unavoidable. Among these immunosuppressive treatments, anti TNF-α drugs have been a revolution in the management of non-infectious uveitis.

Coriorretinopatia de Birdshot: relato de caso e perspectivas sobre o tratamento / Birdshot chorioretinopathy: a case report and perspectives on its treatment

RESUMO A coriorretinopatia de Birdshot é uma uveíte posterior bilateral crônica rara que acomete, preferencialmente, mulheres de meia-idade. O quadro clínico é composto de pouco ou nenhum processo inflamatório de segmento anterior, associado a vitreíte e lesões coriorretinianas ovoides branco-amareladas de característica hiperfluorescente na angiofluoresceinografia e hipofluorescente na angiografia com indocianina verde. O tratamento se dá por meio de corticoides e outras drogas imunossupressoras. Todavia, em alguns casos, a doença é refratária a tal terapêutica, sendo necessário lançar mão de outras drogas, como os agentes biológicos. O presente artigo busca relatar um caso de coriorretinopatia de Birdshot em ajuste de terapia imunossupressora que evoluiu com má resposta às drogas iniciais e bom controle após uso de imunobiológico e discutir as opções terapêuticas disponíveis atualmente.

ABSTRACT Birdshot chorioretinopathy is a rare chronic bilateral posterior uveitis that preferentially affects middle-aged women. The clinical picture is composed of little or no anterior segment inflammatory process, associated with vitritis and yellowish-white ovoid chorioretinal lesions with hyperfluorescent characteristics on fluorescein angiography and hypofluorescent characteristics on green indocyanine green angiography. Treatment is with corticosteroids and other immunosuppressive drugs. However, in some cases, the disease is refractory to such therapy, making it necessary to resort to other drugs such as biological agents. The present article seeks to report a case of Birdshot chorioretinopathy in an adjustment of immunosuppressive therapy that evolved with poor response to the initial drugs and good control after the use of immunobiologicals and discuss the currently available therapeutic options.

Congenital ocular toxoplasmosis in consecutive siblings / Toxoplasmose ocular congênita em irmãos consecutivos

ABSTRACT Toxoplasma gondii infection can cause ocular manifestations after acquired and congenital disease. We report two cases of symptomatic congenital toxoplasmosis with ocular involvement in non-twin siblings, with a 2-year interval between pregnancies. Vertical transmission of toxoplasmosis in successive pregnancies, which was once considered impossible, is now found to be plausible even in immunocompetent subjects.

RESUMO A infecção pelo Toxoplasma gondii pode causar manifestações oculares tanto após a sua forma congênita quanto a sua forma adquirida. Reportamos aqui dois casos de toxoplasmose congênita sintomática com envolvimento ocular em irmãos não gêmeos, com intervalo de 2 anos entre gestações. A transmissão vertical da toxoplasmose em gestações sucessivas, outrora considerada impossível, é um evento plausível mesmo em indivíduos imunocompetentes.

Infliximab therapy in Behçet's uveitis.

PURPOSE: To evaluate the efficacy of infliximab (IFX) therapy in patients with Behçet's uveitis (BU) refractory to conventional immunomodulatory treatment (IMT). MATERIALS AND METHODS: This study, trial registration number TCTR20200806007, included cases of BU with a minimum of 18 months follow-up on IFX treatment. Demographic characteristics, ophthalmological examination findings, control of ocular inflammation with IFX, response to treatment and the rate of clinical remission were analyzed in this study. RESULTS: Sixty-two eyes of 35 patients on IFX therapy were included in the study. The mean follow-up was 49.5±25.9months. The mean frequency of recurrences during the IMT was 1.47±0.78 (attacks/year), decreasing to 0.31±0.40 (attacks/year) with IFX (P<0.001). Visual acuity improved significantly in the 1st month of IFX treatment (P=0.026). Partial response to treatment was achieved in 91.4% of cases. CONCLUSION: IFX is a safe and effective treatment in cases of BU refractory to conventional IMT.

[Colchicine and non-severe ocular inflammation excluding Behcet's disease: 16 cases and literature review]. / Colchicine et inflammation oculaire non sévère hors maladie de Behcet : 16 cas et revue de la littérature.

INTRODUCTION: Colchicine is a narrow therapeutic margin drug that does not have the adverse effects of corticosteroids and immunosuppressants. Its use in non-severe ocular inflammatory disease excluding Behcet's disease has not been studied. METHODS: We included patients seen in the internal medicine department of Dijon University Hospital consecutively between September 2020 and September 2021 if they had received colchicine during their pathology. Patients with suspected Behçet's disease were excluded. Treatment efficacy was studied in patients with at least one year of disease progression who had received more than one year of colchicine. Successful treatment was defined as a 50 % reduction in the number of annual relapses on colchicine. RESULTS: Sixteen patients were included (9 women and 7 men). They had recurrent anterior uveitis (n=10), recurrent scleritis (n=5) and intermediate uveitis. Opthalmological involvement was neither severe nor complicated. All patients combined, the annual relapse ratio (ARR) decreased from 1.8 (0.8-3.5) to 0.3 (0-1.6), (P=0.06). Colchicine was considered effective in three of 10 analyzable patients. In only one patient, treatment was stopped for adverse effects after six weeks. CONCLUSION: In view of the interesting benefit-risk ratio of colchicine, it seems appropriate to focus on this molecule in non-granulomatous anterior uveitis and non-severe recurrent scleritis.

[XEN 45® gel stent implantation in glaucoma surgery: Dual-center comparative study between steroid-induced glaucoma and primary open angle glaucoma]. / Implantation du dispositif XEN 45® dans la chirurgie du glaucome : étude bicentrique bispective s'intéressant au glaucome cortisonique par rapport au glaucome primitif à angle ouvert.

INTRODUCTION: XEN 45® gel stent is an ab interno aqueous humor drainage device indicated for moderate glaucoma refractory to medical management. Its efficacy has been demonstrated in primary open-angle glaucoma (POAG). However, there are few studies on secondary glaucoma, including steroid-induced glaucoma (CG), defined as optic neuropathy induced by using local or systemic corticosteroids without increased flare. METHODS: We conducted a dual-center comparative cohort study between April 2019 and January 2021. 66 operated eyes were included, divided into two groups: POAG (56 eyes) and GC (10 eyes). The primary endpoint was the relative reduction in intraocular pressure (IOP) at three months postoperatively in the GC group. Three outcomes were defined: total success, partial success and failure. RESULTS: The total success rate was 100% in the GC group and 42.6% in the POAG group. Preoperative IOP was 36.1±9.1mmHg and 19.0±7.3mmHg respectively. IOP reduction was 69.1±11.7% in the GC group and 21.8±30.3% in the POAG group. Patients were younger in the GC group (49.3±21.2 versus 71.1±8.4 years), and preoperative conjunctival preparation was longer in this group (12 versus 5 weeks). The needling rate was 17.9% in the POAG group and 10% in the GC group. CONCLUSION: The XEN 45® gel stent is effective in the treatment of steroid-induced glaucoma. Further studies will be required to identify predictive factors for success and to establish criteria for good candidacy.

[Ocular toxicity of targeted therapies with MEK inhibitors and BRAF inhibitors in the treatment of metastatic cutaneous melanoma]. / Toxicité oculaire des thérapies ciblées anti-MEK et anti-BRAF dans le traitement des mélanomes cutanés métastatiques.

INTRODUCTION: Cutaneous melanoma is a malignant tumor, which develops from dermal melanocytes. Targeted therapies have changed the therapeutic management of metastatic melanoma and improved the survival rate. Among the various targeted therapies, MEK inhibitors and BRAF inhibitors have demonstrated efficacy, but they may lead to ocular toxicity. The goal of this study was to assess the incidence of ocular complications caused by the use of MEK inhibitors and BRAF inhibitors and to report their clinical features and therapeutic management. MATERIAL AND METHODS: This retrospective, observational, descriptive, single center study was conducted between May 2015 and December 2019 and included all patients with metastatic cutaneous melanomas treated with MEK inhibitors and BRAF inhibitors in whom ophthalmic toxicity was suspected. The data collected were demographic data (age, sex), the type of MEK inhibitors and BRAF inhibitors used, the length of time from melanoma diagnosis, mean duration of ophthalmological follow-up, time differential between starting therapy and the emergence of ocular complications, initial and final logMAR visual acuity, biomicroscopic examination of the anterior segment, dilatated fundus examination, and treatment administered. RESULTS: Fifty-four eyes of 27 patients with a mean age of 61.3±14.3 were included. The mean time delay between melanoma diagnosis and initiation of treatment was 23.2±8 months. Twenty patients (74%) were treated with a combination of MEK inhibitors and BRAF inhibitors (trametinib/dabrafenib), 5 patients (19%) were treated with MEK inhibitor monotherapy (cobimetinib), and 2 patients (7%) were treated with BRAF inhibitor monotherapy (vemurafenib). The mean duration of ophthalmological follow-up was 77.8±29 days, and the delay between the start of therapy and the emergence of symptoms was 87.2±78 days. The mean initial visual acuity was 0.075±0.13 logMAR, and the final visual acuity was 0.01±0.03 logMAR. Twelve patients (44%) developed ocular complications due to the targeted therapy. In the patients who received combination trametinib/dabrafenib, 5 patients (18.5%) developed clinical signs of uveitis, from acute anterior uveietis to panuveitis, and 2 patients (7.4%) developed bilateral central serous chorioretinopathy; in the patients who received cobimetinib, 4 patients (14.8%) developed bilateral central serous chorioretinopathy; and one patient (3.7%) who received vemurafenib developed acute anterior uveitis. For these 12 patients with ophthalmic side effects, temporary discontinuation of therapy was chosen for six patients (22.2%), three patients (11.1%) received half the initial dose, and for three patients (11.1%), normal dosing was continued. CONCLUSION: The two main side effects of targeted therapies are uveitis for BRAF inhibitors and central serous chorioretinopathy for MEK inhibitors. A multidisciplinary approach including ophthalmologists, dermatologists and oncologists is essential in order to adapt treatment in the advent of these ocular complications.

Simultaneous ocular and osseous syphilis: a case report / Sífilis com envolvimentos ocular e ósseo simultâneos: um relato de caso

ABSTRACT Syphilis is a reemerging and potentially serious disease. Owing to its ubiquity and pleomorphism, it is called "the great imitator". We report the case of a young woman with secondary syphilis who presented with bilateral acute syphilitic posterior placoid chorioretinopathy along with a syphilitic skull periostitis. A pachymeningeal enhancement was observed on magnetic resonance imaging, but we believe it was an extension of the bone process rather than a meningitis itself on the basis of the normal cerebrospinal fluid analysis results. Treatment with intravenous crystalline penicillin resulted in complete resolution of the signs, symptoms, and imaging findings. Secondary syphilis is the stage with the highest bacteremia and the highest transmissibility, presenting mainly with mucocutaneous disorders and, less frequently, with involvement of other organs. High suspicion and a pragmatic approach are essential to the diagnosis because this disease can affect several organs, as in the present case, in which the eyes, bones, and skin were affected.

RESUMO A sífilis é uma doença reemergente e potencialmente grave. Por sua onipresença e pleomorfismo, é denominada "grande imitadora". Relatamos caso de paciente jovem com sífilis secundária, que se apresentou com coriorretinopatia placóide sifilítica posterior aguda bilateral, simultaneamente a periostite craniana sifilítica. A despeito de realce paquimeníngeo observado na ressonância magnética, acreditamos que este tenha sido uma extensão do processo ósseo e não, uma meningite em si, uma vez que o exame do líquido cefalorraquidiano estava completamente normal. Tratamento com penicilina cristalina intravenosa resultou em completa resolução dos sinais, sintomas e achados de imagem. A sífilis secundária é o estágio de maior bacteremia e maior transmissibilidade da doença, apresentando-se principalmente com quadros mucocutâneos, mas também, menos frequentemente, com envolvimento de outros órgãos. Elevada suspeição e uma abordagem pragmática são necessárias para o diagnóstico, uma vez que essa doença pode afetar vários órgãos, como no caso relatado, em que foram acometidos olhos, ossos e pele.

Causes and characteristics of uveitis cases at a reference university hospital in Rio de Janeiro, Brazil / Causas e características das uveítes em hospital universitário de referência no Rio de Janeiro, Brasil

ABSTRACT Purpose: The aim of this study was to describe the epidemiological profile of uveitis cases treated at University Hospital Clementino Fraga Filho and to identify the presentation pattern of intraocular inflammation on the basis of clinical, anatomical, etiological, and demographic criteria. Methods: A retrospective study was conducted using data from the medical records of 408 patients with active disease who attended the ophthalmology service between March and October 2018. Age, sex, visual acuity at the time of diagnosis, anatomical and etiological diagnoses, the clinical aspect, and the main symptoms reported during anamnesis were described. Results: Of the 408 patients in the study, 52% were male and 48% were female. The patients' mean age was 42 years, and most (84%) were between 19 and 64 years old. Anterior uveitis was observed in 37.75% of the patients; posterior uveitis, in 49.75%; panuveitis, in 4.66%; and intermediate uveitis, in 3.43%. Only 18 patients (4.41%) presented with scleritis. Of the 390 patients with anatomical classifications, 76% had known etiologies, with the most prevalent diagnoses being toxoplasmosis (35.4%), followed by juvenile idiopathic arthritis (6.4%), ankylosing spondylitis (5.9%), and syphilis (4.9%). Infectious uveitis corresponded to 49.7% of the patients, while 26.6% of the cases were of noninfectious origin. Anterior uveitis had the highest number of cases classified as idiopathic (49.4%). In the cases of posterior uveitis, the etiology was established 94% of the time. The most frequent symptoms were ocular pain (71.8%) and blurring vision (56.8%). Conclusions: The present study confirmed the historical importance of infectious uveitis in our population, especially ocular toxoplasmosis. Uveitis appears to have no predilection for sex but mainly affects young people of working age, thus generating social and economic consequences. Despite the evolution of diagnostic methods, idiopathic uveitis remains one of the major etiologies. Epidemiological studies point to different presentation patterns of uveitis in different populations, but these may reflect the distinct characteristics of each institution.

RESUMO Objetivo: Descrever o perfil epidemiológico das uveítes atendidas no Hospital Universitário Clementino Fraga Filho - UFRJ. Identificando o padrão de apresentação da inflamação intraocular a partir de critérios clínicos, anatômicos, etiológicos e demográficos. Métodos: Estudo retrospectivo, com base em prontuários de 408 pacientes com doença ativa, atendidos no serviço de oftalmologia no período de março a outubro de 2018. Foram descritos a idade, sexo, acuidade visual no momento do diagnóstico, diagnóstico anatômico e etiológico, aspecto clínico, além dos principais sintomas relatados durante a anamnese. Resultados: Dos 408 pacientes do estudo, 52% eram do sexo masculino e 48% do feminino. A idade média dos pacientes foi de 42 anos, a maioria (84%) entre 19 e 64 anos. Uveíte anterior foi observada em 37,75% dos pacientes, uveíte posterior em 49,75%, panuveíte em 4,66% e uveíte intermediária em 3,43%; apenas 18 pacientes (4,41%) apresentaram diagnóstico de esclerite. Dos 390 pacientes com classificação anatômica, a etiologia foi determinada em 76% deles, com os diagnósticos mais prevalentes sendo Toxoplasmose (35,4%), artrite idiopática juvenil (6,4%), espondilite anquilosante (5,9%) e sífilis (4,9%). ) A uveíte infecciosa correspondeu a 49,7% desses pacientes, enquanto 26,6% eram de origem não infecciosa. A uveíte anterior teve o maior número de casos classificados como idiopáticos (49,4%), enquanto a uveíte posterior teve a etiologia estabelecida em 94% das vezes. Os sintomas mais frequentes foram dor ocular (71,8%) e visão embaçada (56,8%). Conclusões: O presente estudo confirmou a importância histórica da uveíte infecciosa em nossa população, principalmente a toxoplasmose ocular. As uveítes parecem não ter predileção por sexo, mas afetam principalmente jovens em idade ativa, gerando consequências sociais e econômicas. Apesar da evolução nos métodos diagnósticos, a uveíte idiopática continua sendo uma das principais causas. Estudos epidemiológicos apontam para diferentes padrões de uveíte nas populações, estes podem refletir características particulares de cada instituição.

Psychometric properties of CVFQ7-BR-toxo to evaluate vision-related quality of life in children with congenital toxoplasmosis in Brazil / Propriedades psicométricas do CVFQ7-BR-toxo para avaliar a qualidade de vida relacionada à visão em crianças com toxoplasmose congênita no Brasil

ABSTRACT Purpose: The high prevalence and severity of congenital toxoplasmosis in Brazil, with several affected individuals progressing to low vision, emphasize the importance of evaluating their quality of life. In this study, the Children's Visual Function Questionnaire (CVFQ) was adapted to the sociocultural context of Brazilian children, and its psychometric properties were investigated for evaluating the vision-related quality of life of these individuals. Methods: This was a nested cross-sectional epidemiological study that prospectively monitored a cohort of 142 preschool children at a single referral university hospital in Belo Horizonte, Brazil. All children underwent complete ophthalmological examination, including visual acuity testing and binocular indirect ophthalmoscopy. Questionnaires were applied to their parents and caregivers to evaluate quality of life perception, as well as socioeconomic status of their families. Psychometric properties of the quality of life scale were evalua­ted by multivariate statistical analyses. Results: Adaptation to the Brazilian version of CVFQ-7 resulted in CVFQ-BR-toxo, a questionnaire for evaluating the perception of parents/caregivers about the vision-related quality of life of preschool children with congenital toxoplasmosis. The following six subscales were identified based on description, variability structure, and interpretation/grouping of items: general health, visual acuity, visual performance/functional vision, personal and social behavior, impact on family, and treatment. Children with low vision related to congenital toxoplasmosis had significantly lower scores for the following subscales: visual acuity (p=0.004), visual performance/functional vision (p=0.008), impact on family (p=0.001), and overall health (p=0.001). Conclusion: Psychometric properties were appropriate concerning the validity of the quality of life construct. CVFQ-BR-toxo could demonstrate the impact of vision impairment on families of children with congenital toxoplasmosis.

RESUMO Objetivo: A alta prevalência e gravidade da toxoplasmose congênita no Brasil, com muitos indivíduos afetados desenvolvendo baixa visão, reforça a importância da avaliação da sua qualidade de vida. Este estudo tem como objetivo adaptar o Children's Visual Function Questionnaire (CVFQ) para a realidade sociocultural de crianças brasileiras e investigar suas propriedades psicométricas para avaliação da qualidade de vida relacionada à visão nesses indivíduos. Métodos: Estudo epidemiológico transversal aninhado de coorte de 142 crianças pré-escolares acompanhadas prospectivamente em hospital universitário de referência em Belo Horizonte, Brasil. Todas foram submetidos a exame oftalmológico completo, incluindo medida da acuidade visual e oftalmoscopia binocular indireta. Questionários foram aplicados aos pais e cuidadores, para avaliar a percepção da qualidade de vida, bem como o nível sócio-econômico das famílias. Análise estatística multivariada foi realizada para avaliar as propriedades psicométricas da escala de qualidade de vida. Resultados: Adaptações na versão brasileira do Children's Visual Function Questionnaire-7 originaram o Children's Visual Function Questionnaire-7-BR-toxo, um questionário para avaliar a percepção de pais/cuidadores sobre a qualidade de vida relacionada à visão de crianças pré-escolares com toxoplasmose congênita. Pela descrição, estrutura de variabilidade, e interpretação do agrupamento dos itens do questionário adaptado, identificaram-se seis subescalas: saúde geral, capacidade visual, desempenho visual/visão funcional, comportamento social e pessoal, impacto na família e tratamento. Crianças com baixa visão associada a toxoplasmose congênita tiveram escores mais baixos nas seguintes subescalas: acuidade visual (p=0,004), desempenho visual/visão funcional (p=0,008), impacto na família (p=0,001) e saúde geral (p=0,001). Conclusão: As propriedades psicométricas foram adequadas no tocante à validade do construto. O Children's Visual Function Questionnaire-7-BR-toxo foi capaz de registrar o impacto da deficiência visual nas famílias de crianças com toxoplasmose congênita.

Postoperative anterior uveitis in a patient submitted to combined treatment with cataract surgery and iStent inject®. How to manage? / Uveíte anterior no pós-operatório de facectomia combinada com implante de iStent inject®. Como manejar?

ABSTRACT Minimally invasive glaucoma surgeries are surgical treatment alternatives for glaucoma aimed at reducing intraocular pressure with a better safety profile compared to traditional trabeculectomy. However, in spite of less invasive techniques, complications may develop in any surgical procedure. To the best of our knowledge, this is the first case report of anterior uveitis following combined treatment with cataract surgery and iStent inject® which addresses the management of postoperative inflammation.

RESUMO As cirurgias minimamente invasivas para glaucoma consistem em uma opção de tratamento cirúrgico para glaucoma, a qual promove redução da pressão intraocular com melhor perfil de segurança do que a trabeculectomia. Todavia, complicações são inerentes à realização de procedimentos cirúrgicos, apesar do uso de técnicas menos invasivas. Este é o primeiro relato que apresenta um caso de uveíte anterior após cirurgia combinada de catarata e iStent inject®, além de orientações quanto ao manejo do quadro inflamatório.

Treatment of noninfectious uveitis / Tratamento das uveítes não-infecciosas

ABSTRACT Uveitis is a broad term that refers to a large group of eye disorders categorized by intraocular inflammation, a leading cause of visual impairment. Historically, treatment of noninfectious uveitis has depended on corticosteroid drugs. Owing to the myriad of side effects caused by corticosteroids, immunomodulatory therapy has become the preferred treatment for chronic noninfectious intraocular inflammation. Recently, biological response modifiers have established a new era in uveitis therapy, with the range of targets continuing to expand. In this review, we aimed to convey up-to-date information on the treatment of noninfectious uveitis to the general ophthalmologist.

RESUMO Uveíte é um termo amplo utilizado para denominar várias desordens categorizadas como inflamação intraocular, uma causa importante de deficiência visual. Historicamente, o tratamento das uveítes não infecciosas baseou-se no uso de corticosteróides. Devido aos diversos efeitos colaterais do uso de corticosteróides a longo prazo, a terapia imunomoduladora é indicada no tratamento das uveítes não infecciosas crônicas. A introdução dos medicamentos biológicos estabeleceu uma nova era no tratamento das uveítes, com constante desenvolvimento de novas drogas. O objetivo desta revisão é trazer informações atuais sobre tratamento das uveítes não infecciosas para a prática clínica do oftalmologista geral.