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OBJECTIVES: To investigate clinical features associated with lack of response to MTX in juvenile idiopathic arthritis associated uveitis (JIA-U). METHODS: Clinical records of JIA-U patients were retrospectively reviewed. Differences among variables were assessed by Mann-Whitney and χ 2 or Fisher's exact tests as appropriate. Association between predictors and requirement of a biological disease modifying antirheumatic drug (bDMARD) was evaluated by univariate Cox regression analysis and Kaplan-Meier curves. A multivariable logistic model was applied to estimate strength of association, adjusting for potential confounders. RESULTS: Data from 99 JIA-U patients treated with MTX were analysed (82.8% female), with a mean follow up of 9.2 years and a mean age at uveitis onset of 5.7 years. In 65 patients (65.7%) at least one bDMARD to control uveitis was required. Children requiring a bDMARD for uveitis had lower age at JIA and uveitis onset, more frequent polyarticular course, higher frequency of bilateral uveitis at onset and higher prevalence of systemic steroids' use. Despite similar frequency of ocular damage at onset, MTX non responders showed a higher percentage of ocular damage at last visit. Younger age at JIA onset, polyarticular course and a history of systemic steroids' use resulted independent factors associated to lack of response to MTX at Cox regression analysis. Kaplan-Meier curves and the multivariate model confirms the independent role of both polyarticular course and systemic steroids' use. CONCLUSIONS: Younger age at JIA onset, polyarticular course and a history of systemic steroids' use are predictors of a worse response to MTX in JIA-U.
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OBJECTIVES: This study aims to evaluate the efficacy and safety of JAK inhibitors (JAKi) in a monocentric cohort of adult patients with juvenile idiopathic arthritis (JIA). METHODS: Patients attending a rheumatology transition clinic were retrospectively included in case of: i) JIA diagnosis according to current classification criteria (1); ii) age ≥18 years and iii) treatment with JAKi for at least 3 months. RESULTS: Seventeen adult patients with JIA were treated with JAKi (as first JAKi, 9 patients (52.9%) received tofacitinib and 8 (47.1%) baricitinib). At 3 months after JAKi initiation, 8 patients (47%) achieved a response and 4 patients (23.5%) achieved disease remission (3 patients with baricitinib and 1 with tofacitinib, 37.5% vs. 16.7%, p=0.294). None of those with systemic JIA and enthesitis-related arthritis obtained remission; the remission rate at 3 months was higher, although not significantly, in the oligoarticular subset compared to the polyarticular subset (37.5% vs. 20%). Patients with ≤1 active joint involvement at JAKi start had a higher remission rate (50% vs. 22.2%). Subjects who achieved remission on JAKi had a significantly lower pre-treatment DAS28-CRP compared to those with still active disease (p=0.010, Mann-Whitney U=4). A pre-treatment DAS28-CRP <3.76 predicted response to JAKi with 100% sensitivity and 84.6% specificity (p=0.023). The remission rate was lower among patients who had been treated with ≥2 biological drugs before JAKi start (9% vs. 66.7%; p=0.05). One patient in concomitant treatment with leflunomide developed severe arterial hypertension. CONCLUSIONS: JAKi may represent an effective and safe treatment option for adult JIA patients with low/moderate disease activity, particularly in case of oligoarticular involvement.
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Artritis Juvenil , Azetidinas , Inhibidores de las Cinasas Janus , Piperidinas , Purinas , Pirazoles , Pirimidinas , Inducción de Remisión , Sulfonamidas , Humanos , Artritis Juvenil/tratamiento farmacológico , Estudios Retrospectivos , Inhibidores de las Cinasas Janus/uso terapéutico , Inhibidores de las Cinasas Janus/efectos adversos , Masculino , Femenino , Adulto , Piperidinas/uso terapéutico , Piperidinas/efectos adversos , Pirimidinas/uso terapéutico , Pirimidinas/efectos adversos , Resultado del Tratamiento , Pirazoles/uso terapéutico , Pirazoles/efectos adversos , Azetidinas/uso terapéutico , Azetidinas/efectos adversos , Adulto Joven , Sulfonamidas/uso terapéutico , Adolescente , Purinas/uso terapéutico , Purinas/efectos adversos , Antirreumáticos/uso terapéutico , Antirreumáticos/efectos adversos , Factores de TiempoRESUMEN
PURPOSE: To investigate the pathophysiology and prognostic significance of acute Henle fiber layer (HFL) hyperreflectivity in placoid diseases by examining its relationship with impaired choroidal flow and persistent photoreceptor disruption. METHODS: Retrospective-prospective observational study on patients with placoid diseases. Indocyanine green angiography and optical coherence tomography were performed during the acute phase and follow-up. Impaired choroidal flow, HFL hyperreflectivity, and persistent ellipsoid zone disruption, their colocalization index, and their associations with initial and final visual acuity were explored. RESULTS: Sixteen eyes from eight patients (mean age, 25.3 ± 6.44 years) were included (median follow-up, 13.5 months). Quantitative analysis revealed significant correlations between areas of impaired choroidal flow, HFL hyperreflectivity, and persistent ellipsoid zone disruption (correlation coefficients of 0.69, 0.63, and 0.46, respectively). Impaired choroidal flow area exceeded HFL hyperreflectivity (P = 0.002) and ellipsoid zone disruption (P = 0.003). A noteworthy 94% nonrandom overlap between HFL hyperreflectivity and ellipsoid zone disruption was observed. Worse initial visual acuity correlated with foveal involvement (P = 0.0002), thicker choroid (P = 0.001), larger impaired choroidal flow areas (P = 0.02), and thinner outer retina post lesion inactivation (P = 0.04). CONCLUSION: Henle fiber layer hyperreflectivity predicted photoreceptor recovery potential in placoid diseases. If HFL hyperreflectivity corresponds to acute HFL damage, it may suggest more severe involvement of the entire photoreceptor length.
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Coroides , Angiografía con Fluoresceína , Tomografía de Coherencia Óptica , Agudeza Visual , Humanos , Tomografía de Coherencia Óptica/métodos , Femenino , Masculino , Adulto , Estudios Retrospectivos , Agudeza Visual/fisiología , Angiografía con Fluoresceína/métodos , Estudios Prospectivos , Pronóstico , Adulto Joven , Coroides/patología , Coroides/diagnóstico por imagen , Coroides/irrigación sanguínea , Adolescente , Estudios de Seguimiento , Enfermedades de la Retina/fisiopatología , Enfermedades de la Retina/diagnóstico , Fondo de Ojo , Enfermedad AgudaRESUMEN
PURPOSE: To perform an unsupervised machine learning clustering of patients with punctate inner choroidopathy (PIC) and provide new insights into the significance of pachychoroid disease features in PIC eyes. METHODS: Retrospective multicenter study, including 102 eyes from 82 patients diagnosed with PIC. Demographics, clinical data, and multimodal imaging, including fundus photography, optical coherence tomography, and indocyanine green angiography, were collected. Clusters of eyes were identified, and multilevel logistic regression analysis was performed to compare between-group differences. RESULTS: Using 17 clinical features, two distinct clusters of patients with PIC were identified. Cluster 1 patients were characterized by older age, high myopia, myopic maculopathy features, thin choroids, multiple lesions, and a higher likelihood of developing patchy chorioretinal atrophy. Cluster 2 consisted of younger age, emmetropia or low myopia, thick choroids, choroidal vascular hyperpermeability on late-phase indocyanine green angiography, and high prevalence of focal choroidal excavation. These features exhibited significant differences ( P < 0.05) between the two clusters. CONCLUSION: While PIC typically affects young myopic female patients with thin choroids, a subset of patients with PIC exhibits features associated with pachychoroid disease. Considering the potential influence of choroidal venous insufficiency on PIC manifestations and secondary complications, we propose the term "punctate inner pachychoroidopathy" to characterize this distinct subtype of PIC.
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Miopía , Síndromes de Puntos Blancos , Femenino , Humanos , Coroides/patología , Demografía , Angiografía con Fluoresceína/métodos , Verde de Indocianina , Inflamación , Miopía/patología , Estudios Retrospectivos , Tomografía de Coherencia Óptica/métodos , Síndromes de Puntos Blancos/diagnóstico , MasculinoRESUMEN
PURPOSE: To describe the clinical characteristics and multimodal imaging features of a distinctive subtype of active idiopathic multifocal choroiditis (iMFC) lesions with grey-yellow chorioretinal lesions surrounded by smaller satellite dots, a presentation referred to as "chrysanthemum lesions." METHODS: Retrospective, observational, multicenter case series of eyes with active iMFC and chrysanthemum lesions. Multimodal imaging features were reviewed and presented. RESULTS: Twenty-five eyes from 20 patients (12 women and 8 men), with a mean age of 35.8 ± 17.0 years (range, 7-78 years) were included. Chrysanthemum lesions were equally located in the macula (48.0%) or the mid/far periphery (52.0%). The number of lesions per eye varied from 1 (16.0%) to more than 20 (56.0%). On optical coherence tomography, chrysanthemum lesions showed typical features of iMFC, including subretinal hyperreflective material splitting the retinal pigment epithelium/Bruch membrane. Chrysanthemum lesions were hypoautofluorescent on fundus autofluorescence imaging, hyperfluorescent on fluorescein angiography, hypofluorescent on indocyanine green angiography, and associated with choriocapillaris flow signal deficit on optical coherence tomography angiography. CONCLUSION: Active iMFC may present with findings resembling chrysanthemum lesions. The distinctive lesion morphology on ophthalmoscopic examination, the large number of lesions, and the high prevalence of exclusive midperipheral and far peripheral involvement may represent a distinctive phenotype of iMFC.
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Coroiditis , Humanos , Coroiditis Multifocal , Estudios Retrospectivos , Fondo de Ojo , Coroiditis/diagnóstico , Coroides/patología , Angiografía con Fluoresceína/métodos , Tomografía de Coherencia Óptica/métodosRESUMEN
PURPOSE: To describe the clinical and multimodal imaging features of stellate multiform amelanotic choroidopathy (SMACH; also known as serous maculopathy due to aspecific choroidopathy). METHODS: Retrospective observational case series of eyes presenting with SMACH. Multimodal imaging including fundus photography, optical coherence tomography (OCT), OCT angiography (OCTA), and indocyanine green angiography (ICGA) was analyzed. RESULTS: Eighteen eyes from 18 patients (mean age: 28 ± 19 years) were included. The mean follow-up duration was 9 years. Ophthalmoscopy showed a yellowish orange, dendriform choroidal lesion. At presentation, subretinal fluid (SRF) was seen in 10 of 18 cases (56%). Eight patients (44%) showed no evidence of SRF during a mean follow-up of 6 years. Cross-sectional OCT showed hyperreflective fibrous-like changes within the inner choroid with choriocapillaris flow preservation on OCTA. En face OCT showed a hyperreflective choroidal lesion with finger-like projections oriented in a stellate configuration. On ICGA, SMACH showed early and late hypofluorescence. None of the cases showed lesion growth. CONCLUSION: SMACH seems to be a unilateral choroidopathy characterized by distinctive multimodal imaging features. As SRF was absent in some cases, while a dendriform pattern was a consistent finding in all eyes, the authors propose renaming this entity "stellate multiform amelanotic choroidopathy," a name that retains its previous abbreviation "SMACH."
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Enfermedades de la Retina , Adolescente , Adulto , Niño , Humanos , Persona de Mediana Edad , Adulto Joven , Coroides/patología , Estudios Transversales , Angiografía con Fluoresceína/métodos , Verde de Indocianina , Imagen Multimodal/métodos , Enfermedades de la Retina/patología , Estudios Retrospectivos , Tomografía de Coherencia Óptica/métodosRESUMEN
PURPOSE: To identify the characteristics and outcome of panuveitis of undetermined origin (PUO) after diagnostic vitrectomy. METHODS: Retrospective analysis of all patients who underwent vitrectomy for diagnostic/therapeutic purposes from 2013 to 2020, whose vitreous biopsies turned out negative and final diagnoses were not clinically supported. RESULTS: Of 122 operated eyes, 36 eyes (29.5%) were defined as PUO (67.8 ± 14.9 years). The presenting clinical picture revealed a predominantly bilateral condition (70% of eyes) with significant posterior segment involvement: 3.1 ± 0.6 vitritis, 61.1% of eyes with retinal vasculitis, 44.4% with macular edema, and 30.6% with exudative retinal detachment. Presenting visual acuity was 1.2 ± 0.7 logMAR, and up to 90% remained stable or improved vision over a ~ 3.5 year observation period. None of the presenting clinical features turned out to be predictive of final visual outcome or survival. CONCLUSIONS: PUO is present in up to 30% of cases after diagnostic/therapeutic vitrectomy. This mainly bilateral condition shows chronic and overall stable long-term outcome, generally with retained steady visual function.
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Panuveítis , Desprendimiento de Retina , Humanos , Vitrectomía , Estudios Retrospectivos , Centros de Atención Terciaria , Desprendimiento de Retina/cirugía , Panuveítis/diagnóstico , Panuveítis/cirugía , Resultado del TratamientoRESUMEN
PURPOSE: This study aims to report the long-term outcomes of uveitis-associated optic disc and epiretinal neovascularization (NV) treated with immunomodulatory therapy alone. METHODS: This is a retrospective, multi-center chart review conducted at Northwestern University (Chicago, IL) and San Raffaele Scientific Institute (Milan, Italy) from 2014 to 2021 of patients with optic disc and/or retinal neovascularization associated with uveitis. The data collected included age at the time of NV detection, gender, medications, and follow-up period. Imaging was reviewed if available. RESULTS: Eight eyes of six patients were identified. The mean age was 22 years (range 10-52 years); the median follow-up was 3 years (range 6 months to 7 years). All eyes presented with active NV at the time of uveitis onset; 7 eyes were treatment-naïve. None had clinical or angiographic evidence of retinal ischemia. All patients received a variable combination of local steroids, systemic steroids, and systemic immunosuppression. Complete resolution of uveitic NV occurred in all eyes within a median of 8 weeks (ranging 2-20 weeks) from initiating treatment. No NV recurrence was noted. CONCLUSION: Immunomodulatory therapy alone may be successful in achieving long-term control of uveitis-associated NV, without the use of destructive measures.
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Disco Óptico , Neovascularización Retiniana , Uveítis , Adolescente , Adulto , Niño , Estudios de Seguimiento , Humanos , Inmunomodulación , Persona de Mediana Edad , Estudios Retrospectivos , Uveítis/diagnóstico , Uveítis/tratamiento farmacológico , Adulto JovenRESUMEN
PURPOSE: To investigate the frequency, risk factors, and functional prognosis of chorioretinal atrophy (CRA) in vitreoretinal lymphoma (VRL). METHODS: This was a retrospective cohort study of consecutive patients with vitreoretinal lymphoma. The demographic, clinical, and retinal features and the treatment modalities of each patient were collected. The charts and the multimodal imaging at each visit were reviewed. The risk factors associated with CRA were investigated with a mixed-model Cox regression. RESULTS: Of the 79 eyes of 40 patients included, 41 eyes (52%) had CRA; 27 and 14 eyes had focal and diffuse CRA, respectively. The rate of vitreoretinal lymphoma lesions in the macula was similar between focal and diffuse CRA (96% vs. 93%). The eyes with CRA had worse best-corrected visual acuity (P = 0.006) than eyes with no CRA; diffuse atrophy had the worst best-corrected visual acuity (P < 0.001). The presence of retinal infiltrates (hazard ratio = 3.75, 95% confidence interval [CI] 1.46-9.59, P = 0.006) and vertical hyperreflective lesions (hazard ratio= 4.13 95% CI 1.14-14.93, P = 0.03) on optical coherence tomography and macular involvement (hazard ratio = 6.59, 95% CI 1.41-30.53, P = 0.02) were associated with a higher risk of CRA. CONCLUSION: Vitreoretinal lymphoma presenting with retinal infiltrates and macular involvement carried a higher risk of CRA. Risk factors for CRA should be identified for the potential of severe visual loss. Prompt diagnosis of vitreoretinal lymphoma may allow better control of the disease.
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Distrofias Hereditarias de la Córnea/etiología , Linfoma Intraocular/complicaciones , Neoplasias de la Retina/complicaciones , Cuerpo Vítreo/patología , Anciano , Anciano de 80 o más Años , Distrofias Hereditarias de la Córnea/diagnóstico por imagen , Distrofias Hereditarias de la Córnea/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Imagen Multimodal , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Factores de Riesgo , Tomografía de Coherencia Óptica , Agudeza Visual/fisiologíaRESUMEN
PURPOSE: To evaluate the retinal circulation in patients with active acute leukemia, to correlate the perfusion metrics with systemic laboratory values, and to assess the vascular perfusion after leukemia remission. METHODS: Longitudinal study of 22 eyes from 12 patients with acute leukemia; healthy eyes were recruited as control subjects. All patients underwent optical coherence tomography angiography at baseline. Optical coherence tomography angiography was repeated in case of morphologic leukemia remission. RESULTS: Patients' age ranged from 37 to 74 years. All participants had a 20/20 vision. In all leukemic eyes, optical coherence tomography angiography detected vascular alterations in the macula and the peripapillary region. Vessel density values in the superficial capillary plexus were lower in patients with leukemia than control subjects (46.8 ± 3.6 vs. 49.2 ± 2%, P = 0.08), irrespective of the presence of leukemic retinopathy (7 eyes, 32%). Lower vessel density was associated with lower white blood cells ( P = 0.09) and lower platelets ( P = 0.001). Reappearance of small capillaries, increase in vessel density, reduction in vessel diameter, and increase in fractal dimension were seen after remission. CONCLUSION: Subclinical, reversible reduction in vessel density and complexity on optical coherence tomography angiography occurs in patients with active acute leukemia and is presumably associated with bone marrow function failure. Further studies are warranted to explore its functional and prognostic significance.
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Leucemia , Mácula Lútea , Adulto , Anciano , Capilares , Angiografía con Fluoresceína/métodos , Humanos , Estudios Longitudinales , Mácula Lútea/irrigación sanguínea , Persona de Mediana Edad , Vasos Retinianos , Tomografía de Coherencia Óptica/métodosRESUMEN
PURPOSE: To report a cohort of patients with a punctate inner choroidopathy (PIC)-like reaction in concurrent, unrelated, chorioretinal disorders. METHODS: This was a retrospective observational study of patients seen at two referral centers with lesions consistent with PIC on multimodal imaging; patients with lesions resembling idiopathic multifocal choroiditis were also included. Active PIC-like lesions appeared as focal hyperreflective lesions splitting the retinal pigment epithelium/Bruch membrane (RPE/BrM) complex on optical coherence tomography. Chronic PIC-like lesions included subretinal fibrosis, multifocal punched-out chorioretinal atrophy, and curvilinear streaks. Patients' demographics, additional imaging features, and treatment responses were collected and summarized. RESULTS: Twenty-two eyes of 16 patients with a PIC-like reaction were included (75% females; median age 40 years). Underlying diagnoses included hereditary retinal conditions (10 patients, 63%) and acquired etiologies, all characterized by the RPE/BrM or outer retinal disruption. Fifteen eyes (68%) had active PIC-like lesions; seven eyes (32%) had chronic PIC-like lesions. Active PIC-like lesions regressed with time and responded to systemic steroids. Subretinal fibrosis (3 eyes, 20%), macular atrophy (3 eyes, 20%), and concomitant subretinal fibrosis and macular atrophy (5 eyes, 33%) developed on follow-up. Recurrences occurred in five eyes (23%). CONCLUSION: RPE/BrM or outer retina disruption may trigger a PIC-like reaction in susceptible patients, presumably because of the loss of immune privilege. A PIC-like reaction may influence the clinical progression and the visual prognosis of the primary chorioretinal disease.
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Coroiditis , Enfermedades de la Retina , Síndromes de Puntos Blancos , Femenino , Humanos , Adulto , Masculino , Agudeza Visual , Coroiditis/diagnóstico , Coroiditis/tratamiento farmacológico , Tomografía de Coherencia Óptica/métodos , Enfermedades de la Retina/patología , Epitelio Pigmentado de la Retina/patología , Estudios Retrospectivos , Atrofia/patología , Fibrosis , Angiografía con FluoresceínaRESUMEN
PURPOSE: To clarify the histologic basis of bacillary layer detachment (BALAD) through a review of the current literature and an analysis of retinal imaging. METHODS: The literature for previous reports of BALAD were reviewed. An analysis of retinal images was performed to support anatomical conclusions. RESULTS: A total of 164 unique patients with BALAD on optical coherence tomography (OCT) were identified from the published literature. Twenty-two underlying etiologies, all associated with subretinal exudation, were identified. Forty-one different OCT terminologies were found. The defining OCT feature of BALAD was a split at the level of the photoreceptor inner segment myoid creating a distinctive intraretinal cavity. Resolution of BALAD was followed by a rapid restoration of the ellipsoid zone. Histology of age-related macular degeneration eyes suggests that individual photoreceptors can shed inner segments. Furthermore, detachment of the entire layer of inner segments is a common postmortem artifact. It is proposed that BALAD occurs when outwardly directed forces promoting attachment of photoreceptor outer segments to the retinal pigment epithelium exceed the tensile strength of the photoreceptor inner segment myoid. CONCLUSION: This review serves to strengthen the OCT nomenclature "bacillary layer detachment," based on specific reflectance information obtained by OCT and previously published histologic observations.
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Células Fotorreceptoras Retinianas Conos/patología , Desprendimiento de Retina/diagnóstico , Células Fotorreceptoras Retinianas Bastones/patología , Terminología como Asunto , Tomografía de Coherencia Óptica/métodos , HumanosRESUMEN
PURPOSE: To detect the presence of MYD88 L265P mutation in the aqueous humor of patients with cytologically proven vitreoretinal lymphoma. METHODS: Eight consecutive patients with bilateral vitreoretinal lymphoma (16 eyes) were prospectively evaluated. Genomic DNA was extracted from aqueous samples after paracentesis and vitreous humor samples after diagnostic vitrectomy. MYD88 codon 265 mutation was investigated by both amplification-refractory mutation system polymerase chain reaction approach and pyrosequencing assay in the aqueous humor of all patients and in the vitreous of 6 patients. A control group of 8 age-matched patients with established diagnosis of noninfectious uveitis was also tested for the presence of MYD88 L265P mutation in the aqueous humor. RESULTS: Eight patients (three men, five women) with mean age of 69.5 years (range 50-85 years) were considered. All the patients tested for MYD88 L265P in the vitreous (six) were positive, and this result was consistent with cytological examination in all samples but one. The MYD88 L265P mutation was found in the aqueous of 6 patients (75%), and in 3 of them, the mutation was present in both eyes. Results of MYD88 L265P mutation in aqueous and vitreous sample were consistent in 7 of the 8 eyes with available samples. The aqueous humor of the noninfectious uveitis control group was negative for the detection of MYD88 L265P mutation. CONCLUSION: MYD88 mutation was detected in the aqueous humor of 75% of patients with cytologically proven vitreoretinal lymphoma. This technique may be considered as an additional diagnostic tool in the detection of the disease.
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Humor Acuoso/metabolismo , Biomarcadores de Tumor/genética , ADN de Neoplasias/genética , Linfoma Intraocular/genética , Mutación , Factor 88 de Diferenciación Mieloide/genética , Macroglobulinemia de Waldenström/genética , Anciano , Anciano de 80 o más Años , Análisis Mutacional de ADN , Femenino , Humanos , Linfoma Intraocular/diagnóstico , Linfoma Intraocular/cirugía , Masculino , Persona de Mediana Edad , Reacción en Cadena de la Polimerasa , Estudios Prospectivos , Microscopía con Lámpara de Hendidura , Vitrectomía , Cuerpo Vítreo/metabolismo , Cuerpo Vítreo/patología , Macroglobulinemia de Waldenström/diagnóstico , Macroglobulinemia de Waldenström/cirugíaRESUMEN
PURPOSE: To describe vitreal, retinal, and choroidal features of eyes affected by Endogenous candida endophthalmitis (ECE) analyzed by spectral domain optical coherence tomography (SD-OCT) and to evaluate their clinical impact. METHODS: Medical records and SD-OCT images from eyes diagnosed with ECE at four retina and uveitis tertiary referral centers were retrospectively evaluated. Spectral domain optical coherence tomography images were analyzed to evaluate the structural changes occurring in the vitreous, the retina, and the choroid in areas involved by ECE. Baseline and final best-corrected visual acuity were correlated with SD-OCT findings. RESULTS: Fifteen eyes from nine patients were enrolled. Vitreous involvement (vitreous cells, posterior hyaloid thickening) was detected in 13/15 eyes. Peculiar hyper-reflective preretinal aggregates obscuring the underlying retina because of a shadowing effect ("rain-cloud" sign) were noticed in all eyes with vitreous involvement. Two patterns of retinal and choroidal findings were identified: the first (six eyes) confined within the inner retinal layers (Intraretinal Pattern), the second (nine eyes) involving both retina (full thickness) and choriocapillaris (Chorioretinal Pattern). None of the eyes showed both patterns. None of the eyes, regardless the pattern, showed choroidal thickening. Baseline best-corrected visual acuity was not associated with OCT pattern (P = 0.09). On the contrary, final best-corrected visual acuity was significantly higher in patients showing Intraretinal Pattern (â¼20/25, 0.06 ± 0.08 LogMAR) than in subjects with Chorioretinal Pattern (â¼20/50, 0.44 ± 0.30 LogMAR) (P = 0.01). CONCLUSION: Endogenous candida endophthalmitis showed peculiar features on SD-OCT. Two distinct patterns of chorioretinal involvement influencing the final best-corrected visual acuity were identified. Spectral domain optical coherence tomography could be useful in the diagnosis, management, and outcome prediction in ECE.
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Candidiasis/patología , Endoftalmitis , Infecciones Fúngicas del Ojo/patología , Tomografía de Coherencia Óptica/métodos , Adulto , Anciano , Anciano de 80 o más Años , Coroides/patología , Endoftalmitis/diagnóstico por imagen , Endoftalmitis/microbiología , Endoftalmitis/patología , Infecciones Fúngicas del Ojo/diagnóstico por imagen , Infecciones Fúngicas del Ojo/microbiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Retina/patología , Estudios Retrospectivos , Agudeza Visual , Cuerpo Vítreo/patologíaRESUMEN
Ocular involvement is a common manifestation of inflammatory rheumatic diseases, often requiring a multidisciplinary collaboration between rheumatologists and ophthalmologists. The aim of this study was to standardize "red flags" for referral for rheumatologists and ophthalmologists using a Delphi consensus for the management of rheumatic diseases with ocular involvement. The scientific board comprised 11 Italian hospital-based rheumatologists (N = 6) and ophthalmologists (N = 5). A systematic review identified potential red flags for referral. The panel developed 19 statements consisting of (a) referral from ophthalmologist to rheumatologist (b) referral from rheumatologist to ophthalmologist and (c) overarching principles relating to multidisciplinary roles/goals and management. Voting was performed anonymously using an online Delphi method. Each participant expressed a level of agreement on each statement using a 5-point scale (1="strongly disagree"; 5="strongly agree"). Total cumulative agreement was defined as the sum of the percentage of response to items 4 ("agree") and 5 ("absolutely agree"), consensus defined as ≥ 80% cumulative agreement for each statement. Positive consensus among 11 participants was reached for 15/19 (78.9%) statements. Statements not reaching consensus were discussed in a face-to-face meeting prior to the second vote (10 participants). Positive consensus was reached for all 19 statements, with final total cumulative agreement of 90-100%. This is the first Delphi consensus undertaken to standardize red flags for referral to rheumatologists and ophthalmologists for patients with rheumatic diseases and ocular involvement.
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Consenso , Oftalmopatías/diagnóstico , Derivación y Consulta/normas , Enfermedades Reumáticas/diagnóstico , Reumatólogos/psicología , Manejo de Caso/normas , Técnica Delphi , Oftalmopatías/complicaciones , Humanos , Italia , Oftalmólogos , Selección de Paciente , Enfermedades Reumáticas/complicaciones , Reumatólogos/normasRESUMEN
Raynaud's phenomenon (RP) is a reversible vasospastic response of the extremities to cold or emotion, and can be the first manifestation or may be present before the development of an overt systemic sclerosis (SSc). The disturbance of the balance between vasodilation and vasoconstriction is not limited to the peripheral microcirculation of the skin, but it is also observed in other organs, such as the choroidal plexus of the eye. We aimed to examine the choroidal thickness (CT), the macular thickness and ganglion cell complex (GCC) average in thirty consecutive patients, without visual symptoms, classified as primary RP (pRP), RP secondary to suspected SSc, and overt SSc. All the patients underwent rheumatologic and ophthalmologic examination, capillaroscopy, test for anti-nuclear antibodies, anti-dsDNA, and anti-extractable nuclear antigens. Ophthalmologic examination included: best corrected visual acuity; slit lamp biomicroscopy; intraocular pressure measurements, fundus examination, and Spectral Domain-Optical Coherence Tomography (SD-OCT) with enhanced depth imaging scan system. Twenty-seven healthy subjects similar for gender and age were analyzed. In pRP, CT was significantly thinner than controls in the outer nasal and temporal regions. In secondary RP, the inner and outer nasal areas were significantly thinner than controls. In SSc, the central, inner inferior, inner nasal, inner superior, outer temporal, outer inferior, and outer nasal regions were significantly thinner than controls. A decreasing trend of central foveal thickness was noted. All the patients had GCC average significantly lower than controls. A thinning of choroidal and macular thickness, as well as of GCC was observed in patients with pRP and becomes more severe and extensive in RP secondary to suspected SSc and overt SSc. To our knowledge, this is the first study to analyze the choroidal features using SD-OCT in RP. These data may be clinically useful in suggesting an early involvement of ocular microcirculation with significant reduction of choroidal perfusion.
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Enfermedades de la Coroides/etiología , Coroides/patología , Mácula Lútea/patología , Enfermedad de Raynaud/etiología , Enfermedades de la Retina/etiología , Esclerodermia Sistémica/complicaciones , Biomarcadores/sangre , Estudios de Casos y Controles , Coroides/fisiopatología , Enfermedades de la Coroides/diagnóstico , Enfermedades de la Coroides/fisiopatología , Femenino , Humanos , Presión Intraocular , Mácula Lútea/fisiopatología , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Enfermedad de Raynaud/diagnóstico , Enfermedad de Raynaud/fisiopatología , Enfermedades de la Retina/diagnóstico , Enfermedades de la Retina/fisiopatología , Células Ganglionares de la Retina/patología , Esclerodermia Sistémica/diagnóstico , Esclerodermia Sistémica/fisiopatología , Índice de Severidad de la Enfermedad , Lámpara de Hendidura , Tomografía de Coherencia Óptica , Agudeza VisualAsunto(s)
Factor 88 de Diferenciación Mieloide , Neoplasias de la Retina , Humor Acuoso , Humanos , Mutación , Pacientes , Cuerpo VítreoAsunto(s)
Síndrome Antifosfolípido/complicaciones , Isquemia/diagnóstico por imagen , Oclusión de la Arteria Retiniana/diagnóstico por imagen , Oclusión de la Vena Retiniana/diagnóstico por imagen , Vasos Retinianos/diagnóstico por imagen , Anciano , Femenino , Angiografía con Fluoresceína , Humanos , Isquemia/etiología , Angiografía por Resonancia Magnética , Oclusión de la Arteria Retiniana/etiología , Vasculitis Retiniana/diagnóstico por imagen , Vasculitis Retiniana/etiología , Oclusión de la Vena Retiniana/etiología , Vasos Retinianos/patologíaRESUMEN
BACKGROUND: Syphilis has historically been referred to as "the great imitator", for the extent of disease manifestations secondary to infection. Ocular manifestations include a wide range of intra-ocular inflammation. METHODS: In this study, we report the case of a 52 years-old male patient with syphilitic hemorrhagic necrotizing retinitis. RESULTS: The patient presented to the emergency room for rapid and progressive vision loss and ocular redness lasting three weeks and was under immunosuppressive treatment. The diagnosis was syphilitic hemorrhagic necrotizing retinitis mimicking the typical clinical picture of retinitis caused by Cytomegalovirus infection in immunocompromised patients. CONCLUSIONS: The presented case highlights the need to consider ocular syphilis as a great masquerader even in the presence of atypical presentations such as hemorrhagic retinitis. Syphilis should be tested for treponemal and non-treponemal tests, and it should be ruled out as an etiological agent in every case of new-onset intra-ocular inflammation.