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Klinische Monatsblatter Fur... Apr 2021The aim of this study was to report on the disease course and management modalities in patients with juvenile idiopathic arthritis-related uveitis (JIA uveitis) or...
PURPOSE
The aim of this study was to report on the disease course and management modalities in patients with juvenile idiopathic arthritis-related uveitis (JIA uveitis) or intermediate uveitis of the pars planitis type (PP) who were followed up using precise ocular investigational techniques.
METHODS
This is a retrospective single-centre study. All charts of patients seen in our centre over 15 years (2005 - 2019) with a diagnosis of JIA uveitis or PP were retrieved and analysed for clinical course, severity of inflammation, type of management, and the role played by precise investigational techniques (laser flare photometry [LFP], optical coherence tomography [OCT] and fluorescein angiography [FA]) in therapeutic decisions.
RESULTS
26 out of a total of 64 patients with sufficient data could be included in the study. Mean age was 11.2 years (SD ± 5.4). 13/26 (50%) patients presented with JIA uveitis and 13/26 (50%) with PP. PP patients had a more benign course (mean LFP values at presentation 9.9 ± 3.2 ph/ms), and at the end of follow-up, only 2/13 PP patients (15%) still needed systemic treatment. In contrast, JIA uveitis as a whole was more severe, although benign forms were present (LFP values at presentation 105.9 ± 19.5 ph/ms), and at the end of follow-up, 5/13 patients (38%) still needed systemic treatment. Complications were also more severe and frequent in JIA uveitis patients. In 6/26 patients (24%, 3 JIA and 3 PP patients), the precise monitoring methods allowed unjustified systemic treatment to be discontinued, and to avoid such a treatment, it was recommended that it should be replaced by topical treatment or observation.
CONCLUSION
Nowadays, new investigational techniques have made precise follow-up of uveitis possible. We determined the precise inflammatory pattern of JIA uveitis and PP, which is crucial information to determine the therapeutic intervention. As these two entities are common in young and paediatric patients, such precise monitoring is essential to determine adequate treatment paradigms and avoid unnecessary systemic treatment, especially corticosteroids. When the ophthalmic status requires it, multidisciplinary collaboration between the ophthalmologist, the paediatrician and the rheumatologist may be needed to offer optimal management to the patient. In cases of purely ocular involvement, it is the ophthalmologist who should determine the management.
Topics: Arthritis, Juvenile; Child; Humans; Pars Planitis; Retrospective Studies; Uveitis; Uveitis, Intermediate
PubMed: 33607692
DOI: 10.1055/a-1354-6452 -
Ocular Immunology and Inflammation Jul 2020To describe the finding of inferior corneal haze secondary to presumed inflammatory endothelio-10 pathy in a series of patients with pars planitis.
PURPOSE
To describe the finding of inferior corneal haze secondary to presumed inflammatory endothelio-10 pathy in a series of patients with pars planitis.
METHODS
Single-center retrospective observational consecutive case-series.
RESULTS
Seven patients with an established diagnosis of pars planitis are described in this case series including four females and three males. The ages ranged from 5 to 31 years at presentation. Pars planitis was bilateral in six patients and unilateral in one patient. Fundus examinations revealed vitreous opacities and pars plana exudates in all seven patients, cystoid macular edema in four patients, and peripheral retinal vasculitis in two patients. Corneal examination revealed opacification of the posterior cornea in an inferior location in 10 of the 13 eyes with pars planitis. Only one of these eyes had keratic precipitates. For a given patient, corneal involvement was more commonly seen in the more inflamed eye. In one patient with active inflammation, microcystic corneal edema was noted to predate the formation of inferior corneal endothelial opacification, suggesting that physical proximity to the site of inflammation at the inferior pars plana is the cause of this notable physical finding.
CONCLUSIONS
Inferior posterior corneal haze related to inflammatory endotheliopathy may occur in eyes with pars planitis. As patients with pars planitis may be otherwise asymptomatic, this corneal finding shouldprompt a detailed funduscopic examination to rule out this form of uveitis.
Topics: Adolescent; Adult; Child; Child, Preschool; Corneal Opacity; Endothelium, Corneal; Female; Humans; Inflammation; Male; Pars Planitis; Retrospective Studies; Young Adult
PubMed: 31573365
DOI: 10.1080/09273948.2019.1629603 -
Ocular Immunology and Inflammation Dec 2023To describe the long-term prevalence of ocular complications and visual prognosis in patients with pediatric uveitis.
PURPOSE
To describe the long-term prevalence of ocular complications and visual prognosis in patients with pediatric uveitis.
METHODS
Demographics, etiology and location of uveitis, type of complications, treatment and visual outcomes were recorded in 296 children at first examination and at 1-, 2-, 3-, 5- and 10-year time points.
RESULTS
Αnterior uveitis represented 53.4% of cases, followed by intermediate (28.0%), posterior uveitis (11.1%) and panuveitis (7.4%). The leading diagnoses were idiopathic uveitis (31.1%), juvenile idiopathic arthritis (27.0%) and pars planitis (22.6%). Posterior synechiae was the most frequent complication of anterior uveitis and panuveitis, cystoid macular edema and disc edema of intermediate and posterior uveitis respectively. Posterior uveitis and panuveitis had more severe final vision loss (23.1% and 20% respectively).
CONCLUSIONS
This study provides clinical characteristics and main complications in a longitudinal long-term follow-up of a large non-infectious pediatric uveitis Greek population. Early diagnosis and close monitoring remain of fundamental importance.
Topics: Child; Humans; Retrospective Studies; Uveitis; Uveitis, Posterior; Panuveitis; Vision Disorders
PubMed: 36693268
DOI: 10.1080/09273948.2022.2162422 -
American Journal of Ophthalmology Aug 2021To determine classification criteria for intermediate uveitis, non-pars planitis type (IU-NPP, also known as undifferentiated intermediate uveitis).
PURPOSE
To determine classification criteria for intermediate uveitis, non-pars planitis type (IU-NPP, also known as undifferentiated intermediate uveitis).
DESIGN
Machine learning of cases with IU-NPP and 4 other intermediate uveitides.
METHODS
Cases of intermediate uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated on the validation set.
RESULTS
Five hundred eighty-nine of cases of intermediate uveitides, including 114 cases of IU-NPP, were evaluated by machine learning. The overall accuracy for intermediate uveitides was 99.8% in the training set and 99.3% in the validation set (95% confidence interval 96.1, 99.9). Key criteria for IU-NPP included unilateral or bilateral intermediate uveitis with neither snowballs in the vitreous humor nor snowbanks on the pars plana. Other key exclusions included multiple sclerosis, sarcoidosis, and syphilis. The misclassification rates for IU-NPP were 0% in the training set and 0% in the validation set.
CONCLUSIONS
The criteria for IU-NPP had a low misclassification rate and seemed to perform well enough for use in clinical and translational research.
Topics: Adult; Female; Humans; Machine Learning; Male; Middle Aged; Pars Planitis; Uveitis, Intermediate; Visual Acuity; Young Adult
PubMed: 33839089
DOI: 10.1016/j.ajo.2021.03.054 -
Ophthalmology and Therapy Aug 2023Scientific evidence of the effectiveness of the tumor necrosis factor inhibitor adalimumab (ADA) in pediatric patients with non-infectious non-anterior uveitis is still...
INTRODUCTION
Scientific evidence of the effectiveness of the tumor necrosis factor inhibitor adalimumab (ADA) in pediatric patients with non-infectious non-anterior uveitis is still limited. The aim of this study is to investigate the therapeutic role of ADA in a cohort of pediatric patients with non-anterior uveitis.
METHODS
This is an international multicenter study analyzing real-life data referred to pediatric patients treated with ADA for intermediate uveitis/pars planitis, posterior uveitis and panuveitis. Data were drawn from the AutoInflammatory Disease Alliance (AIDA) registry for patients with uveitis.
RESULTS
Twenty-one patients (36 affected eyes) were enrolled, and all patients benefited from ADA administration. In detail, 11 patients (19 affected eyes) did not experience further ocular inflammation after ADA introduction; 10 cases (17 affected eyes) showed a significant clinical improvement consisting of a decrease in severity and/or frequency of ocular relapses. The number of ocular flares dropped from 3.91 to 1.1 events/patient/year after ADA introduction (p = 0.0009); macular edema and retinal vasculitis were respectively observed in 18 eyes and 20 eyes at the start of ADA and in 4 eyes and 2 eyes at the last assessment. The mean daily glucocorticoid dosage significantly decreased from 26.8 ± 16.8 mg/day at the start of ADA to 6.25 ± 6.35 mg/day at the last assessment (p = 0.002). Intermediate uveitis/pars planitis (p = 0.01) and posterior uveitis (p = 0.03) were more frequently observed in patients with full response to ADA; panuveitis (p = 0.001) was significantly more frequent among patients continuing to experience uveitic flares. This could be related to a higher use of systemic glucocorticoids (p = 0.002) and conventional immunosuppressants (p = 0.007) at the start of ADA when treating intermediate uveitis/pars planitis. Regarding the safety profile, only one adverse event was reported during ADA treatment, consisting of the development of generalized adenopathy.
CONCLUSIONS
ADA proved to have an effective therapeutic role in all pediatric patients with non-anterior uveitis enrolled in the study. An overall glucocorticoid-sparing effect was observed despite the severity of cases enrolled. A more aggressive treatment of panuveitis and posterior uveitis at start of ADA could increase the likelihood of full response to therapy.
PubMed: 37166627
DOI: 10.1007/s40123-023-00712-1 -
International Ophthalmology May 2021To evaluate spectral domain optical coherence tomography (SD-OCT) findings of 42 eyes with pars planitis and to identify risk factors affecting visual acuity.
PURPOSE
To evaluate spectral domain optical coherence tomography (SD-OCT) findings of 42 eyes with pars planitis and to identify risk factors affecting visual acuity.
METHODS
Medical records and SD-OCT findings were retrospectively reviewed.
RESULTS
Mean best-corrected visual acuity (BCVA) was 0.248 ± 0.3 on the logMAR scale at baseline. SD-OCT findings included epiretinal membrane (ERM) in 16 (38.1%) eyes, loss of normal foveal contour appearance in 8 (19.0%), ellipsoid zone (EZ) damage in 5 (11.9%), external limiting membrane (ELM) damage in 3 (7.1%), disruption of retinal pigment epithelium (RPE) in 2 (4.8%), and macular atrophy in 1 (2.4%). There was macular edema in 10 (23.8%) eyes [cystoid macular edema (CME) in 8 (19.0%), diffuse macular edema (DME) in 6 (14.3%), and serous retinal detachment in 2 (4.8%)]. The mean central macular thickness (CMT) was 272.1 ± 319.5 μm. Patients were followed up for a mean of 50.6 ± 36.7 months. Mean BCVA was 0.210 ± 0.3 at the final evaluation. SD-OCT findings included ERM in 28 (66.7%) eyes, EZ damage in 6 (14.3%), ELM damage in 3 (7.1%), disruption of RPE in 4 (9.5%), loss of normal foveal contour appearance in 12 (28.6%), and macular atrophy in 2 (4.8%). There was CME and/or DME in 6 (14.3%) eyes. The mean CMT was 238 ± 220.9 μm and was significantly lower than the baseline (p < 0.001). According to multivariate linear regression analysis, the presence of DME, and loss of normal foveal contour appearance at baseline were the independent factors associated with BCVA at the final examination (B = 0.726, p < 0.001; B = 0.766, p < 0.001, respectively).
CONCLUSIONS
DME and loss of normal foveal contour appearance were more likely to have adverse effects on visual acuity.
Topics: Humans; Pars Planitis; Retrospective Studies; Risk Factors; Tomography, Optical Coherence; Visual Acuity
PubMed: 33559832
DOI: 10.1007/s10792-021-01734-z -
Current Eye Research Jul 2024To evaluate the microvasculature during remission in patients with pars planitis (PP-IU) and multiple sclerosis-associated intermediate uveitis (MS-IU) using optical...
PURPOSE
To evaluate the microvasculature during remission in patients with pars planitis (PP-IU) and multiple sclerosis-associated intermediate uveitis (MS-IU) using optical coherence tomography angiography (OCT-A).
METHODS
Single-center, descriptive, case-control study was conducted. Adult patients (≥16 years) with IU in remission (PP-IU and MS-IU) and healthy age-sex matched healthy controls (HC) were enrolled to the study. Demographic/clinical features, best-corrected visual acuity (BCVA), ocular findings, neurological symptoms and preferred treatments were recorded. The presence of cystoid macular edema (CME) during follow-up was recorded. All IU patients in remission and HC subjects were scanned with OCT-A. Foveal avascular zone (FAZ) areas of superficial and deep capillary plexus (SCP/DCP) and vascular densities of SCP, DCP and choriocapillaris were obtained from OCT-A and compared between the groups.
RESULTS
Sixty-nine eyes of 37 IU patients in remission and 20 HC were included (44 eyes/23 patients in PP-IU, 25/14 in MS-IU, 40/20 in HC). No statistically significant differences were observed in terms of demographic or clinical characteristics of the patients. The vascular density in the SCP was significantly reduced in the PP-IU and MS-IU groups compared to the HC group ( < .05). Nevertheless, there were no significant changes in any of the OCTA parameters between the IU groups. Uveitis duration was found to be correlated with enlargement of the FAZ area in PP-IU ( = .039).
CONCLUSION
OCTA may not be useful in differentiating between PP-IU and MS-IU. Nevertheless, the primary implication in SCP potentially elucidates the pathogenesis of these two subtypes of IU, which are characterized by a shared pathogenesis. The monitoring of the FAZ area in the PP-IU group is valuable in terms of chronicity.
Topics: Humans; Tomography, Optical Coherence; Male; Female; Adult; Uveitis, Intermediate; Fluorescein Angiography; Visual Acuity; Multiple Sclerosis; Retinal Vessels; Pars Planitis; Young Adult; Middle Aged; Follow-Up Studies; Case-Control Studies; Fundus Oculi; Adolescent
PubMed: 38433455
DOI: 10.1080/02713683.2024.2323520 -
Arthritis Care & Research Nov 2020Chronic uveitis is a common manifestation of pediatric rheumatologic conditions and may result in irreversible blindness and long-term disability. While chronic anterior...
OBJECTIVE
Chronic uveitis is a common manifestation of pediatric rheumatologic conditions and may result in irreversible blindness and long-term disability. While chronic anterior uveitis is the most commonly encountered ocular manifestation of rheumatic disease, little is known about the clinical presentation, management, and long-term outcome of more complex eye conditions such as pars planitis (PP), panuveitis (PU), and Vogt-Koyanagi-Harada disease (VKH). The present study was undertaken to comprehensively assess the long-term safety and efficacy of disease-modifying antirheumatic drugs (DMARDs) and biologics for the treatment of pediatric and adolescent patients with PP, PU, and VKH.
METHODS
We retrospectively reviewed a cohort of 75 children and adolescents with idiopathic PP (n = 50), PU (n = 12), and VKH (n = 14) followed by the Pediatric Rheumatology Core at Children's Hospital Los Angeles and evaluated referral patterns, clinical presentation, treatment response, and long-term clinical outcome.
RESULTS
Patients were followed for an average of 52 months. Their mean age at disease onset was 10 years. Bilateral eye involvement was seen in 87% of the patients. At first presentation to an ophthalmologist, glaucoma was noted in 21% of patients and vision loss (<20/40) in 87% of patients, while legal blindness (≤20/200 in the better-seeing eye) was diagnosed in 18 of 75 (24%) of patients (PP 22%, PU 36%, and VKH 21%). The average referral time to a pediatric rheumatologist was 13 months (range 1-96 months). Topical steroids were used in all patients, but 98% of patients required additional DMARDs, and 73% required therapy with biologics. After a mean of 52 months, 35% of patients across all disease groups had significant vision loss or were blind, and only 28% were in clinical remission without medications. The worst outcome was observed in children with PU. Regression analysis, young age at onset, delayed referral to a pediatric rheumatologist, and chronic disease were strong predictors for the risk of long-term blindness.
CONCLUSION
PP, PU, and VKH involve a high risk of permanent vision loss and should be managed by a skilled rheumatologist as early and as aggressively as possible.
Topics: Adolescent; Antirheumatic Agents; Biological Products; Child; Female; Humans; Male; Panuveitis; Pars Planitis; Regression Analysis; Retrospective Studies; Uveomeningoencephalitic Syndrome; Vision Disorders
PubMed: 31444859
DOI: 10.1002/acr.24056 -
Journal of Ophthalmic Inflammation and... Jan 2020In previous studies, authors use ultrasound biomicroscopy (UBM) to analyze the characteristics of cyclitic membranes and the associated complications in patients with...
BACKGROUND
In previous studies, authors use ultrasound biomicroscopy (UBM) to analyze the characteristics of cyclitic membranes and the associated complications in patients with pars planitis. However, there are no reports regarding the prevalence of cyclitic membranes or complications at diagnosis and during follow-up.
PURPOSE
To describe the characteristics and complications of cyclitic membranes, as determined by UBM in patients with pars planitis using AVISO-S™ (Quantel Medical) equipment with a 50-MHz linear probe with a focus at the pars plana.
DESIGN
This retrospective study reviewed UBM images of patients diagnosed with pars planitis, from the Inflammatory Eye Disease Clinic in Mexico City from January 2010 to June 2016.
RESULTS
Cyclitic membranes were observed in the first UBM image in 67 eyes (56.7%) and during follow-up in 81 eyes (68.62%). In 67 eyes (82.71%), the cyclitic membranes extended through one or two quadrants. Extension toward the posterior lens capsule was recognized in 15 eyes (18.52%) and extension toward the peripheral retina in 12 eyes (14.81%). Complications included ciliary body detachments in 10 eyes (12.35%) and peripheral retinal traction in 8 eyes (9.88%).
CONCLUSIONS
UBM is a valuable tool for the diagnosis of cyclitic membranes at admittance and during follow-up of patients with pars planitis; it helps the clinician to detect this complication early.
PubMed: 31997032
DOI: 10.1186/s12348-020-0194-7 -
International Ophthalmology Oct 2023To analyse the clinical characteristics of adult patients with pars planitis (PP-IU), non-pars planitis (NPP-IU) and multiple sclerosis-associated intermediate uveitis...
PURPOSE
To analyse the clinical characteristics of adult patients with pars planitis (PP-IU), non-pars planitis (NPP-IU) and multiple sclerosis-associated intermediate uveitis (MS-IU) and distinguish between groups.
METHODS
Seventy-three adult patients with intermediate uveitis (IU) reviewed retrospectively and divided as PP-IU, NPP-IU and MS-IU according to 'The standardization of uveitis nomenclature working group classification criteria.' Demographic and clinical characteristics, OCT and fluorescein angiography (FA) findings, complications and treatments were recorded.
RESULTS
A total of 134 eyes of 73 patients were included, and 42 of the patients were classified as PP-IU, 12 as NPP-IU, and 19 as MS-IU. If a patient presenting with blurred vision, or tent-shaped vitreous band/snowballs/snowbank on examination, or vascular leakage on FA and accompanying neurological symptoms, the frequency of demyelinating plaque detection on cranial MRI and the risk of MS-IU increased. Mean BCVA was increased from 0.22 ± 0.30 logMAR to 0.19 ± 0.31 logMAR (p = 0.021). Gender, initial BCVA, snowbank formation, disc oedema and periphlebitis on examination, and disc leakage/occlusion on FA were found predictive of decreased BCVA at final visit (p < 0.05).
CONCLUSIONS
The clinical features of these three groups are similar, some features that can guide the differential diagnosis. It may be recommended to periodically evaluate "suspicious" patients with MRI for MS.
Topics: Humans; Adult; Retrospective Studies; Uveitis, Intermediate; Uveitis; Pars Planitis; Vision Disorders
PubMed: 37395903
DOI: 10.1007/s10792-023-02778-z