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Ocular Manifestations in Juvenile Behçet's Disease: A Registry-Based Analysis from the AIDA Network.Ophthalmology and Therapy Jun 2024This study aims to characterize ocular manifestations of juvenile Behçet's disease (jBD).
INTRODUCTION
This study aims to characterize ocular manifestations of juvenile Behçet's disease (jBD).
METHODS
This was a registry-based observational prospective study. All subjects with jBD from the Autoinflammatory Diseases Alliance (AIDA) Network BD Registry showing ocular manifestations before 18 years were enrolled.
RESULTS
We included 27 of 1000 subjects enrolled in the registry (66.7% male patients, 45 affected eyes). The median (interquartile range [IQR]) age at ocular involvement was 14.2 (4.7) years. Uveitis affected 91.1% of eyes (anterior 11.1%, posterior 40.0%, panuveitis 40.0%), retinal vasculitis 37.8% and other manifestations 19.8%. Later onset (p = 0.01) and male predominance (p = 0.04) characterized posterior involvement. Ocular complications occurred in 51.1% of eyes. Patients with complications had earlier onset (p < 0.01), more relapses (p = 0.02) and more prolonged steroidal treatment (p = 0.02). The mean (standard deviation [SD]) central macular thickness (CMT) at the enrolment and last visit was 302.2 (58.4) and 293.3 (78.2) μm, respectively. Fluorescein angiography was pathological in 63.2% of procedures, with a mean (SD) Angiography Scoring for Uveitis Working Group (ASUWOG) of 17.9 (15.5). At the last visit, ocular damage according to the BD Overall Damage Index (BODI) was documented in 73.3% of eyes. The final mean (SD) best corrected visual acuity (BCVA) logMAR was 0.17 (0.47) and blindness (BCVA logMAR < 1.00 or central visual field ≤ 10°) occurred in 15.6% of eyes. At multivariate regression analysis, human leukocyte antigen (HLA)-B51 + independently predicted a + 0.35 change in the final BCVA logMAR (p = 0.01), while a higher BCVA logMAR at the first assessment (odds ratio [OR] 5.80; p = 0.02) independently predicted blindness.
CONCLUSIONS
The results of this study may be leveraged to guide clinical practice and future research on this rare sight-threatening condition.
PubMed: 38563868
DOI: 10.1007/s40123-024-00916-z -
Medicina (Kaunas, Lithuania) Mar 2024Inflammation plays a key role in the induction of choroidal neovascularization (CNV). Inflammatory choroidal neovascularization (iCNV) is a severe but uncommon... (Review)
Review
Inflammation plays a key role in the induction of choroidal neovascularization (CNV). Inflammatory choroidal neovascularization (iCNV) is a severe but uncommon complication of both infectious and non-infectious uveitides. It is hypothesized that its pathogenesis is similar to that of wet age-related macular degeneration (AMD), and involves hypoxia as well as the release of vascular endothelial growth factor, stromal cell-derived factor 1-alpha, and other mediators. Inflammatory CNV develops when inflammation or infection directly involves the retinal pigment epithelium (RPE)-Bruch's membrane complex. Inflammation itself can compromise perfusion, generating a gradient of retinal-choroidal hypoxia that additionally promotes the formation of choroidal neovascularization in the course of uveitis. The development of choroidal neovascularization may be a complication, especially in conditions such as punctate inner choroidopathy, multifocal choroiditis, serpiginous choroiditis, and presumed ocular histoplasmosis syndrome. Although the majority of iCNV cases are well defined and appear as the "classic" type (type 2 lesion) on fluorescein angiography, the diagnosis of iCNV is challenging due to difficulties in differentiating between inflammatory choroiditis lesions and choroidal neovascularization. Modern multimodal imaging, particularly the recently introduced technology of optical coherence tomography (OCT) and OCT angiography (noninvasive and rapid imaging modalities), can reveal additional features that aid the diagnosis of iCNV. However, more studies are needed to establish their role in the diagnosis and evaluation of iCNV activity.
Topics: Humans; Vascular Endothelial Growth Factor A; Choroiditis; Choroidal Neovascularization; Inflammation; Tomography, Optical Coherence; Hypoxia
PubMed: 38541191
DOI: 10.3390/medicina60030465 -
Ceska a Slovenska Oftalmologie :... 2024To draw attention to the higher proportion of Fuchs heterochromic iridocyclitis (FHI) cases in patients with multiple sclerosis (MS).
PURPOSE
To draw attention to the higher proportion of Fuchs heterochromic iridocyclitis (FHI) cases in patients with multiple sclerosis (MS).
MATERIALS AND METHODS
Retrospective study of data collected at the Center for the Diagnosis and Treatment of Uveitis.
RESULTS
An analysis of the medical records of 3016 patients with uveitis (in the years 2003-2020) was performed with a focus on MS. MS-associated uveitis was diagnosed in 90 patients (3%): anterior uveitis (n = 7), intermediate uveitis (n = 23), retinal vasculitis (n = 24), and panuveitis (n = 36). A clinical examination revealed signs of FHI in the anterior segment in 11 out of 90 cases (12%). Atypical manifestations of FHI included a higher incidence of bilateral involvement (45%), retinal vasculitis (27%), and vitreous snowballs (18%). The diagnosis of FHI preceded the diagnosis of MS in 4 cases. The median latency was 10.5 (range 8-15) years. In 4 patients, the diagnosis of demyelinating disease was established within one year of the diagnosis of FHI. We recommended a neurological examination for optic neuritis (n = 1), paresthesia (n = 3), relapse of motor deficit (n = 1), and screening of etiology in cases with involvement of the posterior segment (n = 3). In the other 3 cases, the diagnosis of MS preceded the diagnosis of FHI, with a median latency of 13 (range 8-19) years.
CONCLUSION
We detected clinical symptoms of FHI in 12% of uveitis cases associated with MS, more often in bilateral manifestations of intraocular inflammation. Based on our experience, we recommend an investigation of the medical history of patients with FHI for manifestations of sensitive, sensory and motor deficits, especially in bilateral cases.
Topics: Humans; Iridocyclitis; Retrospective Studies; Multiple Sclerosis; Retinal Vasculitis; Uveitis
PubMed: 38538292
DOI: 10.31348/2024/17 -
CMAJ : Canadian Medical Association... Mar 2024
Topics: Humans; Scleritis; Pamidronate; Uveitis, Anterior; Acute Disease
PubMed: 38527748
DOI: 10.1503/cmaj.230859-f -
Medicine Mar 2024Pathergy test indicates nonspecific hyper-reactivity of the skin to aseptic trauma in Behçet syndrome (BS) and is considered as an adjunctive diagnostic test with a...
Pathergy test indicates nonspecific hyper-reactivity of the skin to aseptic trauma in Behçet syndrome (BS) and is considered as an adjunctive diagnostic test with a good specificity albeit with low sensitivity. We tested the hypothesis that a relationship exists between active clinical manifestations of BS and the pathergy-positivity when performed simultaneously. Pathergy test and detailed dermatologic examination were done in 105 BS patients (60M/45F); who were seen consecutively at the multi-disciplinary BS outpatient clinic in a single tertiary center. Information regarding demographic and clinical characteristics, pathergy test results at diagnosis, and details about treatment were obtained from patient charts. Disease activity was assessed using Behçet Disease Current Activity Form. Among 105 patients, 27 (25.7%) were pathergy-positive at the time of the study visit whereas 40.9% were pathergy-positive at the time of the diagnosis. There was no relation between pathergy test and patient age or disease duration, either. Pathergy-positivity was significantly more common in patients with folliculitis compared to those without folliculitis (40.7% vs 19.2%; P = .026). The test was also positive in all 3 patients with leg ulcers due to venous stasis. We found that among all skin-mucosa lesions only the presence of folliculitis was associated with pathergy positivity with statistical significance. It was also remarkable that the current pathergy was positive in all 3 patients with active leg ulcers but this finding warrants further studies because of the low patient numbers.
Topics: Humans; Behcet Syndrome; Skin; Skin Tests; Folliculitis; Leg Ulcer
PubMed: 38518008
DOI: 10.1097/MD.0000000000037553 -
Arthritis Research & Therapy Mar 2024Autoimmune responses have been suggested to involvement in patients with Behcet's syndrome (BS). There has been growing attention towards the roles of cutaneous...
BACKGROUND
Autoimmune responses have been suggested to involvement in patients with Behcet's syndrome (BS). There has been growing attention towards the roles of cutaneous lymphocyte antigen (CLA) regular T cells (Tregs) in autoimmune diseases. The role of CLA Tregs in BS is still uncertain. This study aims to clarify the impact of CLA Tregs on BS.
METHODS
We collected peripheral blood from a total of 107 patients with BS and 114 healthy controls (HCs). The number of CLA Tregs, natural killer (NK) cells, B cells, and several subtypes of CD4 T cells were detected using flow cytometry and compared between patients and HCs.
RESULTS
The absolute number and proportion of CLA Tregs among CD4 T lymphocytes and CD4 Tregs were lower in patients with BS than in HCs. CLA Tregs were positively related with NK cells (r = 0.500, P < 0.001) and B cells (r = 0.470, P < 0.001) and negatively related with effector T cells (r=-0.402, P < 0.001) in patients with BS. Patients with BS and arterial aneurysms had CLA Treg cell deficiency. A decreased proportion of CLA Tregs was associated with arterial aneurysms in patients with BS. The proportion of CLA Tregs in patients with BS increased with corticosteroids and immunosuppressants.
CONCLUSION
CLA Tregs decrease in association with arterial aneurysm in patients with BS. CLA Tregs may be a predictor of response to BS treatment.
Topics: Humans; Behcet Syndrome; Clinical Relevance; Oligosaccharides; T-Lymphocytes, Regulatory; Aneurysm; Sialyl Lewis X Antigen
PubMed: 38515127
DOI: 10.1186/s13075-024-03306-9 -
Ophthalmology and Therapy May 2024This study aimed to assess the efficacy and safety of adalimumab in pediatric patients with chronic non-infectious posterior uveitis and panuveitis (not associated with...
INTRODUCTION
This study aimed to assess the efficacy and safety of adalimumab in pediatric patients with chronic non-infectious posterior uveitis and panuveitis (not associated with juvenile idiopathic arthritis).
METHODS
The medical records of children (< 18 years old) with chronic non-infectious posterior uveitis and panuveitis were collected and analyzed in this retrospective cohort study. Children were allocated to a conventional adalimumab-free treatment (CT) or adalimumab (ADA) group based on whether they additionally received adalimumab.
RESULTS
In total, 69 children (138 eyes) were included, with 21 (42 eyes) and 48 (96 eyes) in the CT and ADA groups, respectively. During the average follow-up period of 24 months, the improvement in all ocular parameters (best-corrected visual acuity, intraocular inflammation, fluorescein angiography score) was better in the ADA group than in the CT group, except for changes in central macular thickness, which did not significantly differ between the groups. The mean time of first alleviation, which was after 1.03 ± 0.12 months of therapy, was earlier in the ADA group than in the CT group (2.30 ± 0.46 months). In the ADA group, 90.6% of children had remission within 3 months, and 47.9% had no relapse during follow-up. Cough and cold were the most common adverse events in the ADA group; however, the number of adverse events was similar between both the groups.
CONCLUSIONS
Adalimumab was effective in the treatment of chronic noninfectious posterior uveitis and panuveitis in pediatric patients, and disease inactivity was accomplished in the majority of the patients, thereby improving visual outcomes and maintaining disease stability. Adverse events were limited and tolerable.
PubMed: 38498278
DOI: 10.1007/s40123-024-00884-4 -
European Review For Medical and... Mar 2024Behçet's disease etiology is uncertain, and no specific diagnostic markers exist in the laboratory. This retrospective study aimed to evaluate the role of inflammatory...
OBJECTIVE
Behçet's disease etiology is uncertain, and no specific diagnostic markers exist in the laboratory. This retrospective study aimed to evaluate the role of inflammatory and hematological parameters, mainly Pan-Immune-Inflammation-Value (PIV), in predicting vascular Behçet's disease (VBD).
PATIENTS AND METHODS
A total of 85 patients with VBD and 92 patients without vascular involvement (non-VBD) were included in this study. Neutrophil, monocyte, platelet, and lymphocyte subsets are all included in the PIV, a new blood-based biomarker.
RESULTS
The optimal cut-off values for the PIV were determined to be ≥261.6. White blood cell, neutrophil, monocyte, hemoglobin, mean corpuscular hemoglobin, mean corpuscular hemoglobin concentration (MCHC), red cell distribution, platelet, plateletcrit, PIV, neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, sedimentation, c-reactive protein (CRP) values were significantly associated with VBD in univariate analysis. After multivariate analysis, PIV [odds ratio (OR): 2.758; 95% confidence interval (CI): 1.327-5.736; p=0.007] and CRP (OR: 4.029; 95% CI: 1.924-8.438; p<0.001) were found to be a positive predictor for VBD, while MCHC (OR: 0.722; 95% CI: 0.530-0.983; p=0.039) was seen as a negative predictor.
CONCLUSIONS
Based on our results, PIV, an easily accessible, cost-effective, and new composite biomarker, has a significant predictive value in VBD.
Topics: Humans; Behcet Syndrome; Retrospective Studies; Inflammation; C-Reactive Protein; Biomarkers
PubMed: 38497857
DOI: 10.26355/eurrev_202403_35588 -
Journal of Translational Medicine Mar 2024Patients with spondyloarthritis (SpA)/HLA-B27-associated acute anterior uveitis (AAU) experience recurring acute flares, which pose significant visual and financial...
Investigation of the acute pathogenesis of spondyloarthritis/HLA-B27-associated anterior uveitis based on genome-wide association analysis and single-cell transcriptomics.
BACKGROUND
Patients with spondyloarthritis (SpA)/HLA-B27-associated acute anterior uveitis (AAU) experience recurring acute flares, which pose significant visual and financial challenges. Despite established links between SpA and HLA-B27-associated AAU, the exact mechanism involved remains unclear, and further understanding is needed for effective prevention and treatment.
METHODS
To investigate the acute pathogenesis of SpA/HLA-B27-associated AAU, Mendelian randomization (MR) and single-cell transcriptomic analyses were employed. The MR incorporated publicly available protein quantitative trait locus data from previous studies, along with genome-wide association study data from public databases. Causal relationships between plasma proteins and anterior uveitis were assessed using two-sample MR. Additionally, colocalization analysis was performed using Bayesian colocalization. Single-cell transcriptome analysis utilized the anterior uveitis dataset from the Gene Expression Omnibus (GEO) database. Dimensionality reduction, clustering, transcription factor analysis, pseudotime analysis, and cell communication analysis were subsequently conducted to explore the underlying mechanisms involved.
RESULTS
Mendelian randomization analysis revealed that circulating levels of AIF1 and VARS were significantly associated with a reduced risk of developing SpA/HLA-B27-associated AAU, with AIF1 showing a robust correlation with anterior uveitis onset. Colocalization analysis supported these findings. Single-cell transcriptome analysis showed predominant AIF1 expression in myeloid cells, which was notably lower in the HLA-B27-positive group. Pseudotime analysis revealed dendritic cell terminal positions in differentiation branches, accompanied by gradual decreases in AIF1 expression. Based on cell communication analysis, CD141CLEC9A classic dendritic cells (cDCs) and the APP pathway play crucial roles in cellular communication in the Spa/HLA-B27 group.
CONCLUSIONS
AIF1 is essential for the pathogenesis of SpA/HLA-B27-associated AAU. Myeloid cell differentiation into DCs and decreased AIF1 levels are also pivotal in this process.
Topics: Humans; Acute Disease; Bayes Theorem; Gene Expression Profiling; Genome-Wide Association Study; HLA-B27 Antigen; Spondylarthritis; Uveitis, Anterior
PubMed: 38475831
DOI: 10.1186/s12967-024-05077-y -
Eye (London, England) Jun 2024To evaluate the utility of ultrawide-field fluorescein angiography (UWFFA) in patients with anterior uveitis by investigating the detection of retinal vascular leakage...
PURPOSE
To evaluate the utility of ultrawide-field fluorescein angiography (UWFFA) in patients with anterior uveitis by investigating the detection of retinal vascular leakage (RVL) and the subsequent implications on disease diagnosis and management.
STUDY DESIGN/MATERIALS AND METHODS
Patients, who were referred to the National Eye Institute (NEI) for evaluation of anterior uveitis and underwent UWFFA imaging at the initial visit, were included in this study. The electronic medical records of eligible patients were reviewed. The UWFFA images were assessed for severity of retinal vascular leakage, presence of macular leakage, and optic disc leakage by a two-grader system, and intergrader agreement was calculated using the κ-value. Patients with altered diagnoses and management attributable to UWFFA results were noted.
RESULTS
A total of 93 eyes of 63 patients were included in the study. Of 93 eyes, 31 (33.3%) eyes had RVL on UWFFA, with 26 (28.0%) eyes and 5 (5.4%) eyes showing mild and moderate-severe RVL, respectively. Twenty-five (26.9%) eyes showed macular leakage, and 7 (7.5%) eyes showed optic disc leakage. The κ-values ranged from 0.85 - 0.87 indicating excellent intergrader agreement. Of the 31 eyes with RVL, the diagnosis was changed to anterior/intermediate uveitis for 9 (29.0%) eyes and to panuveitis for 4 (12.9%) eyes. Systemic treatment was escalated in 5 patients.
CONCLUSION
Our results suggest that UWFFA imaging is useful in detecting subclinical posterior involvement in patients with anterior uveitis. Moreover, UWFFA results in altered diagnosis and treatment approaches in a portion of patients.
Topics: Humans; Fluorescein Angiography; Female; Male; Middle Aged; Adult; Uveitis, Anterior; Retrospective Studies; Aged; Retinal Vessels; Young Adult; Capillary Permeability; Adolescent; Retinal Diseases
PubMed: 38472380
DOI: 10.1038/s41433-024-03012-5