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Neurology India 2022Behçet's disease (BD) is a multisystem vasculitis, which can involve the central nervous system, the gastrointestinal tract, the blood vessels, the joints, and the...
BACKGROUND
Behçet's disease (BD) is a multisystem vasculitis, which can involve the central nervous system, the gastrointestinal tract, the blood vessels, the joints, and the lungs. Neurological involvement is about 5%, but the headache is common in these patients.
OBJECTIVE
This study aimed to investigate whether differences exist in the onset and types of primary headaches between patients with BD without neurological involvement (BWoNI) and patients with neurological involvement, that is, neuro-Behçet's disease (NBD).
MATERIALS AND METHODS
A total of 136 consecutive BD patients (98 BWoNI and 38 NBD) were evaluated and compared.
RESULTS
Primary headaches beginning before and after the onset of BD were found in 27.6 and 48.1% of BWoNI patients, respectively, and in 10.5 and 34.2% of NBD patients, respectively. The start of primary headaches was more frequent after then before the onset of BD in each group and overall. The primary headache types were similar in both BWoNI and NBD patients.
CONCLUSIONS
The primary headache starts more frequently after then before the onset of BD, and characteristics of primary headaches were similar in both BWoNI and NBD patients.
Topics: Behcet Syndrome; Headache; Humans
PubMed: 35263891
DOI: 10.4103/0028-3886.338704 -
American Journal of Ophthalmology Aug 2021The purpose of this study was to determine classification criteria for punctate inner choroiditis (PIC).
PURPOSE
The purpose of this study was to determine classification criteria for punctate inner choroiditis (PIC).
DESIGN
Machine learning of cases with PIC and 8 other posterior uveitides.
METHODS
Cases of posterior uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on diagnosis by using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used in the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the posterior uveitides. The resulting criteria were evaluated in the validation set.
RESULTS
A total of 1,068 cases of posterior uveitides, including 144 cases of PIC, were evaluated by machine learning. Key criteria for PIC included: 1) "punctate"-appearing choroidal spots <250 µm in diameter; 2) absent to minimal anterior chamber and vitreous inflammation; and 3) involvement of the posterior pole with or without mid-periphery. Overall accuracy for posterior uveitides was 93.9% in the training set and 98.0% (95% confidence interval: 94.3-99.3) in the validation set. The misclassification rates for PIC were 15% in the training set and 9% in the validation set.
CONCLUSIONS
The criteria for PIC had a reasonably low misclassification rate and appeared to perform sufficiently well for use in clinical and translational research.
Topics: Adult; Choroid; Choroiditis; Female; Fluorescein Angiography; Fundus Oculi; Humans; Machine Learning; Male; Visual Acuity
PubMed: 33845011
DOI: 10.1016/j.ajo.2021.03.046 -
Clinical Rheumatology Jun 2022Behçet's syndrome (BS) is a multi-systemic vasculitis characterized by recurrent oral ulcers, genital ulcers, ocular lesions, and other systemic manifestations. As...
BACKGROUND
Behçet's syndrome (BS) is a multi-systemic vasculitis characterized by recurrent oral ulcers, genital ulcers, ocular lesions, and other systemic manifestations. As there is no laboratory diagnostics of BS, the diagnosis is mainly clinical.
OBJECTIVE
To investigate the utility of the autoantibody against tubulin-α-1c in diagnosis of BS and its clinical significance.
METHODS
Sixty BS patients and sixty healthy controls were enrolled in this study. We assessed all patients by Behçet disease current activity form (BDCAF), routine laboratory investigations, and immunological markers (ANA, anti-DNA, ANCA). Anti-endothelial cell antibodies (AECA) and anti-tubulin-alpha-1c antibodies were performed for all participants.
RESULTS
Regarding duration of illness, Birmingham Vasculitis Activity Score (BVAS), and BDCAF, the mean value was 4.77 ± 4.239, 19.80 ± 10.020, and 9.52 ± 5.476, respectively. On comparing laboratory investigations, there was only significant increase in anti-tubulin-alpha-1c antibody in BS patients compared to healthy controls. Regarding AECA, there was no any significant correlation except with CRP. Anti-tubulin-alpha-1c detected significant direct correlation with the presence of posterior uveitis, panuveitis, and venous thrombosis as well as BVAS, C4, and protein/creatinine ratio. Regarding diagnostic performance of both AECA and anti-tubulin-alpha-1c, the cutoff value of AECA for diagnosis was 27.250, with sensitivity and specificity of 93.3% and 96.7%, respectively. The cutoff value of the anti-tubulin-alpha-1c for diagnosis was 22.300, with sensitivity and specificity of 100% and 96.7% respectively.
CONCLUSION
Anti-tubulin-α-1c antibodies are of diagnostic value in BS and are indicative of activity with 100% sensitivity and 96.7% specificity. Key Points • There is lack of specific laboratory, radiological, or histological diagnostics for Behcet syndrome. • We aimed to evaluate the significance of tubulin-α-1c autoantibody in diagnosis of Behcet syndrome. • There is elevation of tubulin-α-1c autoantibody with sensitivity and specificity of 100% and 96.7%, respectively.
Topics: Antibodies, Antinuclear; Behcet Syndrome; Biomarkers; Humans; Sensitivity and Specificity; Tubulin
PubMed: 35128589
DOI: 10.1007/s10067-021-06025-7 -
Scientific Reports Aug 2022Clinical discrimination of posterior uveitis entities remains a challenge. This exploratory, cross-sectional study investigated the green (GEFC) and red emission...
Clinical discrimination of posterior uveitis entities remains a challenge. This exploratory, cross-sectional study investigated the green (GEFC) and red emission fluorescent components (REFC) of retinal and choroidal lesions in posterior uveitis to facilitate discrimination of the different entities. Eyes were imaged by color fundus photography, spectrally resolved fundus autofluorescence (Color-FAF) and optical coherence tomography. Retinal/choroidal lesions' intensities of GEFC (500-560 nm) and REFC (560-700 nm) were determined, and intensity-normalized Color-FAF images were compared for birdshot chorioretinopathy, ocular sarcoidosis, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), and punctate inner choroidopathy (PIC). Multivariable regression analyses were performed to reveal possible confounders. 76 eyes of 45 patients were included with a total of 845 lesions. Mean GEFC/REFC ratios were 0.82 ± 0.10, 0.92 ± 0.11, 0.86 ± 0.10, and 1.09 ± 0.19 for birdshot chorioretinopathy, sarcoidosis, APMPPE, and PIC lesions, respectively, and were significantly different in repeated measures ANOVA (p < 0.0001). Non-pigmented retinal/choroidal lesions, macular neovascularizations, and fundus areas of choroidal thinning featured predominantly GEFC, and pigmented retinal lesions predominantly REFC. Color-FAF imaging revealed involvement of both, short- and long-wavelength emission fluorophores in posterior uveitis. The GEFC/REFC ratio of retinal and choroidal lesions was significantly different between distinct subgroups. Hence, this novel imaging biomarker could aid diagnosis and differentiation of posterior uveitis entities.
Topics: Birdshot Chorioretinopathy; Coloring Agents; Cross-Sectional Studies; Fluorescein Angiography; Humans; Optical Imaging; Sarcoidosis; Tomography, Optical Coherence; Uveitis, Posterior
PubMed: 36038591
DOI: 10.1038/s41598-022-18048-4 -
American Journal of Ophthalmology Aug 2021The purpose of this study was to determine classification criteria for spondyloarthritis/HLA-B27-associated anterior uveitis DESIGN: Machine learning of cases with... (Comparative Study)
Comparative Study
PURPOSE
The purpose of this study was to determine classification criteria for spondyloarthritis/HLA-B27-associated anterior uveitis DESIGN: Machine learning of cases with spondyloarthritis/HLA-B27-associated anterior uveitis and 8 other anterior uveitides.
METHODS
Cases of anterior 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 in the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the anterior uveitides. The resulting criteria were evaluated in the validation set.
RESULTS
A total of 1,083 cases of anterior uveitides, including 184 cases of spondyloarthritis/HLA-B27-associated anterior uveitis, were evaluated by machine learning. The overall accuracy for anterior uveitides was 97.5% in the training set and 96.7% in the validation set (95% CI: 92.4-98.6). Key criteria for spondyloarthritis/HLA-B27-associated anterior uveitis included 1) acute or recurrent acute unilateral or unilateral alternating anterior uveitis with either spondyloarthritis or a positive test result for HLA-B27; or 2) chronic anterior uveitis with a history of the classic course and either spondyloarthritis or HLA-B27; or 3) anterior uveitis with both spondyloarthritis and HLA-B27. The misclassification rates for spondyloarthritis/HLA-B27-associated anterior uveitis were 0% in the training set and 3.6% in the validation set.
CONCLUSIONS
The criteria for spondyloarthritis/HLA-B27-associated anterior uveitis had a low misclassification rate and appeared to perform well enough for use in clinical and translational research.
Topics: Adolescent; Adult; Female; HLA-B27 Antigen; Humans; Machine Learning; Male; Middle Aged; Spondylarthritis; Translational Research, Biomedical; Uveitis, Anterior; Young Adult
PubMed: 33845004
DOI: 10.1016/j.ajo.2021.03.049 -
Genes Mar 2023Recent studies have suggested an association between iritis or uveitis and glaucoma. This study investigated the causal relationship between glaucoma and iritis and... (Meta-Analysis)
Meta-Analysis
Recent studies have suggested an association between iritis or uveitis and glaucoma. This study investigated the causal relationship between glaucoma and iritis and uveitis as exposures in a multi-ethnic population. Single-nucleotide polymorphisms associated with exposures to iritis and uveitis from the genome-wide association study (GWAS) data of Biobank Japan (BBJ) and the meta-analysis data from BBJ and UK Biobank (UKB) were used as instrumental variables (IVs). The GWAS dataset for glaucoma was extracted from the meta-analysis data ( = 240,302) of Genetic Epidemiology Research in Adult Health and Aging and UKB. The casual estimates were assessed with a two-sample Mendelian randomisation (MR) test using the inverse-variance-weighted (IVW) method, weighted median method, MR-Egger method, and MR-Pleiotropy Residual Sum and Outlier test. The IVW method revealed a significant causal association between iritis and glaucoma using IVs ( < 5.0 × 10-8) from the East Asian population ( = 2) (odds ratio [OR] = 1.01, = 0.017), a significant association between iritis exposures ( < 5.0 × 10-8) in the multi-ethnic population ( = 11) (OR = 1.04, = 0.001), and a significant causal association between uveitis exposures ( = 10 with < 5.0 × 10-8) and glaucoma in the multi-ethnic population (OR = 1.04, = 0.001). Iritis and uveitis had causal effects on glaucoma risk based on IVs from the multi-ethnic population. These findings imply that the current classifications of uveitic glaucoma and open-angle glaucoma overlap, indicating the need for further investigating these complex relationships.
Topics: Adult; Humans; Iritis; Genome-Wide Association Study; Glaucoma, Open-Angle; Mendelian Randomization Analysis; Causality
PubMed: 36980914
DOI: 10.3390/genes14030642 -
Journal of Investigative Medicine High... 2023Neuroretinitis, originally described by Leber at the turn of the 20th century, has long perplexed ophthalmologists due to its multiple recognized causes and often...
Neuroretinitis, originally described by Leber at the turn of the 20th century, has long perplexed ophthalmologists due to its multiple recognized causes and often atypical presentation. Optic disk edema and macular star in the affected eye are well-agreed upon findings and are due to increased permeability of blood vessels near the optic disk and in the retina at large. It also is universally painless and presents with a relative afferent pupillary defect (RAPD) in the affected eye or eyes. However, depending on the infectious agent, an underlying autoimmune condition, or undefined idiopathic cause, there can be various degrees of vision loss, visual field loss, progression or recurrence, as well as involvement of the other eye. We present this case of presumed sequential idiopathic neuroretinitis with severe vision and visual field loss with a low-positive anti-MOG test in the border county of El Paso.
Topics: Humans; Retinitis; Optic Disk; Chorioretinitis; Diagnosis, Differential
PubMed: 37942586
DOI: 10.1177/23247096231206619 -
American Journal of Ophthalmology Aug 2021To determine classification criteria for cytomegalovirus (CMV) anterior uveitis.
PURPOSE
To determine classification criteria for cytomegalovirus (CMV) anterior uveitis.
DESIGN
Machine learning of cases with CMV anterior uveitis and 8 other anterior uveitides.
METHODS
Cases of anterior 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 anterior uveitides. The resulting criteria were evaluated on the validation set.
RESULTS
One thousand eighty-three cases of anterior uveitides, including 89 cases of CMV anterior uveitis, were evaluated by machine learning. The overall accuracy for anterior uveitides was 97.5% in the training set and 96.7% in the validation set (95% confidence interval 92.4, 98.6). Key criteria for CMV anterior uveitis included unilateral anterior uveitis with a positive aqueous humor polymerase chain reaction assay for CMV. No clinical features reliably diagnosed CMV anterior uveitis. The misclassification rates for CMV anterior uveitis were 1.3% in the training set and 0% in the validation set.
CONCLUSIONS
The criteria for CMV anterior uveitis had a low misclassification rate and seemed to perform sufficiently well for use in clinical and translational research.
Topics: Adolescent; Adult; Aged; Aqueous Humor; Consensus; Cytomegalovirus; Cytomegalovirus Infections; DNA, Viral; Eye Infections, Viral; Female; Humans; Machine Learning; Male; Middle Aged; Uveitis, Anterior; Young Adult
PubMed: 33845019
DOI: 10.1016/j.ajo.2021.03.060 -
Clinics in Dermatology 2023Adamantiades-Behçet disease is an inflammatory, vascular disease of unknown etiology. The disease is named after two physicians, Benediktos Adamantiades and Hulȗsi...
Adamantiades-Behçet disease is an inflammatory, vascular disease of unknown etiology. The disease is named after two physicians, Benediktos Adamantiades and Hulȗsi Behçet, who both made significant contributions to the study of the disease. It was probably first described by Hippocrates in 500 BCE. Adamantiades-Behçet disease is most common in the region encompassing the ancient trade route known as the Silk Road. In Turkey, the disease is estimated to affect 80 to 370 people per 100,000 inhabitants, and it is also the country with the highest incidence rate. The frequency of the disease associated with the clinical picture differs from the origin of the onset. The disease is characterized by recurrent aphthous ulcers of the mouth, genitals, skin lesions, and eye lesions. The disease process can also involve other organs, including the joints, nervous system, large vessels, heart, and gastrointestinal tract. Aphthous oral ulcers appear as the first harbinger of the disease and affect almost all patients (97%-99%). The scientific interest in Adamantiades-Behçet disease has increased exponentially in the past decade.
Topics: Humans; Behcet Syndrome; Ophthalmology; Dermatology; Stomatitis, Aphthous
PubMed: 37572969
DOI: 10.1016/j.clindermatol.2023.08.001 -
Pediatric Rheumatology Online Journal Oct 2023Pediatric uveitis is a severe inflammatory ocular condition that can lead to sight-threatening complications and can negatively impact quality of life. The retinal...
BACKGROUND
Pediatric uveitis is a severe inflammatory ocular condition that can lead to sight-threatening complications and can negatively impact quality of life. The retinal microcirculation is often affected in intermediate uveitis and panuveitis. Here, we examined the extraocular (i.e., systemic) microcirculation in pediatric uveitis cases and healthy controls using nailfold capillaroscopy (NFC).
METHODS
We performed NFC in 119 children with noninfectious uveitis and 25 healthy pediatric controls, and assessed the following parameters: capillary density (number of capillaries/mm), dilated capillaries (apex > 20 µm), avascular area, the presence of microhemorrhages, and capillary morphology. Differences in NFC parameters between cases and controls were calculated using regression analysis after adjusting for age and sex.
RESULTS
The mean (± SD) age of the patient group was 13.7 (± 3) years, with 56% females; 46%, 18%, and 36% of cases presented as anterior uveitis, intermediate uveitis, and panuveitis, respectively, with an overall mean disease duration of 4.7 (± 4.0) years. Compared to the control group, the pediatric uveitis cases had a significantly higher number of dilated capillaries/mm and a higher prevalence of ramified capillaries. Moreover, compared to the control group the intermediate uveitis cases had a significantly higher number of dilated capillaries, whereas the anterior uveitis cases had a lower capillary density and a higher prevalence of ramified capillaries.
CONCLUSIONS
Children with uveitis without systemic disease can present with changes in systemic microcirculation. These changes vary amongst the subtypes of uveitis.
Topics: Female; Humans; Child; Adolescent; Male; Microcirculation; Quality of Life; Nails; Uveitis; Uveitis, Anterior; Uveitis, Intermediate; Panuveitis; Microscopic Angioscopy
PubMed: 37784087
DOI: 10.1186/s12969-023-00896-7