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American Journal of Ophthalmology Aug 2021To determine classification criteria for birdshot chorioretinitis.
PURPOSE
To determine classification criteria for birdshot chorioretinitis.
DESIGN
Machine learning of cases with birdshot chorioretinitis 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, 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 infectious posterior uveitides / panuveitides. The resulting criteria were evaluated on the validation set.
RESULTS
One thousand sixty-eight cases of posterior uveitides, including 207 cases of birdshot chorioretinitis, were evaluated by machine learning. Key criteria for birdshot chorioretinitis included a multifocal choroiditis with (1) the characteristic appearance of a bilateral multifocal choroiditis with cream-colored or yellow-orange, oval or round choroidal spots ("birdshot" spots); (2) absent to mild anterior chamber inflammation; and (3) absent to moderate vitreous inflammation; or multifocal choroiditis with positive HLA-A29 testing and either classic "birdshot spots" or characteristic imaging on indocyanine green angiography. 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 birdshot chorioretinitis were 10% in the training set and 0% in the validation set.
CONCLUSIONS
The criteria for birdshot chorioretinitis had a low misclassification rate and seemed to perform sufficiently well for use in clinical and translational research.
Topics: Birdshot Chorioretinopathy; Choroid; Consensus; Female; Fluorescein Angiography; Fundus Oculi; Humans; Machine Learning; Male; Middle Aged; Retina
PubMed: 33845003
DOI: 10.1016/j.ajo.2021.03.059 -
Orphanet Journal of Rare Diseases Feb 2021Behçet's disease (BD) is a systemic variable vessel vasculitis that involves the skin, mucosa, joints, eyes, arteries, veins, nervous system and gastrointestinal... (Review)
Review
Behçet's disease (BD) is a systemic variable vessel vasculitis that involves the skin, mucosa, joints, eyes, arteries, veins, nervous system and gastrointestinal system, presenting with remissions and exacerbations. It is a multifactorial disease, and several triggering factors including oral cavity infections and viruses may induce inflammatory attacks in genetically susceptible individuals. BD vasculitis involves different vessel types and sizes of the vascular tree with mixed-cellular perivascular infiltrates and is often complicated by recurrent thrombosis, particularly in the venous compartment. Several new therapeutic modalities with different mechanisms of action have been studied in patients with BD. A substantial amount of new data have been published on the management of BD, especially with biologics, over the last years. These important therapeutic advances in BD have led us to propose French recommendations for the management of Behçet's disease [Protocole National de Diagnostic et de Soins de la maladie de Behçet (PNDS)]. These recommendations are divided into two parts: (1) the diagnostic process and initial assessment; (2) the therapeutic management. Thirty key points summarize the essence of the recommendations. We highlighted the main differential diagnosis of BD according to the type of clinical involvement; the role of genetics is also discussed, and we indicate the clinical presentations that must lead to the search for a genetic cause.
Topics: Behcet Syndrome; Diagnosis, Differential; Genetic Predisposition to Disease; Humans; Vasculitis
PubMed: 33622338
DOI: 10.1186/s13023-020-01620-4 -
The Pan African Medical Journal 2017Our study aimed to determine the clinical, therapeutic and prognostic features of ocular involvement in patients with Behçet's disease treated in our Department of...
Our study aimed to determine the clinical, therapeutic and prognostic features of ocular involvement in patients with Behçet's disease treated in our Department of ophthalmology. We conducted a retrospective data collection from medical records of 20 patients treated at the military hospital in Laayoune. All patients underwent complete ophthalmological examination and fluorescein angiography if necessary. OCT exam was performed in two patients. Ten patients had anterior uveitis, complicated in one case by ocular hypertonia; two patients had intermediate uveitis; eight patients had posterior segment involvement complicated in one case by intravitreal hemorrhage. Behcet's Disease (BD) is an systemic idiopathic inflammatory disease currently classified within primary non-necrotizing vasculitis. Ocular involvement is common and severe in Behçet's disease, with the potential to compromise the visual prognosis. Behcet's disease is common in Morocco. It can compromise patient's visual prognosis making the collaboration between ophthalmologists and internists particularly important.
Topics: Adult; Behcet Syndrome; Eye Diseases; Female; Fluorescein Angiography; Hospitals, Military; Humans; Male; Morocco; Prognosis; Retrospective Studies; Severity of Illness Index; Uveitis, Anterior
PubMed: 28690751
DOI: 10.11604/pamj.2017.26.237.1175 -
Behçet's Disease, Pathogenesis, Clinical Features, and Treatment Approaches: A Comprehensive Review.Medicina (Kaunas, Lithuania) Mar 2024Behçet's disease is a systemic inflammatory disorder of unknown etiology. The disease manifests with diverse clinical symptoms, most commonly recurrent oral and genital... (Review)
Review
Behçet's disease is a systemic inflammatory disorder of unknown etiology. The disease manifests with diverse clinical symptoms, most commonly recurrent oral and genital ulcers, skin lesions, and uveitis, though it can affect multiple organ systems. Diagnosis is primarily clinical due to the lack of a definitive diagnostic test, and management involves a multidisciplinary approach to control inflammation and manage symptoms. Current treatment strategies involve corticosteroids, immunosuppressive agents, and, increasingly, biological therapies. Behçet's disease exhibits a higher prevalence along the Silk Road, suggesting a role of environmental and genetic factors. Despite significant progress in understanding its clinical characteristics and treatment approaches, gaps remain in our understanding of its pathogenesis. Future research is needed to elucidate the disease's pathophysiology and optimize treatment strategies.
Topics: Humans; Adrenal Cortex Hormones; Behcet Syndrome; Immunosuppressive Agents
PubMed: 38674208
DOI: 10.3390/medicina60040562 -
Indian Journal of Ophthalmology Dec 2022To evaluate efficacy of topical interferon alfa-2b (IFN) in the treatment of uveitic macular edema (UME).
PURPOSE
To evaluate efficacy of topical interferon alfa-2b (IFN) in the treatment of uveitic macular edema (UME).
METHODS
This is a prospective, interventional case study of patients with UME. Injection IFN was reconstituted into eye drops and a four times/day (QID) application was prescribed. Central macular thickness (CMT) on optical coherence tomography (OCT) scan was evaluated. Improvement in CMT by ≥50 μm from the baseline was studied in eyes with presenting CMT ≥400 μm.
RESULTS
Twenty eyes of 20 patients with UME were studied: anterior uveitis (n = 3), anterior + intermediate uveitis (n = 5), posterior uveitis (n = 3), retinal vasculitis (n = 3), and panuveitis (n = 6). Mean CMT at the presentation was 423.3 μm (range: 270-604 μm), which improved at 1 month (n = 16), 2 months (n = 10), and ≥3 months (n = 11) follow-up, to 415.3 μm (range: 247-579 μm) (P = 0.411), 364.4 μm (range: 258-566 μm) (P = 0.099), 344 μm (range: 258-484 μm) (P = 0.001), respectively. Twelve eyes of 12 patients had presenting CMT ≥400 μm. In these cases, decrease in CMT by ≥50 μm was seen in 4/10, 4/5, and 5/6 eyes at 1 and 2 months and ≥3 months follow-up. Mean follow-up was 4 months (range: 1-17 months). Complete resolution of UME was seen only in three eyes. No ocular or systemic side effects were observed.
CONCLUSION
Topical IFN therapy in QID doses is safe but may have limited role in UME. Long-term therapy may improve its efficacy. Larger studies with dose modification, combination with other drugs, and with homogeneous uveitis population are recommended.
Topics: Humans; Macular Edema; Prospective Studies; Uveitis; Eye; Uveitis, Anterior
PubMed: 36453344
DOI: 10.4103/ijo.IJO_1450_22 -
Ophthalmology Jun 2021To evaluate long-term efficacy and safety of extended treatment with adalimumab in patients with noninfectious intermediate, posterior, or panuveitis. (Randomized Controlled Trial)
Randomized Controlled Trial
PURPOSE
To evaluate long-term efficacy and safety of extended treatment with adalimumab in patients with noninfectious intermediate, posterior, or panuveitis.
DESIGN
Open-label, multicenter, phase 3 extension study (VISUAL III).
PARTICIPANTS
Adults who had completed a randomized, placebo-controlled phase 3 parent trial (VISUAL I or II) without treatment failure (inactive uveitis) or who discontinued the study after meeting treatment failure criteria (active uveitis).
METHODS
Patients received subcutaneous adalimumab 40 mg every other week. Data were collected for ≤ 362 weeks. Adverse events (AEs) were recorded until 70 days after the last dose.
MAIN OUTCOME MEASURES
Long-term safety and quiescence; other efficacy variables included inflammatory lesions, anterior chamber cell and vitreous haze grade, macular edema, visual acuity, and dose of uveitis-related systemic corticosteroids.
RESULTS
At study entry, 67% of patients (283/424) showed active uveitis and 33% (141/424) showed inactive uveitis; 60 patients subsequently met exclusion criteria, and 364 were included in the intention-to-treat analysis. Efficacy variables were analyzed through week 150, when approximately 50% of patients (214/424) remained in the study. Patients showing quiescence increased from 34% (122/364) at week 0 to 85% (153/180) at week 150. Corticosteroid-free quiescence was achieved by 54% (66/123) and 89% (51/57) of patients with active or inactive uveitis at study entry. Mean daily dose of systemic corticosteroids was reduced from 9.4 ± 17.1 mg/day at week 0 (n = 359) to 1.5 ± 3.9 mg/day at week 150 (n = 181). The percentage of patients who achieved other efficacy variables increased over time for those with active uveitis at study entry and was maintained for those with inactive uveitis. The most frequently reported treatment-emergent AEs of special interest were infections (n = 275; 79 events/100 patient-years [PY]); AEs and serious AEs occurred at a rate of 396 events/100 PY and 15 events/100 PY, respectively.
CONCLUSIONS
Long-term treatment with adalimumab led to quiescence and reduced corticosteroid use for patients who entered VISUAL III with active uveitis and led to maintenance of quiescence for those with inactive uveitis. AEs were comparable with those reported in the parent trials and consistent with the known safety profile of adalimumab.
Topics: Adalimumab; Adult; Aged; Aged, 80 and over; Anti-Inflammatory Agents; Dose-Response Relationship, Drug; Female; Follow-Up Studies; Humans; Injections, Subcutaneous; Male; Middle Aged; Panuveitis; Retrospective Studies; Time Factors; Treatment Outcome; Uveitis, Intermediate; Uveitis, Posterior; Visual Acuity; Young Adult
PubMed: 33157077
DOI: 10.1016/j.ophtha.2020.10.036 -
Clinical and Experimental Rheumatology 2020Ocular involvement in Behçet's syndrome still represents a challenge for both rheumatologists and ophthalmologists; over the past 20 years the availability of new... (Review)
Review
Ocular involvement in Behçet's syndrome still represents a challenge for both rheumatologists and ophthalmologists; over the past 20 years the availability of new diagnostic tools and the concomitant introduction of biologic drugs led to a significant improvement in the management of these patients. The lack of uniform definitions and the diversity of the outcome measures still represent an obstacle for the prompt and correct management of ocular manifestations. The aim of the present review is to summarise the current evidences related to correct diagnosis and proper management of patients with Behçet's syndrome and ocular involvement.
Topics: Behcet Syndrome; Humans
PubMed: 33253088
DOI: No ID Found -
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