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Orphanet Journal of Rare Diseases Jan 2013Neuro-Behçet's disease (NBD) involves the central nervous system; peripheral nervous system involvement is not often reported. NBD is quite common in adult patients and... (Review)
Review
Neuro-Behçet's disease (NBD) involves the central nervous system; peripheral nervous system involvement is not often reported. NBD is quite common in adult patients and occurs rarely during childhood and adolescence. Young patients may share symptoms and signs of NBD with other neuro-ophthalmological disorders (e.g. idiopathic intracranial hypertension); thus, making the differential diagnosis difficult. Neuroimaging is mandatory and necessary for a correct NBD diagnosis but in children radiological examinations are often difficult to perform without sedation. From 1971 to 2011, 130 patients aged ≤16 years have been reported with NBD, according to retrospective surveys, case series, and case reports. The origin of the reported cases met the well-known geographical distribution of Behçet's disease (BD); the mean age at presentation of neurological findings was 11.8 years, with male gender prevalence (ratio, 2.9:1). We considered in detail the neuro-ophthalmological features of the 53 cases whose neuroimaging alterations were described with an assigned radiological pattern of the disease (parenchymal: 14 cases, non-parechymal: 35 cases, and mixed: 4 cases). In 19/53 patients (36%), neuro-ophthalmological symptoms anticipated any pathognomonic sign for a BD diagnosis, or only occasional aphtae were recalled by the patients. Family history was positive in 17% of subjects. Headache was reported in 75% of the patients; in those presenting with cerebral vascular involvement, headache was combined to other symptoms of intracranial hypertension. Papilledema was the most frequently reported ophthalmological finding, followed by posterior uveitis. Treatment consisted of systemic steroids in 93% of patients, often combined with other immunosuppressive drugs (especially colchicine and azathioprine). Clinical recovery or improvement was documented in the large majority of patients. Nine subjects had definitive alterations, and one died. Based on our review and personal experience, a delayed diagnosis, and the consequently delayed immunosuppressive treatment, may favour permanent sequelae, in particular, optic atrophy.
Topics: Adolescent; Behcet Syndrome; Child; Diagnosis, Differential; Eye Diseases; Female; Humans; Immunosuppressive Agents; Male; Nervous System Diseases
PubMed: 23360593
DOI: 10.1186/1750-1172-8-18 -
JAMA Ophthalmology Jun 2017Adalimumab was recently approved for the treatment of noninfectious intermediate uveitis, posterior uveitis, and panuveitis. (Randomized Controlled Trial)
Randomized Controlled Trial
IMPORTANCE
Adalimumab was recently approved for the treatment of noninfectious intermediate uveitis, posterior uveitis, and panuveitis.
OBJECTIVE
To assess the effect of adalimumab on the visual functioning and quality of life in patients with corticosteroid-dependent noninfectious intermediate uveitis, posterior uveitis, and panuveitis.
DESIGN
A post hoc analysis of clinical trials of adults with active (VISUAL-1) and inactive (VISUAL-2) noninfectious intermediate uveitis, posterior uveitis, and panuveitis was conducted in the United States, Canada, Europe, Israel, Australia, Latin America, and Japan. A total of 217 patients (110 adalimumab, 107 placebo) in VISUAL-1 and 226 patients (115 adalimumab, 111 placebo) in VISUAL-2 were studied using intent-to-treat analyses. The clinical trials were conducted between August 10, 2010, and May 14, 2015.
INTERVENTIONS
In VISUAL-1 and VISUAL-2, patients were randomized to receive adalimumab, 80-mg, subcutaneous loading dose followed by 40 mg every other week or placebo for 80 weeks. All patients underwent prednisone tapering, with patients in VISUAL-1 receiving an initial prednisone burst.
MAIN OUTCOMES AND MEASURES
The 25-item National Eye Institute Vision Function Questionnaire (NEI VFQ-25) composite score questionnaire assessed the impact of visual impairment from the patient's perspective; scores on the questionnaire range from 0 to 100, with higher scores indicating better vision-related quality of life. The change in NEI VFQ-25 from best state achieved prior to week 6 (VISUAL-1) and from baseline state (VISUAL-2) to the final or early termination visit was determined in each group and statistically compared using analysis of variance. The temporal effects of adalimumab and placebo on NEI VFQ-25 were investigated using a longitudinal model.
RESULTS
Of the 217 patients in VISUAL-1, 124 (57.1%) were women; the mean (SD) age was 42.7 (14.9) years. Of the 226 patients in VISUAL-2, 138 (61.1%) were women; the mean (SD) age was 42.5 (13.4). In VISUAL-1, the change from final score to best score in NEI VFQ-25 was -1.30 for adalimumab and -5.50 for placebo-a difference of 4.20 (95% CI, 1.04 to 7.36; P = .01) associated with adalimumab compared with placebo. In VISUAL-2, the change from baseline NEI VFQ-25 was 3.36 for adalimumab and 1.24 for placebo-a difference of 2.12 (95% CI, -0.81 to 5.04; P = .16). In both trials, the longitudinal models showed a significant difference in NEI VFQ-25 between adalimumab and placebo of 3.07 (95% CI, 2.09 to 4.06; P < .001) and 4.66 (95% CI, 0.05 to 9.26; P = .048) in the VISUAL-1 (74.15 vs 71.08) and VISUAL-2 (82.39 vs 77.73) trials, respectively.
CONCLUSIONS AND RELEVANCE
This post hoc analysis suggests that adalimumab is associated with statistically significant and clinically meaningful improvements in patient-reported visual functioning for patients with noninfectious intermediate uveitis, posterior uveitis, and panuveitis.
TRIAL REGISTRATION
clinicaltrials.gov Identifiers: NCT01138657 (VISUAL-1) and NCT01124838 (VISUAL-2).
Topics: Adalimumab; Adult; Anti-Inflammatory Agents; Dose-Response Relationship, Drug; Double-Blind Method; Female; Follow-Up Studies; Health Status; Humans; Injections, Subcutaneous; Male; Middle Aged; Panuveitis; Retrospective Studies; Treatment Outcome; Uveitis, Intermediate; Uveitis, Posterior; Visual Acuity
PubMed: 28426849
DOI: 10.1001/jamaophthalmol.2017.0603 -
The Pan African Medical Journal 2015
Topics: Adult; Choroid; Choroiditis; Female; Fluorescein Angiography; Humans; Macula Lutea
PubMed: 26327987
DOI: 10.11604/pamj.2015.21.150.7188 -
The British Journal of Ophthalmology Mar 1993A 29-year-old woman suffered from headaches, diarrhoea, and high grade fever followed by a unilateral retinal vasculitis, papillitis, and chorioretinitis. Abnormal...
A 29-year-old woman suffered from headaches, diarrhoea, and high grade fever followed by a unilateral retinal vasculitis, papillitis, and chorioretinitis. Abnormal electrocardiographic findings and antibody titre dynamics strongly suggested a coxsackievirus B3 infection. With respect to prior observations on coxsackievirus B group associated uveitis this viral infection may be considered in patients with well defined extraocular manifestations and uveitis.
Topics: Adult; Coxsackievirus Infections; Enterovirus B, Human; Eye Infections, Viral; Female; Humans; Panuveitis
PubMed: 8384474
DOI: 10.1136/bjo.77.3.182 -
BioMed Research International 2015Adamantiades-Behçet's disease is a multisystemic vasculitis with multiorgan involvement. Ocular disorders occur often in this syndrome typically in the form of a... (Review)
Review
Adamantiades-Behçet's disease is a multisystemic vasculitis with multiorgan involvement. Ocular disorders occur often in this syndrome typically in the form of a relapsing-remitting panuveitis and vasculitis and can lead to blindness as one of its most disabling complications if left untreated. There are known risk factors related with the worst visual prognosis, which require early and intensive treatment in order to obtain a rapid suppression of inflammation and to prevent future relapses. The management strategy to avoid vision loss and blindness currently involves the use of local and systemic drugs including steroids and immunosuppressive and biologic agents. This review aims to demonstrate how the introduction and the use of biologic agents improves the visual outcome of patients with Adamantiades-Behçet's disease.
Topics: Behcet Syndrome; Biological Factors; Blindness; Humans; Immunosuppressive Agents; Prognosis; Visual Acuity
PubMed: 26558256
DOI: 10.1155/2015/120519 -
Acta Ophthalmologica Jun 2013Relapsing ocular involvement is one of the major manifestations in Behçet disease (BD). When ocular BD is left untreated, it often results in blindness in <5 years.... (Review)
Review
Relapsing ocular involvement is one of the major manifestations in Behçet disease (BD). When ocular BD is left untreated, it often results in blindness in <5 years. Combining systemic corticosteroids with cyclosporine A and other conventional immune modulating agents has been the only choice for treatment. More recently, the introduction of 'biologics' seems to be an effective therapy in patients affected with BD, especially in those with ocular disease. Our purpose was to survey the current knowledge regarding the biological therapeutic approaches for Behçet uveitis. We focused on the most commonly used biological agents: 'tumor necrosis factor-alpha antagonists' and 'interferon alpha'. We attempted to compare the effectiveness of these two biologicals and tried to balance on the pros and cons of these agents in clinical practice. The impressive efficacy of both approaches in sight-threatening BD argues for their expanding role. Earlier introduction of these agents in the course of disease may prevent irreversible structural damage and may result in improved long-term prognosis.
Topics: Behcet Syndrome; Diagnosis, Differential; Humans; Immunosuppressive Agents; Uveitis
PubMed: 22520021
DOI: 10.1111/j.1755-3768.2011.02348.x -
Annals of the Academy of Medicine,... Jun 2021
Topics: Humans; Panuveitis; Retinal Detachment; Typhoid Fever; Visual Acuity
PubMed: 34195760
DOI: 10.47102/annals-acadmedsg.2020595 -
American Journal of Ophthalmology Oct 2020This study evaluated the risk and risk factors for exudative retinal detachment (ERD) in ocular inflammatory diseases.
PURPOSE
This study evaluated the risk and risk factors for exudative retinal detachment (ERD) in ocular inflammatory diseases.
DESIGN
Retrospective cohort study.
METHODS
Patients with noninfectious ocular inflammation had been followed longitudinally between 1978 and 2007 at 4 US subspecialty uveitis centers. The main outcome measurements were occurrences of ERD and predictive factors.
RESULTS
A total of 176 of 14,612 eyes with ocular inflammation presented with ERD. Among uveitis cases, Vogt-Koyanagi-Harada syndrome (VKH) (odds ratio [OR] = 109), undifferentiated choroiditis (OR = 9.18), sympathetic ophthalmia (OR = 8.43), primary or secondary panuveitis (OR = 7.09), multifocal choroiditis with panuveitis (OR = 4.51), and "other" forms of posterior uveitis (OR = 16.9) were associated with a higher prevalence of ERD. Among the 9,209 uveitic or scleritic eyes initially free of ERD and followed, 137 incident ERD cases were observed over 28,949 eye-years at risk (incidence rate = 0.47% [0.40%-0.56%/eye-year]). VKH (HR = 13.2), sympathetic ophthalmia (HR = 5.82), undifferentiated choroiditis (HR = 6.03), primary or secondary panuveitis (HR = 4.21), and rheumatoid arthritis (HR = 3.30) were significantly associated with incident ERD. A significant dose-response relationship with the prevalence and incidence of ERD were observed for AC cells and vitreous cell activity. African Americans had significantly higher prevalence and incidence of ERD.
CONCLUSIONS
Other ocular inflammatory conditions in addition to VKH syndrome and posterior scleritis were associated with increased risk of ERD, indicating that ERD does not necessarily dictate a diagnosis of VKH or posterior scleritis. In addition, the relationship between ERD and inflammatory severity factors implies that inflammation is a key predictive factor associated with developing ERD and requires early and vigorous control.
Topics: Adult; Choroiditis; Exudates and Transudates; Female; Fluorescein Angiography; Humans; Incidence; Inflammation; Male; Middle Aged; Ophthalmia, Sympathetic; Prevalence; Retinal Detachment; Retrospective Studies; Risk Assessment; Risk Factors; Uveitis, Posterior; Uveomeningoencephalitic Syndrome; Visual Acuity
PubMed: 32621891
DOI: 10.1016/j.ajo.2020.06.019 -
Orphanet Journal of Rare Diseases May 2016Birdshot chorioretinopathy (BCR) is a rare form of chronic, bilateral, posterior uveitis with a distinctive clinical phenotype, and a strong association with HLA-A29. It... (Review)
Review
Birdshot chorioretinopathy (BCR) is a rare form of chronic, bilateral, posterior uveitis with a distinctive clinical phenotype, and a strong association with HLA-A29. It predominantly affects people in middle age. Given its rarity, patients often encounter delays in diagnosis leading to delays in adequate treatment, and thus risking significant visual loss. Recent advances have helped increase our understanding of the underlying autoimmune mechanisms involved in disease pathogenesis, and new diagnostic approaches such as multimodality imaging have improved our ability to both diagnose and monitor disease activity. Whilst traditional immunosuppressants may be effective in BCR, increased understanding of immune pathways is enabling development of newer treatment modalities, offering the potential for targeted modulation of immune mediators. In this review, we will discuss current understanding of BCR and explore recent developments in diagnosis, monitoring and treatment of this disease. Synonyms for BCR: Birdshot chorioretinopathy, Birdshot retinochoroiditis, Birdshot retino-choroidopathy, Vitiliginous choroiditis. Orphanet number: ORPHA179 OMIM: 605808.
Topics: Birdshot Chorioretinopathy; Chorioretinitis; HLA-A Antigens; Humans; Immunomodulation; Th17 Cells
PubMed: 27175923
DOI: 10.1186/s13023-016-0429-8 -
Ophthalmology May 2024Sympathetic ophthalmia (SO) is a sight-threatening granulomatous panuveitis caused by a sensitizing event. Primary enucleation or primary evisceration, versus primary... (Meta-Analysis)
Meta-Analysis Review
TOPIC
Sympathetic ophthalmia (SO) is a sight-threatening granulomatous panuveitis caused by a sensitizing event. Primary enucleation or primary evisceration, versus primary repair, as a risk management strategy after open-globe injury (OGI) remains controversial.
CLINICAL RELEVANCE
This systematic review was conducted to report the incidence of SO after primary repair compared with that of after primary enucleation or primary evisceration. This enabled the reporting of an estimated number needed to treat.
METHODS
Five journal databases were searched. This review was registered with International Prospective Register of Systematic Reviews (identifier, CRD42021262616). Searches were carried out on June 29, 2021, and were updated on December 10, 2022. Prospective or retrospective studies that reported outcomes (including SO or lack of SO) in a patient population who underwent either primary repair and primary enucleation or primary evisceration were included. A systematic review and meta-analysis were carried out in accordance with Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Random effects modelling was used to estimate pooled SO rates and absolute risk reduction (ARR).
RESULTS
Eight studies reporting SO as an outcome were included in total. The included studies contained 7500 patients and 7635 OGIs. In total, 7620 OGIs met the criteria for inclusion in this analysis; SO developed in 21 patients with OGI. When all included studies were pooled, the estimated SO rate was 0.12% (95% confidence interval [CI], 0.00%-0.25%) after OGI. Of 779 patients who underwent primary enucleation or primary evisceration, no SO cases were reported, resulting in a pooled SO estimate of 0.05% (95% CI, 0.00%-0.21%). For primary repair, the pooled estimate of SO rate was 0.15% (95% CI, 0.00%-0.33%). The ARR using a random effects model was -0.0010 (in favour of eye removal; 95% CI, -0.0031 [in favor of eye removal] to 0.0011 [in favor of primary repair]). Grading of Recommendations, Assessment, Development, and Evaluations analysis highlighted a low certainty of evidence because the included studies were observational, and a risk of bias resulted from missing data.
DISCUSSION
Based on the available data, no evidence exists that primary enucleation or primary evisceration reduce the risk of secondary SO.
FINANCIAL DISCLOSURE(S)
Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
Topics: Humans; Ophthalmia, Sympathetic; Retrospective Studies; Eye Enucleation; Eye Injuries; Eye Evisceration
PubMed: 38086434
DOI: 10.1016/j.ophtha.2023.12.006