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Journal Francais D'ophtalmologie Jan 2018Anterior Uveitis is the most common form of uveitis. There are several known and many possible etiologies for anterior uveitis. After examining the posterior segment and... (Review)
Review
Anterior Uveitis is the most common form of uveitis. There are several known and many possible etiologies for anterior uveitis. After examining the posterior segment and ruling out masquerade syndromes, the main step of etiologic diagnosis is clinical characterization. It is essential to establish the presence or absence of unilateral versus bilateral and granulomatous features. Subsequently, a directed work-up may be obtained which then helps to confirm diagnostic hypotheses based on the detailed history and clinical examination. The priority is to rule out an infection. Treatments are adapted according to etiology and disease severity. Finally, biologics have greatly changed the management and prevention of some forms of anterior uveitis, in particular uveitis associated with HLA-B27 and juvenile idiopathic arthritis-associated anterior uveitis.
Topics: Diagnosis, Differential; HLA-B27 Antigen; Humans; Uveitis, Anterior
PubMed: 29290458
DOI: 10.1016/j.jfo.2017.11.003 -
Clinics in Dermatology 2017Behçet disease is currently considered an "autoinflammatory disease" triggered by infection and environmental factors in genetically predisposed individuals. Although... (Review)
Review
Behçet disease is currently considered an "autoinflammatory disease" triggered by infection and environmental factors in genetically predisposed individuals. Although the disease is characterized by recurrent oral and genital aphthous ulcers and ocular involvement, it can affect multiple organ systems. Complex aphthosis is characterized by recurrent oral and/or genital aphthous ulcers. It is important to evaluate the patient with complex aphthosis for Behçet disease and related systemic disorders. We discuss the etiopathogenesis, clinical features, diagnostic criteria, and treatment approaches for complex aphthosis and Behçet disease in light of the current literature.
Topics: Behcet Syndrome; Humans; Recurrence; Stomatitis, Aphthous
PubMed: 28916023
DOI: 10.1016/j.clindermatol.2017.06.004 -
Indian Journal of Ophthalmology Jun 2022Sympathetic ophthalmia is a rare, bilateral, granulomatous, panuveitis following penetrating trauma or surgery to one eye. Clinical presentation commonly occurs within... (Review)
Review
Sympathetic ophthalmia is a rare, bilateral, granulomatous, panuveitis following penetrating trauma or surgery to one eye. Clinical presentation commonly occurs within the first year of trauma occurrence but can be delayed by several years. It manifests as acute/chronic granulomatous uveitis with yellowish-white choroidal lesions or Dalen-Fuchs nodules. Initially, patients respond rapidly to corticosteroid therapy, but a majority require long-term use of corticosteroid-sparing agents to prevent recurrences. The purpose of this review is to elaborate on the current understanding of the pathophysiology, the importance of multimodal imaging in early diagnosis, and the role of newer immunomodulatory and biological agents in recalcitrant cases.
Topics: Adrenal Cortex Hormones; Choroid; Humans; Ophthalmia, Sympathetic
PubMed: 35647958
DOI: 10.4103/ijo.IJO_2363_21 -
Anais Brasileiros de Dermatologia 2017Behçet's disease is a systemic vasculitis characterized by attacks of acute inflammation, which can affect almost every vascularized area of the body. There is a close... (Review)
Review
Behçet's disease is a systemic vasculitis characterized by attacks of acute inflammation, which can affect almost every vascularized area of the body. There is a close correlation between the geographical distribution of HLA-B51 and its prevalence. In the etiopathogenesis there are indications of genetic susceptibility associated with environmental influence. Among the involved genes are those that encompass innate and adaptive immunities. Polymorphisms and epistatic interactions in several genes are described, as well as the presence of imbalance lineage between HLA-B51 and A (MICA). Herpes simplex and Streptococcus sanguinis may be important extrinsic factors. An increase of Th1 response and of IL-21 is observed. The production of IL-21 is positively related to Th17 cells and negatively to T-regs. The mucocutaneous manifestations are Behcet´s disease markers, and their earlier onset indicates a worse prognosis. Recurrent oral ulcers have varied sizes and arrangements, genital ulcers are recurrent, leaving scars, skin lesions are multivaried, and pathergy, although not so frequent, is important for the diagnosis. There are numerous attempts to validate indexes that can evaluate the disease activity and among them the Mucocutaneous Activity Index. This is a specific score that can help with therapeutic decisions and to reduce morbidity, but still lacks validation. The clinical manifestations of other organs are described as well as treatment options.
Topics: Behcet Syndrome; Diagnosis, Differential; Gene-Environment Interaction; Humans; Immunity, Cellular; Review Literature as Topic
PubMed: 28954091
DOI: 10.1590/abd1806-4841.20177359 -
American Journal of Ophthalmology Aug 2021To determine classification criteria for Behçet disease uveitis. (Comparative Study)
Comparative Study
PURPOSE
To determine classification criteria for Behçet disease uveitis.
DESIGN
Machine learning of cases with Behçet disease and 5 other panuveitides.
METHODS
Cases of panuveitides 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
One thousand twelve cases of panuveitides, including 194 cases of Behçet disease with uveitis, were evaluated by machine learning. The overall accuracy for panuveitides was 96.3% in the training set and 94.0% in the validation set (95% confidence interval 89.0, 96.8). Key criteria for Behçet disease uveitis were a diagnosis of Behçet disease using the International Study Group for Behçet Disease criteria and a compatible uveitis, including (1) anterior uveitis; (2) anterior chamber and vitreous inflammation; (3) posterior uveitis with retinal vasculitis and/or focal infiltrates; or (4) panuveitis with retinal vasculitis and/or focal infiltrates. The misclassification rates for Behçet disease uveitis were 0.6% in the training set and 0% in the validation set, respectively.
CONCLUSIONS
The criteria for Behçet disease uveitis had a low misclassification rate and seemed to perform sufficiently well for use in clinical and translational research.
Topics: Adult; Behcet Syndrome; Consensus; Female; Humans; Machine Learning; Male; Retrospective Studies; Uveitis; Young Adult
PubMed: 33845008
DOI: 10.1016/j.ajo.2021.03.058 -
Ocular Immunology and Inflammation Dec 2023Uveitis is uncommon in children and its diagnosis and treatment are challenging. Little is known of the epidemiology of pediatric uveitis. Indeed, population-based... (Review)
Review
Uveitis is uncommon in children and its diagnosis and treatment are challenging. Little is known of the epidemiology of pediatric uveitis. Indeed, population-based studies in the literature are rare. However, there are many tertiary referral center reports that describe the patterns of uveitis in childhood, although few are from developed countries, and their comparison presents some issues. Anterior uveitis is the most frequent entity worldwide, especially in Western countries, where juvenile idiopathic arthritis is diffuse. Most cases of intermediate uveitis do not show any association with infectious or noninfectious systemic diseases. In low- and middle-income countries, posterior uveitis and panuveitis are prevalent due to the higher rates of infectious etiologies and systemic diseases such as Behçet disease and Vogt-Koyanagi-Harada disease. In recent decades, idiopathic uveitis rate has decreased thanks to diagnostic improvements.
Topics: Humans; Child; Retrospective Studies; Uveitis; Behcet Syndrome; Uveomeningoencephalitic Syndrome; Uveitis, Posterior
PubMed: 37922466
DOI: 10.1080/09273948.2023.2271988 -
Ophthalmology Feb 2021The Collaborative Ocular Tuberculosis Study (COTS), supported by the International Ocular Inflammation Society, International Uveitis Study Group, and Foster Ocular...
Collaborative Ocular Tuberculosis Study Consensus Guidelines on the Management of Tubercular Uveitis-Report 2: Guidelines for Initiating Antitubercular Therapy in Anterior Uveitis, Intermediate Uveitis, Panuveitis, and Retinal Vasculitis.
TOPIC
The Collaborative Ocular Tuberculosis Study (COTS), supported by the International Ocular Inflammation Society, International Uveitis Study Group, and Foster Ocular Immunological Society, set up an international, expert-led consensus project to develop evidence- and experience-based guidelines for the management of tubercular uveitis (TBU).
CLINICAL RELEVANCE
The absence of international agreement on the use of antitubercular therapy (ATT) in patients with TBU contributes to a significant heterogeneity in the approach to the management of this condition.
METHODS
Consensus statements for the initiation of ATT in TBU were generated using a 2-step modified Delphi technique. In Delphi step 1, a smart web-based survey based on background evidence from published literature was prepared to collect the opinion of 81 international experts on the use of ATT in different clinical scenarios. The survey included 324 questions related to tubercular anterior uveitis (TAU), tubercular intermediate uveitis (TIU), tubercular panuveitis (TPU), and tubercular retinal vasculitis (TRV) administered by the experts, after which the COTS group met in November 2019 for a systematic and critical discussion of the statements in accordance with the second round of the modified Delphi process.
RESULTS
Forty-four consensus statements on the initiation of ATT in TAU, TIU, TPU, and TRV were obtained, based on ocular phenotypes suggestive of TBU and corroborative evidence of tuberculosis, provided by several combinations of immunologic and radiologic test results. Experts agreed on initiating ATT in recurrent TAU, TIU, TPU, and active TRV depending on the TB endemicity. In the presence of positive results for any 1 of the immunologic tests along with radiologic features suggestive of past evidence of tuberculosis infection. In patients with a first episode of TAU, consensus to initiate ATT was reached only if both immunologic and radiologic test results were positive.
DISCUSSION
The COTS consensus guidelines were generated based on the evidence from published literature, specialists' opinions, and logic construction to address the initiation of ATT in TBU. The guidelines also should inform public policy by adding specific types of TBU to the list of conditions that should be treated as tuberculosis.
Topics: Algorithms; Antitubercular Agents; Chemotherapy, Adjuvant; Delphi Technique; Eye Infections, Bacterial; Glucocorticoids; Humans; Interferon-gamma Release Tests; Panuveitis; Radiography, Thoracic; Retinal Vasculitis; Retrospective Studies; Surveys and Questionnaires; Tomography, X-Ray Computed; Tuberculosis, Ocular; Uveitis, Anterior; Uveitis, Intermediate
PubMed: 32603726
DOI: 10.1016/j.ophtha.2020.06.052 -
Der Ophthalmologe : Zeitschrift Der... Sep 2021The standardization of uveitis nomenclature (SUN) working group is an international expert committee, which follows the aim to develop a standardized and... (Review)
Review
BACKGROUND
The standardization of uveitis nomenclature (SUN) working group is an international expert committee, which follows the aim to develop a standardized and internationally recognized terminology for the field of uveitis. This appears to be important in view of the demand for evidence-based medicine, especially for relatively rare diseases such as uveitis.
METHODS
A databank of > 4000 uveitis patients was compiled using formal consensus techniques, for whom a majority consensus was previously achieved in the diagnosis. The patient data were analyzed within the subclasses of uveitis and divided into a training set and a validation set. Multinomial logistic regressions with LASSO regularization were carried out on the training set with machine learning (ML). The accuracy of the rules that were developed to express the criteria of ML, were assessed by a masked observer in a random sample of 10%.
RESULTS
The estimations of total accuracy according to the uveitis classes in the validation set were high for all forms of uveitis: anterior uveitis 96.7% (95% confidence interval, CI 92.4-98.6%), intermediate uveitis 99.3% (95% CI 96.1-99.9%), posterior uveitis 98.0% (95% CI 94.3-99.3%), panuveitis 94.0% (95% CI 89.0-96.8%) and infectious posterior uveitis/panuveitis 93.3% (95% CI 89.1-96.3%).
CONCLUSION
Classification criteria are presented, which show a high degree of accuracy (low misclassification rates) and are therefore suitable for future clinical and translational research.
Topics: Humans; Panuveitis; Reference Standards; Uveitis; Uveitis, Anterior; Uveitis, Intermediate
PubMed: 34459962
DOI: 10.1007/s00347-021-01486-2 -
Harefuah Oct 2022A 68-year-old healthy female, with no past systemic or ocular history, presented with decreased vision in both eyes. On initial examination, there were extensive vitreal...
A 68-year-old healthy female, with no past systemic or ocular history, presented with decreased vision in both eyes. On initial examination, there were extensive vitreal opacities in both eyes. On the following visit, bilateral panuveitis was newly observed, without otherwise systemic clinical manifestations. The patient responded well to treatment with systemic and topical steroids. Initial workup excluded tuberculosis and syphilis. Angiotensin-converting enzyme (ACE) was within normal range. In order to make a definitive diagnosis and to exclude lymphoma, we decided to perform a vitreous biopsy after 2-weeks of steroids withdrawal. After cessation of steroids, new lesions appeared beneath the right eye and over the nasal bridge. Moreover, iris nodules over the stroma, pupil's margin and anterior chamber angle were noticed in both eyes. Biopsy from a skin lesion demonstrated non-caseating granulomas, supporting diagnosis of sarcoidosis. A chest computed tomography (CT) scan demonstrated compatible radiologic findings of bilateral hilar and mediastinal lymphadenopathy. Sarcoidosis is a chronic multisystem, autoimmune, granulomatous disease. Ocular involvement is common among patients with sarcoidosis, with the most common ocular manifestation being uveitis. We report a case that presented with bilateral panuveitis due to sarcoidosis with no systemic manifestations or elevated serum biomarkers. Definitive diagnosis was verified in histopathological findings from a skin biopsy and radiological findings in chest CT.
Topics: Humans; Female; Aged; Sarcoidosis; Uveitis; Biopsy; Tomography, X-Ray Computed; Panuveitis
PubMed: 36315210
DOI: No ID Found -
Journal Francais D'ophtalmologie Feb 2018Anterior uveitis is the most common form of uveitis. There are several known and many possible etiologies for anterior uveitis. After examining the posterior segment and... (Review)
Review
Anterior uveitis is the most common form of uveitis. There are several known and many possible etiologies for anterior uveitis. After examining the posterior segment and ruling out masquerade syndromes, the main step of etiologic diagnosis is clinical characterization. It is essential to establish unilateral versus bilateral involvement and presence or absence of granulomatous features. Subsequently, a work-up may be obtained which then helps to confirm diagnostic hypotheses based on the detailed history and clinical examination. The priority is to rule out an infection, although less frequent, before starting steroid therapy, adapted to the severity of the clinical picture. Finally, biologics have greatly changed the management and prevention of some forms of anterior uveitis, in particular uveitis associated with HLA-B27 and juvenile idiopathic arthritis-associated anterior uveitis.
Topics: Adrenal Cortex Hormones; Arthritis, Juvenile; Diagnosis, Differential; Diagnostic Techniques, Ophthalmological; HLA-B27 Antigen; Humans; Severity of Illness Index; Uveitis, Anterior
PubMed: 29395599
DOI: 10.1016/j.jfo.2017.06.012