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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 -
Rheumatology (Oxford, England) May 2020Behçet's syndrome (BS) is a systemic vasculitis characterized by a relapsing and remitting course. It can involve the skin, mucosa, joints, vessels (arteries and/or... (Review)
Review
Behçet's syndrome (BS) is a systemic vasculitis characterized by a relapsing and remitting course. It can involve the skin, mucosa, joints, vessels (arteries and/or veins), eyes, and nervous and gastrointestinal systems, and so is referred to as a syndrome rather than as a unique and nosologically distinct condition. These involvements may present alone or co-exist in the same patient. Although all the possible combinations of the above-mentioned manifestations may occur, clusters of commonly co-existing involvements (also referred to as 'disease phenotypes') have been suggested, namely 'mucocutaneous and articular', 'peripheral vascular and extra-parenchymal neurological' and 'parenchymal neurological and ocular' phenotypes have been described. Patient-specific demographic and genetic features have been described as positively or negatively associated with specific disease phenotypes. This review will focus on the different clinical features of Behçet's syndrome, summarizing current evidence on the distinct disease manifestations as well as the major phenotypes.
Topics: Behcet Syndrome; Humans
PubMed: 32348523
DOI: 10.1093/rheumatology/kez626 -
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 -
Arthritis Research & Therapy Oct 2022Clustering is an important clinical feature of Behçet's syndrome (BS) and may have pathogenetic and therapeutic implications. Recent and previous studies on BS...
Clustering is an important clinical feature of Behçet's syndrome (BS) and may have pathogenetic and therapeutic implications. Recent and previous studies on BS phenotype differ substantially in terms of methodology. Correlation matrices and factor analyses were not efficient enough to uncover clusters. Clustering patterns may change according to demographic factors such as age and sex. Clustering patterns may also be profoundly influenced by the misperception of symptoms that are assumed to be secondary to BS, when, in fact, they represent manifestations of BD mimics. This can give rise to misleading conclusions and should be kept in mind when interpreting data obtained by clustering or other phenotype analyses of BS. A true geographical/racial variability in disease expression could be studied in a multinational consensus cohort. Pathogenetic studies in separate clusters of BS have still been lacking.
Topics: Humans; Behcet Syndrome; Cluster Analysis; Phenotype
PubMed: 36309718
DOI: 10.1186/s13075-022-02937-0 -
Therapeutische Umschau. Revue... Jun 2022Behçet's disease Behçet's disease (BD) represents a diagnostic and therapeutic challenge for the treating physician. The rarity of the disease and its heterogeneous...
Behçet's disease Behçet's disease (BD) represents a diagnostic and therapeutic challenge for the treating physician. The rarity of the disease and its heterogeneous clinical presentations hamper physicians in easily gaining knowledge about the disease. Moreover, the diagnosis of BD commonly relies on clinical grounds only and treatment decision are guided by relatively few data from clinical trials. In this article, some basic principles for diagnosing and managing BD are reviewed.
Topics: Behcet Syndrome; Humans
PubMed: 35583026
DOI: 10.1024/0040-5930/a001354 -
Ophthalmology Dec 2023
Topics: Humans; Suppuration; Uveitis, Anterior; Eye Abnormalities
PubMed: 36849294
DOI: 10.1016/j.ophtha.2023.01.018 -
Nature Reviews. Disease Primers Sep 2021
Topics: Behcet Syndrome; Humans
PubMed: 34531407
DOI: 10.1038/s41572-021-00306-w -
Graefe's Archive For Clinical and... Feb 2024To report the clinical and fluorescein angiographic (FA) features of demyelinating plaque-associated uveitis (DPU), a subset of uveitis in which patients have... (Observational Study)
Observational Study
PURPOSE
To report the clinical and fluorescein angiographic (FA) features of demyelinating plaque-associated uveitis (DPU), a subset of uveitis in which patients have demyelinating plaques on the brain/cervical magnetic resonance image (MRI) but do not meet the criteria for multiple sclerosis (MS).
METHODS
In this retrospective observational study, Persian Patients were diagnosed with DPU and included if (1) they never satisfied the MS criteria, (2) all other possible etiologies were excluded, and (3) they were followed for at least 2 years.
RESULTS
After a median follow-up of 3 years (interquartile range, 2.0-5.3), 8 out of 40 (20%) patients diagnosed with DPU were excluded as they subsequently met the MS criteria. Of remaining 32 patients studied, the mean age was 36.3±9.9 (range 20-56 years), and 30 (93.8%) were female. Twenty-four (75.0%) showed bilateral involvement and 27 (84.4%) had insidious-chronic course. Uveitis was classified as intermediate (with or without anterior uveitis) in 29 (90.6%) and isolated anterior in 3 (9.4%) patients. Nine (28.1%) patients had at least one systemic neurological complaint. Ocular findings were: granulomatous keratic precipitates in 43/44 (97.7%) eyes; snowballs in 25/52 (48.1%) eyes; snowbanks in 4/52 (7.7%) eyes; cystoid macular edema in 20/56 (35.7%) eyes; and optic neuritis in 5/56 (8.9%) eyes. Visual acuity was ≥ 20/40 in 39 eyes (69.6%) at presentation which improved to 46 eyes (81.2%) at 2-year follow up. The two most frequent findings in FA were optic disc leakage/staining in 44/52 (81.5%) eyes, and peripheral retinal perivascular leakage in 39/52 (76.9%) eyes, which in 14/52 (26.9%) eyes extended beyond the equator.
CONCLUSION
DPU usually presents as a bilateral chronic granulomatous intermediate and, less often, isolated anterior uveitis, especially in females. Most are neurologically asymptomatic. Visual outcome is generally favorable. In FA, peripheral retinal perivascular leakage is common. DPU patients have an increased tendency to develop MS and should be prohibited from anti-TNF treatment.
Topics: Humans; Female; Young Adult; Adult; Middle Aged; Male; Tumor Necrosis Factor Inhibitors; Uveitis; Uveitis, Anterior; Retina; Fluorescein Angiography; Retrospective Studies; Plaque, Atherosclerotic; Uveitis, Intermediate
PubMed: 37855958
DOI: 10.1007/s00417-023-06270-3 -
Clinical Immunology (Orlando, Fla.) Jun 2023Disease assessment has been challenging in Behçet syndrome due to the heterogeneous disease course and multiorgan involvement with variable treatment response. There... (Review)
Review
Disease assessment has been challenging in Behçet syndrome due to the heterogeneous disease course and multiorgan involvement with variable treatment response. There have been several recent improvements regarding outcome measures including development of a Core Set of Domains for Behçet syndrome and novel instruments for assessing specific organs and overall damage. This review focuses on the current state of outcome measures in Behçet syndrome, unmet needs, and a research agenda towards the development of standardized and validated outcome measure instruments.
Topics: Humans; Behcet Syndrome; Outcome Assessment, Health Care; Disease Progression
PubMed: 37100337
DOI: 10.1016/j.clim.2023.109341