Medicine › Ophthalmology

Ocular Diseases and Behçet’s Syndrome

Works Count: 50753

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Description

This cluster of papers focuses on the epidemiology, diagnosis, and management of ocular inflammatory diseases, particularly uveitis and Behçet's disease. It covers topics such as the use of immunosuppressive drugs, anti-TNF therapy, and interferon alpha2a therapy in the treatment of these conditions. Additionally, it discusses the epidemiological patterns and prevalence of ocular inflammatory diseases, including their association with juvenile idiopathic arthritis.

Keywords

Uveitis; Behçet's Disease; Vogt-Koyanagi-Harada Disease; Inflammatory Eye Diseases; Immunosuppressive Therapy; Anti-TNF Therapy; Juvenile Idiopathic Arthritis; Epidemiology; Interferon Alpha2a Therapy; Choroiditis

A new model of autoimmune disease. Experimental autoimmune uveoretinitis induced in mice with two different retinal antigens.

1988-03-01

Rachel R Caspi , François Roberge , C.–C. Chan +5 more

Experimental autoimmune uveoretinitis (EAU) is an organ-specific, T lymphocyte-mediated autoimmune disease, which serves as a model for several human ocular inflammations of an apparently autoimmune nature. EAU pathology in some … Experimental autoimmune uveoretinitis (EAU) is an organ-specific, T lymphocyte-mediated autoimmune disease, which serves as a model for several human ocular inflammations of an apparently autoimmune nature. EAU pathology in some rodents and in monkeys can readily be induced by immunization with several different retinal proteins; however, advancing research into the cellular mechanisms of this disease has raised the need for an EAU model in an immunologically and genetically well defined species. We report here the induction of EAU in the mouse, which has hitherto been considered a species refractory to EAU, with two retinal Ag, the retinal soluble Ag and the interphotoreceptor retinoid-binding protein. Although all the mouse strains tested exhibited lymphocyte responses and antibody titers to both retinal Ag, EAU was inducible in only some of the strains, and the uveitogenic responses to retinal soluble Ag and interphotoreceptor retinoid-binding protein appeared to be mutually exclusive. The EAU model in mice was found to differ in several respects from the EAU model in other rodent species. Induction of the disease was achieved with a relatively high dose of Ag and an intensified immunization protocol, and the onset of disease was later, the duration was longer, and the course was less acute. Anterior segment involvement was slight or nonexistent, and damage to the retina and uvea was of a focal rather than of a diffuse nature. Murine EAU appeared to approximate some types of human uveitis more closely than the EAU models described in other rodent species with respect to its pathologic manifestations as well as its more chronic course. The relatively longer duration of the active stage of disease in murine EAU should facilitate therapeutic intervention in established disease, which was not feasible in the more acute models of EAU. The extensive knowledge of the immunologic parameters of the mouse and the availability of genetically defined strains should be of great value in the study of cellular mechanisms and immunogenetics of ocular autoimmune disease.

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Uveitis: Fundamentals and Clinical Practice

1990-04-01

Robert B. Nussenblatt , Scott M. Whitcup

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA Ophthalmology HomeNew OnlineCurrent … Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA Ophthalmology HomeNew OnlineCurrent IssueFor Authors Podcast Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA Ophthalmology journal

Incidence and prevalence of uveitis in Northern California The Northern California Epidemiology of Uveitis Study

2004-02-27

David C. Gritz

Causes of Uveitis in the General Practice of Ophthalmology

1996-01-01

Colin A. McCannel , Gary N. Holland , Craig J. Helm +3 more

A Controlled Trial of Azathioprine in Behçet's Syndrome

1990-02-01

Hasan Yazıcı , Halit Pazarlı , C G Barnes +7 more

Cytotoxic agents have long been used in Behçet's syndrome, especially for eye involvement, but their effectiveness has been uncertain. We conducted a two-year randomized, placebo-controlled, double-blind trial of azathioprine (2.5 … Cytotoxic agents have long been used in Behçet's syndrome, especially for eye involvement, but their effectiveness has been uncertain. We conducted a two-year randomized, placebo-controlled, double-blind trial of azathioprine (2.5 mg per kilogram of body weight per day) in Turkish men with Behçet's syndrome without eye disease (group 1; n = 25) or with eye disease (group 2; n = 48). Corticosteroid treatment remained available to all the patients.

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The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria

2013-02-26

Fereydoun Davatchi , Samir H. Assaad‐Khalil , Kenneth Calamia +7 more

Abstract Objective Behçet's disease ( BD ) is a chronic, relapsing, inflammatory vascular disease with no pathognomonic test. Low sensitivity of the currently applied International Study Group ( ISG ) … Abstract Objective Behçet's disease ( BD ) is a chronic, relapsing, inflammatory vascular disease with no pathognomonic test. Low sensitivity of the currently applied International Study Group ( ISG ) clinical diagnostic criteria led to their reassessment. Methods An International Team for the Revision of the International Criteria for BD (from 27 countries) submitted data from 2556 clinically diagnosed BD patients and 1163 controls with BD ‐mimicking diseases or presenting at least one major BD sign. These were randomly divided into training and validation sets. Logistic regression, ‘leave‐one‐country‐out’ cross‐validation and clinical judgement were employed to develop new International Criteria for BD ( ICBD ) with the training data. Existing and new criteria were tested for their performance in the validation set. Results For the ICBD , ocular lesions, oral aphthosis and genital aphthosis are each assigned 2 points, while skin lesions, central nervous system involvement and vascular manifestations 1 point each. The pathergy test, when used, was assigned 1 point. A patient scoring ≥4 points is classified as having BD . In the training set, 93.9% sensitivity and 92.1% specificity were assessed compared with 81.2% sensitivity and 95.9% specificity for the ISG criteria. In the validation set, ICBD demonstrated an unbiased estimate of sensitivity of 94.8% (95% CI : 93.4–95.9%), considerably higher than that of the ISG criteria (85.0%). Specificity (90.5%, 95% CI : 87.9–92.8%) was lower than that of the ISG ‐criteria (96.0%), yet still reasonably high. For countries with at least 90%‐of‐cases and controls having a pathergy test, adding 1 point for pathergy test increased the estimate of sensitivity from 95.5% to 98.5%, while barely reducing specificity from 92.1% to 91.6%. Conclusion The new proposed criteria derived from multinational data exhibits much improved sensitivity over the ISG criteria while maintaining reasonable specificity. It is proposed that the ICBD criteria to be adopted both as a guide for diagnosis and classification of BD .

Causes and frequency of blindness in patients with intraocular inflammatory disease.

1996-04-01

Aniki Rothová , M S Suttorp-van Schulten , W F Treffers +1 more

AIMS/BACKGROUND: Uveitis, an intraocular inflammatory disease, is a significant cause of visual impairment. It is not known how many patients with uveitis will retain visual acuity and how many develop … AIMS/BACKGROUND: Uveitis, an intraocular inflammatory disease, is a significant cause of visual impairment. It is not known how many patients with uveitis will retain visual acuity and how many develop visual impairment or even blindness. The aim of this study was to assess the frequency of blindness in patients with uveitis and, more specifically, to identify the clinical profile of patients at risk for visual loss. METHODS: A cross sectional and retrospective study of 582 patients with uveitis who visited the ophthalmology departments of two university hospitals in the Netherlands was performed. RESULTS: Within the group of 582 patients, 203 (35%) exhibited blindness or visual impairment; bilateral legal blindness developed in 22 (4%) patients, 26 (4.5%) had one blind eye with visual impairment of the other, and nine (1.5%) had bilateral visual impairment. Unilateral blindness developed in 82 (14%) patients, whereas 64 (11%) exhibited unilateral visual impairment. The most important cause of both blindness and visual impairment was cystoid macular oedema (29% and 41%, respectively). Complications of uveitis were encountered in more than half of the patients and 23% underwent one or more surgical procedures. When the patients were subdivided according to anatomical site, those with panuveitis had the worst visual prognosis. The systemic diseases associated with a poor visual prognosis were juvenile chronic arthritis and sarcoidosis. Ocular toxoplasmosis was the most frequent cause of unilateral visual loss. CONCLUSIONS: Cystoid macular oedema is the most frequent complication of uveitis and its occurrence plays a decisive role in the visual outcome of this disease.

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Expert Panel Recommendations for the Use of Anti–Tumor Necrosis Factor Biologic Agents in Patients with Ocular Inflammatory Disorders

2013-12-17

Grace A. Levy-Clarke , Douglas A. Jabs , Russell W. Read +3 more

BEHÇETʼS DISEASE. REPORT OF 41 CASES AND A REVIEW OF THE LITERATURE

1975-05-01

Tova Chajek , M Fainaru

Several important conclusions may be derived on the basis of our experience with Behcet's disease (B.D.) and a review of the literature, namely: 1. B.D. is a systemic disease characterized … Several important conclusions may be derived on the basis of our experience with Behcet's disease (B.D.) and a review of the literature, namely: 1. B.D. is a systemic disease characterized by exacerbations and remissions of unpredictable duration, which affects mainly males between the ages of 20 to 30 years. Although its incidence is higher in the eastern hemisphere cases presented in all parts of the world. 2. The common histopathological lesion of all clinical manifestations appears to be a vasculitis. The etiology of B.D. is unknown. A viral etiology has been proposed but not confirmed. The presence of autoantibodies and lymphocyte sensitization to mucosal antigen has been observed but the precise significance of these findings in relation to the pathogenesis of the disease is presently undetermined. 3. The clinical manigestations present in most of the patients and considered diagnostic for B.D. are oral and genital ulcers, uveitis and skin lesions (especially erythema nodosum-like lesions or non-specific skin reactivity to needle pricks). The presence of three of the above manifestations are obligatory for the diagnosis. The other common clinical manifestations occurring in B.D. are: arthritis (44%); thrombophlebitis (24%), and various neurological syndromes (18%). Less frequent complications of the disease include: arterial thrombosis and aneurysm of both the systemic and pulmonary circulation, colitis, epididymitis and orchitis. 4. The serious chronic sequelae of this disease consist of blindness in up to 33% of patients with uveitis, vena caval obstruction and paralysis of limbs. Mortality, usually caused by meningoencephalitis, is rare. 5. The patients may present to different medical specialities, e.g., dermatology, gynecology, ophthalmology, neurology and internal medicine. To facilitate the correct diagnosis the essential criteria should be actively sought as they are not always the cause for medical consultation. 6. The diagnosis of B.D. is based only on clinical grounds as there are no pathognomonic laboratory or histopathological features. 7. Evaluation of treatment in B.D. is difficult, because of the naturally unpredictable course of the disease. Many drugs have been tried in the treatment of B.D. including antibiotics, anti-inflammatory drugs and corticosteroids, all with equivocal effects. Recently several additional treatment schedules have been suggested. Immunosuppressive drugs have been shown to have some beneficial effect, especially on uveitis. Fresh blood or plasma transfusions may prolong remissions. 8. The finding of decreased plasma fibrinolytic activity in patients with active B.D. represents the first observation of a possible disturbance in the blood coagulation mechanism and suggests that the use of fibrinolytic agents may be rewarding.

Scleritis and episcleritis.

1976-03-01

Peter Watson , Sohan Singh Hayreh

From the earliest available descriptions of episcleritis (Slade, 1838; Rognetta, i844; Desmarres, 1847) a confusion has existed between scleritis and episcleritis.This has led to contradictory views on the relative severity … From the earliest available descriptions of episcleritis (Slade, 1838; Rognetta, i844; Desmarres, 1847) a confusion has existed between scleritis and episcleritis.This has led to contradictory views on the relative severity and prognosis of these conditions, and the erroneous belief that episcleritis is accompanied by serious complications (Viswaling- ham, 1936; Wood, 1936; Mann and Markson, I950; Clavel and Teulieres, 1958).While Slade (I838), Rognetta (i844), and Desmarres (I847), thought Material Using the above classification, patients were distributed as in Table I.Normal anatomy Before considering the criteria by which the differential diagnosis is reached it is necessary to.

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Behçet disease (Behçet syndrome)

1979-05-01

T Shimizu , George E. Ehrlich , Goro Inaba +1 more

Genome-wide association studies identify IL23R-IL12RB2 and IL10 as Behçet's disease susceptibility loci

2010-07-11

Nobuhisa Mizuki , Akira Meguro , Masao Ôta +7 more

Endotoxin-induced uveitis in rats as a model for human disease

1980-08-01

James T. Rosenbaum , H O McDevitt , R. B. Guss +1 more

Neuro-Behçet's disease: epidemiology, clinical characteristics, and management

2009-01-20

Adnan Al-Araji , Desmond P. Kidd

The natural history of uveitis

1990-10-01

Robert B. Nussenblatt

Intraocular Tuberculosis—An Update

2007-11-01

Vishali Gupta , Amod Gupta , Narsing A. Rao

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Standardizatlon of Vitreal inflammatory Activity in Intermediate and Posterior Uveitis

1985-04-01

Robert B. Nussenblatt , Alan G. Palestine , Chi‐Chao Chan +1 more

The Long-Term Mortality and Morbidity of Behçet Syndrome

2003-01-01

Emire Kural-Seyahi , İzzet Fresko , Nurhan Seyahi +5 more

We surveyed the 20-year outcome of a cohort of patients with Behçet syndrome with emphasis on both mortality and morbidity. During 1999 and 2000, we collected outcome information on 387/428 … We surveyed the 20-year outcome of a cohort of patients with Behçet syndrome with emphasis on both mortality and morbidity. During 1999 and 2000, we collected outcome information on 387/428 (90.4%) of a cohort of patients (262 male, 125 female) who had registered in a dedicated outpatient clinic between July 1977 and December 1983. In 245/345 (71.0%) patients, outcome information was based on a formal hospital reevaluation, and in the remaining patients, on detailed telephone interviews. Forty-two patients (9.8%) (39 male, 3 female) had died, mainly due to major vessel disease and neurologic involvement. Mortality, as measured by standardized mortality ratios (SMR), was specifically increased among young males, among whom morbidity was also the highest. However, the SMR tended to decrease significantly with the passage of time. The same was also true for all mucocutaneous and articular manifestations. Both the onset of eye disease and its greatest damage were also usually within the first few years of disease onset. These suggest that the "disease burden" of Behçet syndrome is usually confined to the early years of its course, and in many patients the syndrome "burns out." However, central nervous system involvement and major vessel disease are exceptions. They can have their onset late (5-10 yr) during the disease course. As reflected in the mortality figures, the disease was less severe among the females for almost each disease manifestation. There were no female patients with arterial aneurysms. Severely impaired vision did not always mean an eventual loss of useful vision, and those patients with a late onset of eye disease had a better visual prognosis.

Dexamethasone Intravitreal Implant for Noninfectious Intermediate or Posterior Uveitis

2012-01-01

Careen Y. Lowder

TH17 cells contribute to uveitis and scleritis and are expanded by IL-2 and inhibited by IL-27/STAT1

2007-05-13

Ahjoku Amadi‐Obi , Cheng‐Rong Yu , Xuebin Liu +6 more

Uveitis in Behçet disease: An analysis of 880 patients

2004-09-01

İlknur Tuğal-Tutkun , Sumru Önal , Rana Altan-Yaycıoğlu +2 more

Genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Behçet's disease

2010-07-11

Elaine F. Remmers , Fulya Coşan , Yohei Kirino +7 more

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DOUBLE-MASKED TRIAL OF CYCLOSPORIN VERSUS COLCHICINE AND LONG-TERM OPEN STUDY OF CYCLOSPORIN IN BEHÇET'S DISEASE

1989-05-01

Kanjiro Masuda , A Urayama , M Kogure +3 more

Peroxidase labelled antibody and Fab conjugates with enhanced intracellular penetration

1971-12-01

Stratis Avraméas , Thérèse Ternynck

Vogt-Koyanagi-Harada syndrome

1995-01-01

Ramana S. Moorthy , Hajime Inomata , Narsing A. Rao

International Uveitis Study Group Recommendations for the Evaluation of Intraocular Inflammatory Disease

1987-02-01

E Bloch-Michel , Robert B. Nussenblatt

Either a Th17 or a Th1 effector response can drive autoimmunity: conditions of disease induction affect dominant effector category

2008-04-07

Dror Luger , Phyllis B. Silver , Jun Tang +7 more

Experimental autoimmune uveitis (EAU) represents autoimmune uveitis in humans. We examined the role of the interleukin (IL)-23–IL-17 and IL-12–T helper cell (Th)1 pathways in the pathogenesis of EAU. IL–23 but … Experimental autoimmune uveitis (EAU) represents autoimmune uveitis in humans. We examined the role of the interleukin (IL)-23–IL-17 and IL-12–T helper cell (Th)1 pathways in the pathogenesis of EAU. IL–23 but not IL-12 was necessary to elicit disease by immunization with the retinal antigen (Ag) interphotoreceptor retinoid-binding protein (IRBP) in complete Freund's adjuvant. IL-17 played a dominant role in this model; its neutralization prevented or reversed disease, and Th17 effector cells induced EAU in the absence of interferon (IFN)-γ. In a transfer model, however, a polarized Th1 line could induce severe EAU independently of host IL-17. Furthermore, induction of EAU with IRBP-pulsed mature dendritic cells required generation of an IFN-γ–producing effector response, and an IL-17 response by itself was insufficient to elicit pathology. Finally, genetic deficiency of IL-17 did not abrogate EAU susceptibility. Thus, autoimmune pathology can develop in the context of either a Th17 or a Th1 effector response depending on the model. The data suggest that the dominant effector phenotype may be determined at least in part by conditions present during initial exposure to Ag, including the quality/quantity of Toll-like receptor stimulation and/or type of Ag-presenting cells. These data also raise the possibility that the nonredundant requirement for IL-23 in EAU may extend beyond its role in promoting the Th17 effector response and help provide a balance in the current Th1 versus Th17 paradigm.

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Thalidomide in the Treatment of the Mucocutaneous Lesions of the Behcet Syndrome

1998-03-15

Vedat Hamuryudan

Background: Recurrent oral and genital ulcers are the most frequent problem in the management of the Behcet syndrome. Uncontrolled experience suggests that thalidomide may help prevent recurrences of these ulcers. … Background: Recurrent oral and genital ulcers are the most frequent problem in the management of the Behcet syndrome. Uncontrolled experience suggests that thalidomide may help prevent recurrences of these ulcers. Objective: To determine the efficacy of two thalidomide dosages in the treatment of mucocutaneous lesions of the Behcet syndrome. Design: Randomized, double-blind, placebo-controlled trial. Setting: Specialist outpatient clinic for the Behcet syndrome in Turkey. Patients: 96 male patients with the Behcet syndrome who primarily had mucocutaneous lesions without major organ involvement. Intervention: Thalidomide, 100 mg/d or 300 mg/d, or placebo for 24 weeks. Measurements: Sustained absence of any oral and genital ulceration during treatment (complete response) and changes in the number of mucocutaneous lesions. An additional evaluation was done 4 weeks after treatment ended. Results: A complete response occurred in 2 of the 32 patients (6% [95% CI, 0.8% to 20.8%]) receiving thalidomide, 100 mg/d; in 5 of the 31 patients (16% [CI, 5.5% to 33.7%]) receiving thalidomide, 300 mg/d; and in none of the 32 patients (0% [CI, 0% to 10.9%]) receiving placebo (P = 0.031). The suppressive effect of thalidomide with either dosage was evident at 4 weeks for oral ulcers (P < 0.001) and at 8 weeks for genital ulcers (P < 0.001) and follicular lesions (P = 0.008). This effect persisted during treatment but diminished rapidly after treatment was discontinued. Both thalidomide dosages led to significant increases in the number of erythema nodosum lesions during the first 8 weeks of treatment (P = 0.03). Polyneuropathy developed in 4 patients (1 in the 100-mg/d group and 3 in the 300-mg/d group); in 3 of these patients, the condition was diagnosed after the trial had ended. Conclusions: Thalidomide is effective for treating the oral and genital ulcers and follicular lesions of the Behcet syndrome. A dosage of 100 mg/d is as effective as a dosage of 300 mg/day.

Relation of Visual Function to Retinal Nerve Fiber Layer Thickness in Multiple Sclerosis

2006-01-11

Jerome P. Fisher , Dina Jacobs , Clyde Markowitz +7 more

Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel

2000-10-01

Douglas A. Jabs , James T. Rosenbaum , C. Stephen Foster +7 more

DESCRIPTION OF BEHCET'S SYNDROME IN THE HIPPOCRATIC THIRD BOOK OF ENDEMIC DISEASES

1956-06-01

Alexandre Feigenbaum

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Revised diagnostic criteria for Vogt-Koyanagi-Harada disease: report of an international committee on nomenclature11The authors constitute the International Committee on Vogt-Koyanagi-Harada Disease Nomenclature, representing the participants of the First International Workshop on Vogt-Koyanagi-Harada Disease. A full list of participants appears at the end of the article.

2001-05-01

Russell W. Read , Gary N. Holland , Narsing A. Rao +6 more

Intravitreal triamcinolone for uveitic cystoid macular edema: an optical coherence tomography study

2001-04-01

R J Antcliff , David J. Spalton , Miles Stanford +3 more

Standardization of Uveitis Nomenclature for Reporting Clinical Data. Results of the First International Workshop

2005-07-19

Douglas A. Jabs , Robert B. Nussenblatt , James T. Rosenbaum

EULAR recommendations for the management of Behcet disease

2008-02-02

Gülen Hatemi , Alan J. Silman , Dongsik Bang +7 more

To develop evidence-based European League Against Rheumatism (EULAR) recommendations for the management of Behçet disease (BD) supplemented where necessary by expert opinion.The multidisciplinary expert committee, a task force of the … To develop evidence-based European League Against Rheumatism (EULAR) recommendations for the management of Behçet disease (BD) supplemented where necessary by expert opinion.The multidisciplinary expert committee, a task force of the EULAR Standing Committee for Clinical Affairs (ESCCA), consisted of nine rheumatologists (one who was also a clinical epidemiologist and one also a Rehabilitation Medicine doctor), three ophthalmologists, one internist, one dermatologist and one neurologist, representing six European countries plus Tunisia and Korea. A patient representative was also present. Problem areas and related keywords for systematic literature research were identified. Systematic literature research was performed using Medline and the Cochrane Library databases from 1966 through to December 2006. A total of 40 initial statements were generated based on the systematic literature research. These yielded the final recommendations developed from two blind Delphi rounds of voting.Nine recommendations were developed for the management of different aspects of BD. The strength of each recommendation was determined by the level of evidence and the experts' opinions. The level of agreement for each recommendation was determined using a visual analogue scale for the whole committee and for each individual aspect by the subgroups, who consider themselves experts in that field of BD. There was excellent concordance between the level of agreement of the whole group and the "experts in the field".Recommendations related to the eye, skin-mucosa disease and arthritis are mainly evidence based, but recommendations on vascular disease, neurological and gastrointestinal involvement are based largely on expert opinion and uncontrolled evidence from open trials and observational studies. The need for further properly designed controlled clinical trials is apparent.

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Effect of infliximab on sight-threatening panuveitis in Behcet's disease

2001-07-01

PP Sfikakis , P. G. Theodossiadis , Christina Katsiari +2 more

The possible impact of uveitis in blindness: a literature survey.

1996-09-01

Maria S.A. Suttorp-Schulten , Aniki Rothová

The inflammatory process of the uvea is called uveitis and may cause sight threatening damage to the eye.The aetio- logical factor in about 30% of the cases of uveitis is … The inflammatory process of the uvea is called uveitis and may cause sight threatening damage to the eye.The aetio- logical factor in about 30% of the cases of uveitis is unknown.If established clinical entities without known cause, such as Fuchs' uveitis, are included, no aetiological agent or association with systemic disease can be identified in about 50% of cases.' Uveitis may be induced by infection, autoimmune disease, trauma, or malignancy.It is recommended that the different forms of uveitis should be classified according to their anatomical localisation- anterior uveitis, intermediate uveitis, posterior uveitis, and panuveitis." 3

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Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients

1999-11-01

G. Akman‐Demir , Piraye Oflazer , B. Taşcı

In order to define the patterns of neurological involvement in Behçet's disease and to assess prognostic factors, 558 files of the neuro-Behçet out-patient clinic were reviewed. Those patients without any … In order to define the patterns of neurological involvement in Behçet's disease and to assess prognostic factors, 558 files of the neuro-Behçet out-patient clinic were reviewed. Those patients without any evidence of objective neurological involvement as well as the patients with other possible explanations for the neurological picture, and cases not fulfilling the criteria for Behçet's disease were excluded. The remaining 200 cases (155 male, 45 female) were evaluated: 162 had parenchymal CNS involvement (brainstem or 'brainstem +' involvement in 51%, spinal cord involvement in 14%, hemispheric involvement in 15% and isolated pyramidal signs in 19%) while 38 had secondary or non-parenchymal CNS involvement. In the first group the most common findings were pyramidal signs, hemiparesis, behavioural changes and sphincter disturbance, whereas in the second group the syndrome of raised intracranial pressure due to dural sinus thrombosis was the main clinical manifestation. In 60% of the cases with parenchymal involvement, CSF was hypercellular and/or had an elevated protein level, whereas in cases with non-parenchymal involvement the CSF was usually normal except for the elevated pressure. In more than half of the patients with parenchymal involvement, MRI showed brainstem and/or basal ganglion lesions. Forty-one per cent of the cases had a course with at least one attack and remission, another 28% also had attack(s) but showed secondary progression, 10% had primary progression and 21% had silent neurological involvement. Survival analysis was performed in patients who had at least a 3-year duration of neurological disease. Parenchymal involvement, elevated protein and/or pleocytosis in the CSF, 'brainstem +' type involvement, primary or secondary progressive course and relapse during steroid tapering were all associated with a poorer prognosis.

Acute Posterior Multifocal Placoid Pigment Epitheliopathy

1968-08-01

J. Donald M. Gass

The clinical and fluorescein angiographic findings are described in three young female patients showing rapid loss of central vision secondary to multifocal, yellow-white, placoid lesions at the level of the … The clinical and fluorescein angiographic findings are described in three young female patients showing rapid loss of central vision secondary to multifocal, yellow-white, placoid lesions at the level of the pigment epithelium and choroid; rapid resolution of these lesions with permanent alterations in the pigment epithelium and minimal damage to the adjacent choroid and retina; and significant visual improvement which continued for several weeks or months after apparent ophthalmoscopic resolution of the acute lesions.

Degree, duration, and causes of visual loss in uveitis

2004-08-17

Omar M. Durrani

Background/aims: Uveitis is a major cause of visual morbidity in the working age group. The authors investigated the duration, degree, and causes of visual loss in uveitis patients with the … Background/aims: Uveitis is a major cause of visual morbidity in the working age group. The authors investigated the duration, degree, and causes of visual loss in uveitis patients with the aim of better defining the visual morbidity and identifying potential risk factors. Methods: A retrospective, non-interventional, observational survey of 315 consecutive patients attending a tertiary referral uveitis service. Results: The mean duration of follow up was 36.7 months. Reduced vision (⩽6/18) was found in 220/315 (69.95%) of the patients with a subset of 120 patients having vision ⩽6/60. Unilateral visual loss occurred in 109 (49.54%), while 111 (50.45%) had bilateral loss. The mean duration of visual loss was 21 months. Of the 148 patients with pan-uveitis, 125 (84.45%) had reduced vision, with 66 (53%) having vision ⩽6/60. Main causes of visual loss were cystoid macular oedema (CMO) (59/220, 26.8%), cataract (39/220, 17.7%), and combination of CMO and cataract (44/220, 20%). The following were predictive of a poorer visual prognosis: pan-uveitis (p = 0.0005), bilateral inflammation (p = 0.0005), increasing duration of reduced vision (p = 0.0005), an Indian or Pakistani ethnic background (p = 0.004), and increasing patient age (p = 0.02). Conclusion: Prolonged visual loss occurred in two thirds of uveitis patients, with 70 (22%) patients meeting the criteria for legal blindness at some point in their follow up. Older patients with bilateral inflammation and an increasing duration of reduced vision are at the greatest risk of severe visual loss (⩽6/60). CMO and cataract were responsible for visual loss in 64.5% of patients.

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Signs and Symptoms of Uveitis*

1959-05-01

Michael J. Hogan , Samuel J. Kimura , Phillips Thygeson

Criteria for diagnosis of Behçet's disease

1990-01-01

B Weichsler , Fereydoun Davatchi , Thomas Lehner +3 more

A Focus on the Epidemiology of Uveitis

2016-07-28

Theodora Tsirouki , Άννα Δαστιρίδου , Chrysanthos Symeonidis +4 more

Purpose: Uveitis is a common, sight-threatening inflammatory ocular disease and includes multiple heterogeneous clinical entities. The prevalence of various types of uveitis depends upon multiple factors, such as age, sex, … Purpose: Uveitis is a common, sight-threatening inflammatory ocular disease and includes multiple heterogeneous clinical entities. The prevalence of various types of uveitis depends upon multiple factors, such as age, sex, race, geographic distribution, environmental influence, genetics, and social habits. Epidemiologic research of uveitis is necessary to understand the etiology and immunopathogenesis of this group of diseases. The present study attempts to concentrate on the most recent information on the epidemiology of uveitis and compare it with previous knowledge.Methods: An extensive literature search was performed in the Medline database (PubMed) and included surveys completed until 2015. Articles that reported prevalence and incidence were studied. References cited in the articles were also studied.Results: The incidence and prevalence of uveitis differs based on age, anatomic location of the inflammatory process (anterior, intermediate, posterior uveitis, panuveitis), gender, histopathology (granulomatous, non-granulomatous), type of inflammatory process (acute, chronic, recurrent), and etiology (infectious, non-infectious). Prevalence differs by geographic location. Idiopathic anterior uveitis is the most common form of uveitis in the community. Infectious causes are common (30–60%) in the developing countries. Herpes and toxoplasmosis are the leading infectious causes of uveitis. Non-infectious uveitic conditions are generally more common in the developed world. An increase in the prevalence of infectious etiologies, including tuberculosis and syphilis, has been seen in developed countries. Introduction of new treatment options has also changed patterns of disease.Conclusions: Introduction of new uveitis entities, changes in the incidence of already known disease and increased availability of diagnostic testing have all altered the epidemiology of uveitis in recent years. Knowledge of regional patterns of disease is essential. A more detailed classification of uveitis with the establishment of uniform diagnostic criteria and prospective population based studies would certainly benefit epidemiologic research and clinical practice.

Adalimumab in Patients with Active Noninfectious Uveitis

2016-09-07

Glenn J. Jaffe , Andrew D. Dick , Antoine P. Brézin +7 more

Patients with noninfectious uveitis are at risk for long-term complications of uncontrolled inflammation, as well as for the adverse effects of long-term glucocorticoid therapy. We conducted a trial to assess … Patients with noninfectious uveitis are at risk for long-term complications of uncontrolled inflammation, as well as for the adverse effects of long-term glucocorticoid therapy. We conducted a trial to assess the efficacy and safety of adalimumab as a glucocorticoid-sparing agent for the treatment of noninfectious uveitis.This multinational phase 3 trial involved adults who had active noninfectious intermediate uveitis, posterior uveitis, or panuveitis despite having received prednisone treatment for 2 or more weeks. Investigators and patients were unaware of the study-group assignments. Patients were randomly assigned in a 1:1 ratio to receive adalimumab (a loading dose of 80 mg followed by a dose of 40 mg every 2 weeks) or matched placebo. All patients received a mandatory prednisone burst followed by tapering of prednisone over the course of 15 weeks. The primary efficacy end point was the time to treatment failure occurring at or after week 6. Treatment failure was a multicomponent outcome that was based on assessment of new inflammatory lesions, best corrected visual acuity, anterior chamber cell grade, and vitreous haze grade. Nine ranked secondary efficacy end points were assessed, and adverse events were reported.The median time to treatment failure was 24 weeks in the adalimumab group and 13 weeks in the placebo group. Among the 217 patients in the intention-to-treat population, those receiving adalimumab were less likely than those in the placebo group to have treatment failure (hazard ratio, 0.50; 95% confidence interval, 0.36 to 0.70; P<0.001). Outcomes with regard to three secondary end points (change in anterior chamber cell grade, change in vitreous haze grade, and change in best corrected visual acuity) were significantly better in the adalimumab group than in the placebo group. Adverse events and serious adverse events were reported more frequently among patients who received adalimumab (1052.4 vs. 971.7 adverse events and 28.8 vs. 13.6 serious adverse events per 100 person-years).In our trial, adalimumab was found to be associated with a lower risk of uveitic flare or visual impairment and with more adverse events and serious adverse events than was placebo. (Funded by AbbVie; VISUAL I ClinicalTrials.gov number, NCT01138657 .).

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Retinal layer segmentation in multiple sclerosis: a systematic review and meta-analysis

2017-09-12

Axel Petzold , Laura J. Balcer , Peter A. Calabresi +7 more

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2018 update of the EULAR recommendations for the management of Behçet’s syndrome

2018-04-06

Gülen Hatemi , Robin Christensen , Dongsik Bang +7 more

Several new treatment modalities with different mechanisms of action have been studied in patients with Behçet's syndrome (BS). The aim of the current effort was to update the recommendations in … Several new treatment modalities with different mechanisms of action have been studied in patients with Behçet's syndrome (BS). The aim of the current effort was to update the recommendations in the light of these new data under the auspices of the European League Against Rheumatism (EULAR) Standing Committee for Clinical Affairs. A task force was formed that included BS experts from different specialties including internal medicine, rheumatology, ophthalmology, dermatology, neurology, gastroenterology, oral health medicine and vascular surgery, along with a methodologist, a health professional, two patients and two fellows in charge of the systematic literature search. Research questions were determined using a Delphi approach. EULAR standardised operating procedures was used as the framework. Results of the systematic literature review were presented to the task force during a meeting. The former recommendations were modified or new recommendations were formed after thorough discussions followed by voting. The recommendations on the medical management of mucocutaneous, joint, eye, vascular, neurological and gastrointestinal involvement of BS were modified; five overarching principles and a new recommendation about the surgical management of vascular involvement were added. These updated, evidence-based recommendations are intended to help physicians caring for patients with BS. They also attempt to highlight the shortcomings of the available clinical research with the aim of proposing an agenda for further research priorities.

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Behçet's Disease

1999-10-21

Tsuyoshi Sakane , Mitsuhiro Takeno , Noboru Suzuki +1 more

BehÇet's disease is an inflammatory disorder of unknown cause, characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions.1,2 All these common manifestations are self-limiting except for the … BehÇet's disease is an inflammatory disorder of unknown cause, characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions.1,2 All these common manifestations are self-limiting except for the ocular attacks. Repeated attacks of uveitis can cause blindness.13 Behçet's disease is not a chronic, persistent inflammatory disease, but rather one consisting of recurrent attacks of acute inflammation. Involvement of the gastrointestinal tract, central nervous system, and large vessels is less frequent (Table 1), although it can be life-threatening.1,2,46 Susceptibility to Behçet's disease is strongly associated with the presence of the HLA-B51 allele.7,8 Environmental . . .

Criteria for diagnosis of Behcet's disease

1990-05-01

INTERNATIONALSTUDYGROUPFORBEHC

The Clinical Profile of Optic Neuritis

1991-12-01

Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease.

1990-05-05

Safety and Efficacy of High-Dosage Infliximab in Recalcitrant Retinal Vasculitis

2025-06-24

Yanliang Li , Thitiporn Thongborisuth , Ryan Lee +4 more | Ocular Immunology and Inflammation

We evaluated the long-term safety and efficacy of high-dosage infliximab and biosimilars (IFX+) in recalcitrant, non-infectious inflammatory retinal vasculitis (RV) after antimetabolite and adalimumab failure. This retrospective study included patients … We evaluated the long-term safety and efficacy of high-dosage infliximab and biosimilars (IFX+) in recalcitrant, non-infectious inflammatory retinal vasculitis (RV) after antimetabolite and adalimumab failure. This retrospective study included patients from the University of Illinois at Chicago Uveitis Service (2014-2024) who transitioned to IFX+ after prior treatment failure. Patients were categorized into low-dosage (LD, ≤5 mg/kg/Q4W, n = 14), medium-dosage (MD, 5.5-9.5 mg/kg/Q4W, n = 8), and high-dosage (HD, ≥10 mg/kg/Q4W, n = 9) groups. Efficacy was defined as ≥ 1 zone fluorescein angiographic (FA) improvement, prednisone reduction to < 10 mg/day with no flares, or both, at 6, 12, and 24 months, and last follow-up. Secondary outcomes included visual acuity (VA) and central macular thickness changes. At 12 months, efficacy was achieved by 57.1%, 87.5%, and 88.9% of LD, MD, and HD IFX+ patients, increasing to 83.3%, 75.0%, and 100% by last visits. FA improvement rate was 60.0% (LD), 50.0% (MD), and 87.5% (HD) at 12 months, increasing to 71.4%, 60.0%, and 100% at last visits. VA significantly improved in HD by 12 months (p = 0.03) and MD by 24 months (p = 0.02). LD and MD groups required dosage increases to sustain inflammation control. By 24 months, no significant dosage differences between groups existed. No adverse events were reported in the HD group. In this retrospective study, medium- and high-dosage IFX+ achieved better inflammation control and visual outcomes versus low-dosage IFX+, with no additional safety concerns, in treating recalcitrant non-infectious inflammatory RV. Early initiation at greater dosages may optimize IFX response, reduce treatment duration, and improve health-related quality of life.

Syphilitic uveitis masquerading as behçet’s disease: a case of bilateral macular edema with fern-like fluorescein leakage

2025-06-23

Ken Fukuda , Kenji Yamashiro | Infection

Artificial Intelligence‐Driven Proteomics Identifies Plasma Protein Signatures for Diagnosis and Stratification of Behçet's Disease

2025-06-23

Linlin Cheng , Mansheng Li , Bai Zhou +4 more | Advanced Science

The diagnosis of Behçet's disease (BD) predominantly relies on clinical symptoms, indicating an urgent for identifying potential biomarkers for early diagnosis and disease stratification. We employed an in-depth proteomics platform … The diagnosis of Behçet's disease (BD) predominantly relies on clinical symptoms, indicating an urgent for identifying potential biomarkers for early diagnosis and disease stratification. We employed an in-depth proteomics platform based on data-independent acquisition mass spectrometry (DIA-MS) and customizable antibody microarray technology, combined with machine learning methods. By analyzing the proteomic data in the training cohort, we trained an XGBoost machine learning model, and validated the model in an independent cohort. The model displayed a favorable performance in BD diagnosis and stratification. In the training set, the area under the curve (AUC) of the diagnostic model was 0.984 with an accuracy of 0.935. In the validation set, the AUC was 0.967 with an accuracy of 0.871. The AUCs for differentiating different severity BD groups ranged from 0.897 to 0.986 in the training set, and from 0.718 to 0.960 in the validation set. Functional analysis indicated that processes such as defense response, protein activation cascade, and complement activation were related to disease severity. Complement C4B was crucial in the protein-protein interaction network. This study is the first to construct an artificial intelligence-based BD diagnosis and stratification model, providing potential biomarkers and new strategies for precise diagnosis and treatment of BD. The diagnosis of BD predominantly relies on clinical symptoms, indicating an urgent for identifying potential biomarkers for early diagnosis and disease stratification. We employed an in-depth proteomics platform based on data-independent acquisition mass spectrometry (DIA-MS) and customizable antibody microarray technology, combined with machine learning methods. By analyzing the proteomic data in the training cohort, we trained an XGBoost machine learning model, and validated the model in an independent cohort. The model displayed a favorable performance in BD diagnosis and stratification. In the training set, the area under the curve (AUC) of the diagnostic model was 0.984 with an accuracy of 0.935. In the validation set, the AUC was 0.967 with an accuracy of 0.871. The AUCs for differentiating different severity BD groups ranged from 0.897 to 0.986 in the training set, and from 0.718 to 0.960 in the validation set. Functional analysis indicated that processes such as defense response, protein activation cascade, and complement activation were related to disease severity. Complement C4B was crucial in the protein-protein interaction network. This study is the first to construct an artificial intelligence-based BD diagnosis and stratification model, providing potential biomarkers and new strategies for precise diagnosis and treatment of BD.

Dexamethasone prodrug cyclodextrin aggregates effectively alleviate experimental autoimmune uveitis

2025-06-21

Xue Wang , Lu Liu , Di Wu +4 more | Colloids and Surfaces B Biointerfaces

Ocular involvement and visual prognosis in juvenile idiopathic arthritis associated uveitis

2025-06-20

Gonçalo Tardão , Filipe Moraes , Rita Carreira +5 more | International Journal of Ophthalmology

AIM: To characterize a Portuguese population of patients with juvenile idiopathic arthritis (JIA) and to evaluate possible associations between clinical factors and ocular involvement. METHODS: Patients diagnosed with JIA in … AIM: To characterize a Portuguese population of patients with juvenile idiopathic arthritis (JIA) and to evaluate possible associations between clinical factors and ocular involvement. METHODS: Patients diagnosed with JIA in the previous 20y in Hospital Garcia de Orta were included. Data were assessed from Reuma.pt database. Associations between demographic (age and sex), clinical (articular involvement, extra-articular manifestations, biological therapy), laboratory data [anti-nuclear antibodies, anti-cyclic citrullinated peptide (CCP) antibodies, rheumatoid factor, human leukocyte antigen B27 (HLA-B27), C-reactive protein, erythrocyte sedimentation rate] and ocular involvement were assessed. Statistical analysis was performed using Chi-square for categorical variables and Mann-Whitney test for continuous variables. RESULTS: Totally 91 patients were included, 11 (12%) with previous episodes of uveitis. There was a statistically significant preponderance of early age at JIA diagnosis (mean 4.73 vs 9.58y, P=0.008), antinuclear antibodies positivity (P=0.01), and oligoarticular subtype (P=0.04) in the Juvenile idiopathic arthritis-associated uveitis (JIA-U) group. Ocular complications occurred in 36.4% of patients (n=4): cataracts (n=2), band keratopathy (n=1) and posterior synechiae (n=1). The occurrence of complications was corelated with a shorter period between JIA diagnosis and the first JIA-U episode (mean 0.67 vs 4.88y, P=0.012) but not with age at JIA diagnosis or articular involvement. There was erythrocyte sedimentation rate elevation in the 12mo preceding uveitis (mean 40.5 mm/h, range 13-83). CONCLUSION: The occurrence of JIA-U shortly after JIA diagnosis is shown to be a potential risk factor for ocular complications.

Assessing disease phenotypes in Behçet’s syndrome: insights from a multiple correspondence analysis

2025-06-20

Rosaria Talarico , Federica Di Cianni , Antonello Sulis +3 more | Frontiers in Immunology

Introduction Behçet’s syndrome (BS) is a rare systemic vasculitis. Clinical manifestations in BS are frequently clustered rather than discrete, and the concept that distinct clinical phenotypes may exist in BS … Introduction Behçet’s syndrome (BS) is a rare systemic vasculitis. Clinical manifestations in BS are frequently clustered rather than discrete, and the concept that distinct clinical phenotypes may exist in BS has recently emerged. The aim of the present work was to identify and analyze the disease phenotypes in a monocentric historical cohort of patients with BS. Methods A total of 202 patients with BS diagnosis followed up at the Behçet Clinic of the Azienda Ospedaliero-Universitaria Pisana were identified, and demographics, clinical, and therapeutic data were retrospectively collected. Pairwise correlation among variables was evaluated by means of Pearson or Spearman correlation coefficient. A multiple correspondence analysis was performed to investigate the possible phenotypes resulting from the different patterns of associations among the demographic and clinical variables. Results Most of the patients were female (67%), Caucasian (92%), and HLA-B51 carriers (65.5%). Mean age at disease onset was 30.06 ± 11.39 years, and oral ulcers (OU) and genital ulcers (GU) were the most common manifestations (96% and 61%, respectively). According to bivariate correlation analysis, significant positive correlations were observed between skin lesions and both OU ( p = 0.005) and arthritis ( p = 0.014), as well as pathergy ( p = 0.001), gastrointestinal (GI) symptoms ( p = 0.001), and other involvement (fever and serositis) ( p = 0.015). Neurological involvement was significantly and positively associated with ocular lesions ( p = 0.0114), GI symptoms ( p = 0.030), pathergy (rho = 0.147, p = 0.037) and vein thromboses ( p = 0.037). Despite the high heterogeneity, four disease phenotypes emerged from the MCA: (A) male Caucasians with greater age at onset and at diagnosis than the median values, with OU and GU, skin lesions, erythema nodosum (EN), arthritis, and GI symptoms; (B) co-existence of benign subset and pathergy; (C) orchitis/epididymitis associated with neurological involvement and ocular lesions; and (D) GI symptoms plus endoscopic lesions, large vessel disease (both arterial and venous), and other involvement. Discussion This study provides valuable insights into the possible BS clinical phenotypes, and the results partially agree with previous association studies on European and extra-European cohorts. Observational comparative studies are warranted to assess the response of tailored phenotype-based therapeutic approaches.

Revising the role of cerebrospinal fluid pleocytosis as a diagnostic criterion in Vogt-Koyanagi-Harada disease

2025-06-19

Cristhian A. Urzúa , Marcelo Vega-Codd , Carlos Pavésio +7 more | Eye

Tocilizumab for Silicone Oil-Induced Recalcitrant Cystoid Macular Edema in a Patient with Recurrent Acute Retinal Necrosis-Associated Retinal Detachment

2025-06-18

Warren Pan , Mahsaw Mansoor , David Riccardi +4 more | Ocular Immunology and Inflammation

We report a case of refractory silicone oil-induced cystoid macular edema in a patient with recurrent acute retinal necrosis-related retinal detachments successfully treated with tocilizumab without silicone oil removal nor … We report a case of refractory silicone oil-induced cystoid macular edema in a patient with recurrent acute retinal necrosis-related retinal detachments successfully treated with tocilizumab without silicone oil removal nor reactivation of the acute retinal necrosis. Single-patient case report. A monocular patient with recurrent acute retinal necrosis-related retinal detachments necessitating indefinite silicone oil tamponade developed intractable cystoid macular edema immediately after silicone oil placement that was unresponsive to multiple therapies over nearly a year. After a single dose of tocilizumab, the patient demonstrated significant clinical improvement with resolution of the macular edema and reduction in petaloid macular leakage on fluorescein angiography. With continued tocilizumab treatment, silicone oil tamponade could be maintained, and no reactivation of acute-retinal necrosis was noted. This case represents the first documented successful use of tocilizumab in treating silicone oil-induced recalcitrant cystoid macular edema. When silicone oil cannot be removed from an eye and when steroids risk reactivating viral retinal disease, tocilizumab may offer a safe and effective treatment alternative for such macular edema.

Paradoxical Exacerbation of Birdshot Retinochoroiditis Following Rituximab

2025-06-18

Anamika Patel , Chi Yun Doreen Ho , William Tucker +2 more | Ocular Immunology and Inflammation

We report a case of exacerbated ocular inflammation following rituximab treatment in a patient with Birdshot Retinochoroiditis. Retrospective single case report. Following rituximab administration for Birdshot Retinochoroiditis, the patient developed … We report a case of exacerbated ocular inflammation following rituximab treatment in a patient with Birdshot Retinochoroiditis. Retrospective single case report. Following rituximab administration for Birdshot Retinochoroiditis, the patient developed bilateral increase in choroidal thickness with retinal pigment epithelium undulations and subretinal fluid on enhanced depth imaging optical coherence tomography (EDI-OCT). Indocyanine green angiography (ICGA) demonstrated bilateral fuzzy and obscured choroidal vessels with diffuse late hyperfluorescence. Electroretinography (ERG) showed delayed amplitudes, indicating retinal dysfunction. The patient was rescued with intravenous methylprednisolone, followed by high-dose oral steroids. Rituximab was discontinued, and abatacept was initiated. This case highlights a potential paradoxical exacerbation of intraocular inflammation following rituximab in Birdshot Retinochoroiditis, an observation already reported in other areas but rarely seen in ophthalmology.

Idiopathic Eruptive Macular Pigmentation Persisting for 11 Years

2025-06-17

Ryuji Matsumura , Reiko Kageyama , Chiaki Maruyama +1 more | The Journal of Dermatology

The Quality of Life Impact of Acute Uveitis: A Prospective Study

2025-06-17

Juliana Rocha de Mendonça da Silva , Ana Luiza Biancardi , Henrique Maciel Vieira de Moraes +1 more | Ocular Immunology and Inflammation

To evaluate the impact on quality of life of acute uveitis diagnosis and management. 50 patients with acute uveitis were followed prospectively for three months in a tertiary hospital in … To evaluate the impact on quality of life of acute uveitis diagnosis and management. 50 patients with acute uveitis were followed prospectively for three months in a tertiary hospital in Rio de Janeiro, Brazil. Vision-related (VR-) and health-related (HR-) quality of life (QoL) were respectively assessed by NEI-VFQ-25 and SF-36 questionnaires at the initial consultation and after three months. Associations between the questionnaire's subscale item scores with clinical/demographical data were assessed. After generalized linear models, in the initial QoL evaluation lower visual acuity (VA) and unemployment/retirement were associated with lower scores in both questionnaires, while bilateral uveitis was associated with NEI-VFQ-25 questionnaire only and older age with SF-36 only. At the three-month QoL evaluation lower VA and older age were associated with lower scores and VA improvement with higher scores in both questionnaires and unemployment/retirement was associated with lower SF-36 score only. When evaluating the variation between the initial QoL scores and those after three months, older age was associated with lower VR- and HR-QoL improvement, while short interval between referral and the first appointment, higher final VA and VA improvement were associated with VR-QoL upgrade. Bilateral uveitis was associated with lower HR-QoL improvement. Older age and worse VA have a significant negative impact on VR-QoL and HR-QoL, while VA improvement has positive impact. Anatomic classification and etiology of uveitis did not seem to impact on variation of QoL during diagnosis and treatment of acute uveitis, as well as education level and self-reported color.

Intraocular inflammation following intravitreal injections of anti-vascular endothelial growth factor drugs

2025-06-16

Ana Margarida Ferreira , Adélia Mendes , João Beato +7 more | Graefe s Archive for Clinical and Experimental Ophthalmology

Association between recurrent acute anterior uveitis and ankylosing spondylitis

2025-06-16

Amena Masrur , Muhammad Hanif , Faris Khan +3 more | Journal of Rehman Medical Institute

Introduction: Anterior uveitis stands out as the most frequently encountered extra articular findings of ankylosing spondylitis (AS). Anterior uveitis associated with AS is frequently encountered as acute anterior recurrent uveitis … Introduction: Anterior uveitis stands out as the most frequently encountered extra articular findings of ankylosing spondylitis (AS). Anterior uveitis associated with AS is frequently encountered as acute anterior recurrent uveitis (AARU). Objective: To analyze the association between recurrent acute anterior uveitis and the subsequent incidence of ankylosing spondylitis. Materials and Methods: This retrospective study was conducted in the Department of Ophthalmology and Orthopedics at Akbar Niazi Teaching Hospital, Islamabad Pakistan, from January 2018 to February 2023. AS patients with newly diagnosed acute anterior recurrent uveitis (AARU) (n=200) and matched control subjects without uveitis (n=200) were included in the study. AARU was diagnosed consecutively at 120-day intervals and managed with local or systemic steroids. AS incidence rates were based on the frequency of uveitis episodes, and incidence ratio were determined using the rate of incidence in control group as a reference. Data analyses were conducted using SPSS v 25. Results: AS rate of incidence after first uveitis episode was 40.6, compared to 5.6 in control group (IRR 3.7; 95% CI, 2.5–5.5). After second episode, increased IRR to 8.9 (95% CI, 5.2–15.0). In male patients with AARU, the incidence rates of AS were 94.7 per person year. In patients ≤ 40 years old, the IRR of AARU was 15.6 (95% CI, 6.5–37.2). In patients aged ≥ 60 years, the incidence of AS in AARU did not differ from the control group. Conclusion: The subsequent AS risk increased with frequency of acute anterior recurrent uveitis episodes. This analysis could help establish an importance for additional evaluation for potential systemic association in AARU.

Validation of the COTS Calculator for Tubercular Uveitis: Predictive Performance and Diagnostic Utility in an Indian Cohort

2025-06-16

Janakiraman Palani , Carlos Cifuentes‐González , Gazal Patnaik +6 more | Ocular Immunology and Inflammation

Diagnosing ocular tuberculosis (OTB) is clinically challenging due to its paucibacillary nature and lack of definitive diagnostic tests. The Collaborative Ocular Tuberculosis Study (COTS) Calculator was developed as a clinical … Diagnosing ocular tuberculosis (OTB) is clinically challenging due to its paucibacillary nature and lack of definitive diagnostic tests. The Collaborative Ocular Tuberculosis Study (COTS) Calculator was developed as a clinical decision-support tool to guide anti-tubercular therapy (ATT) initiation. This study externally validates the COTS Calculator in a high-burden Indian cohort and assesses the additive value of radiological and immunological testing. This retrospective cohort study included 196 OTB patients treated between 2015 and 2022 at a tertiary eye care center in South India. Inclusion required complete diagnostic workup, ≥6-month follow-up post-ATT, and at least one supportive test (TST, IGRA, CT, or CXR). Two thresholds were evaluated: M4I1 (median ≥ 4) and M4I2 (median ≥ 4, IQR ≤ 2). Treatment response and recurrence were primary outcomes. Diagnostic performance was measured using AUC, sensitivity, specificity, PPV, and NPV. Composite scores (CT+CXR and TST+IGRA) were also analyzed. The M4I2 threshold yielded higher sensitivity (83%) than M4I1 (60%) with comparable AUC (0.60 vs 0.58), though at lower specificity (38% vs 56%). Both thresholds achieved high PPV (94%) but poor NPV (11-17%). Composite testing showed similar high PPV (93%) and poor NPV (9-11%). Higher COTS scores and multiple positive tests correlated with favorable treatment response. The COTS Calculator, particularly the M4I2 model, provides a useful clinical tool to guide ATT decisions in OTB. While limited in ruling out TB, its integration with radiological and immunological testing supports early treatment decisions in TB-endemic settings. Prospective validation is warranted.

Recurrence of Intraocular Inflammation Following an Aflibercept 8-mg Injection in Eyes With a History of Intraocular Inflammation From Faricimab: A Case Report

2025-06-16

Shiori Sato , Yoichi Sakurada , Yoshiko Fukuda +2 more | Cureus

Long-Term Effectiveness and Safety of Weekly Adalimumab in Refractory Non-Infectious Uveitis

2025-06-13

Abdülcemal Gürpınar , Mertcan Esenkaya , Bilge Eraydın +2 more | Ocular Immunology and Inflammation

To assess the efficacy and safety of weekly adalimumab after dose escalation in patients with chronic refractory non-infectious uveitis. A retrospective analysis was conducted on patients aged 18 years and … To assess the efficacy and safety of weekly adalimumab after dose escalation in patients with chronic refractory non-infectious uveitis. A retrospective analysis was conducted on patients aged 18 years and older with persistent chronic uveitis despite bi-weekly adalimumab treatment. Data collected included uveitis diagnosis, anatomical site of involvement, history of immunosuppressive treatments, relapse frequency, and other relevant clinical parameters. This study included 30 patients (18 females and 12 males) with a median age of 33.5 (22-57) years and a mean follow-up period of 62.6 ± 25.2 months. Median visual acuity was 0.29 logMAR (0.22-0.40) with bi-weekly adalimumab treatment and improved to 0.20 logMAR (0.10-0.36) with weekly adalimumab treatment (p = 0.009). A significant reduction in central macular thickness was observed with weekly adalimumab treatment compared to the bi-weekly regimen (259.67 µm vs. 336.47 µm, p = 0.001). The frequency of relapses was 1.9 ± 0.66 with bi-weekly treatment and 0.53 ± 0.33 with weekly treatment (p = 0.001). The proportion of patients exhibiting active ocular inflammation requiring systemic steroids was 60% in the bi-weekly treatment, compared to 24.3% in the weekly treatment. In terms of adverse effect profile, both treatment modalities exhibited similar characteristics. Weekly adalimumab is associated with a significantly reduced risk of relapse compared with bi-weekly ADA (HR = 0.267, p = 0.001). Weekly adalimumab treatment is a viable option for managing inflammation in refractory uveitis, providing enhanced efficacy to the standard dose in terms of visual and anatomical outcomes, while maintaining a comparable side effect profile.

Comparison Between Behçet’s Uveitis Patients with Immunologically Positive and Immunologically Negative Tuberculosis Test

2025-06-13

Alaa Abdulrahman Almogahed , Maha Mohamed Youssef , Magda S. Abdelaziz +1 more | Ocular Immunology and Inflammation

To evaluate the clinical features of patients with Behçet's Uveitis in the presence or absence of latent tuberculosis infection (LTBI). A retrospective comparative study was carried out, examining patients with … To evaluate the clinical features of patients with Behçet's Uveitis in the presence or absence of latent tuberculosis infection (LTBI). A retrospective comparative study was carried out, examining patients with Behçet's disease who visited the Uveitis subspecialty clinic at Kasr Al-Ainy Hospital, Cairo University Hospitals. A total of 20 consecutive patients with positive tuberculosis immunology (group 1) and 20 consecutive patients with negative tuberculosis immunology (group 2) were included. Patients in group 2 showed a higher incidence of macular edema (p value = 0.013) and were more likely to receive systemic corticosteroids compared to group 1 (p value = 0.011). Conversely, vascular occlusion was more prevalent among group 1 patients (p value = 0.044). There was a significant increase in the use of systemic corticosteroids, immunosuppressive drugs, biologics, and anti-TB medications from the initial visit to the last visit (p value < 0.001). Genital ulcers, oral ulcers, GIT ulcers, and nodular erythema decreased significantly by the last visit compared to the initial presentation (p value < 0.05). Significant differences in laboratory findings such as anemia, high ESR, high C-reactive protein, and high HBA1c were observed between the two groups (p value < 0.001). Patients with immunologically negative TB tests were more likely to develop macular edema, whereas those with immunologically positive TB tests had a higher risk of vascular occlusion. Additionally, patients who have immunologically positive TB tests often present with elevated inflammatory markers like high ESR and C-reactive protein.

Multiple Evanescent White Dot Syndrome as Epiphenomenon of Infectious Chorioretinopathies

2025-06-13

Luca De Simone , Vanessa Ferraro , Elena Bolletta +6 more | Ocular Immunology and Inflammation

Multiple evanescent white dot syndrome (MEWDS) is a rare idiopathic ocular disorder often affecting young adults. Recently, a secondary form, epiphenomenon MEWDS (Epi-MEWDS), has been described in association with pre-existing … Multiple evanescent white dot syndrome (MEWDS) is a rare idiopathic ocular disorder often affecting young adults. Recently, a secondary form, epiphenomenon MEWDS (Epi-MEWDS), has been described in association with pre-existing retinal conditions. However, its correlation with infectious diseases remains poorly documented. A retrospective review of six patients with infectious chorioretinopathies who developed Epi-MEWDS was conducted. Clinical presentations, imaging findings, and disease progression were analyzed. This study presents a case series of six patients who developed Epi-MEWDS secondary to infectious chorioretinopathies, highlighting its distinct clinical features. Cases included Epi-MEWDS following Candida endophthalmitis, tubercular multifocal serpiginous-like choroiditis, ocular syphilis, and recurrent toxoplasmosis. Fundus autofluorescence and optical coherence tomography confirmed hyperautofluorescent lesions and outer retinal disruption in all cases. Patients were managed conservatively, and in all cases, imaging abnormalities resolved within a few weeks. This case series emphasizes the role of infectious disease-related immune responses in triggering Epi-MEWDS. In these cases disruptions in the outer blood-retinal barrier, leading to photoreceptor antigen exposure, appear to contribute to disease pathogenesis. Recognizing these cases can enhance understanding of post-infectious retinal immune responses and guide appropriate clinical management.

Dangerous intestinal disease: a case of recurrent abdominal pain with lower gastrointestinal bleeding

2025-06-13

Jinghua Hao , Chao-Nan Chen , Xin Fan +1 more | Gut

Importance of potential prothrombotic state markers in Behçet’s disease and correlation with vascular ultrasound

2025-06-12

Hazan Karadeniz , Tunjay Mammadov , Mahi Nur Cerit +7 more | Irish Journal of Medical Science (1971 -)

Recurrent Episcleritis: Unveiling The Causes And Systemic Links

2025-06-11

A Saisangeetha , Aparna AC | Journal of Neonatal Surgery

Background: Episcleritis is a common, typically benign ocular condition characterized by inflammation of the episcleral tissue. While often self-limiting, recurrent episcleritis may signal underlying systemic disease, including autoimmune conditions and … Background: Episcleritis is a common, typically benign ocular condition characterized by inflammation of the episcleral tissue. While often self-limiting, recurrent episcleritis may signal underlying systemic disease, including autoimmune conditions and latent tuberculosis (TB), particularly in TB-endemic regions like India. Objective: To investigate the clinical profile, systemic associations, and treatment outcomes in patients presenting with recurrent episcleritis. Methods: A cross-sectional study was conducted over six months at a tertiary care center involving 76 patients diagnosed with recurrent episcleritis. Detailed ocular and systemic evaluations were performed, including Mantoux testing, autoimmune markers, and chest imaging. Patients were followed up for a minimum of two months to assess recurrence and treatment response. Results: The majority of patients were female (61%) and aged between 30–50 years, with a mean age of 43.03±9.06 years. Unilateral (91%) and diffuse (80%) episcleritis were the most common presentations. Systemic associations were identified in 31 patients (41%), of which 26 (34%) were Mantoux positive. Among these, six patients with latent or prior TB-related findings were initiated on anti-tubercular therapy (ATT), resulting in complete resolution with no further recurrences. Conclusion: Recurrent episcleritis may be an ocular manifestation of systemic diseases, especially latent tuberculosis in endemic regions. Routine Mantoux testing and targeted systemic evaluation are critical in such cases. Initiation of ATT in selected patients demonstrated favorable outcomes, reinforcing the need for a multidisciplinary approach in managing recurrent episcleritis.

Clinicodemographic Data of Patients with Behçet’s Disease: Data from a Tertiary Center in Saudi Arabia

2025-06-10

Yasser Bawazir , Mohammad Zahid Mustafa | Current Rheumatology Reviews

Introduction: Behçet’s disease (BD) is a chronic inflammatory vasculitis involving the arteries and veins. This study was driven by the rarity, chronic multisystemic nature, and heterogeneous spectrum of clinical features … Introduction: Behçet’s disease (BD) is a chronic inflammatory vasculitis involving the arteries and veins. This study was driven by the rarity, chronic multisystemic nature, and heterogeneous spectrum of clinical features and geographical distribution. This study aimed to analyze the demographic and clinical characteristics of patients with BD at the King Abdulaziz University Hospital and identify the association between clinical and laboratory findings and disease severity. Methods: The study was a retrospective core chart review. This study included adult patients who visited the rheumatology clinic of King Abdulaziz University Hospital in Saudi Arabia between 2005 and 2023. The inclusion criteria were age ≥18 years and a diagnosis of Behçet’s disease (BD) based on either the International Criteria for Behçet’s Disease or the International Study Group classification criteria. Results: In total, 81 patients with BD with almost equal male (51.9%) and female (48.1%) distribution, 75.3% Saudi nationals, mean onset age of 38.48 years, and mean body mass index of 27.57 kg/m2 were identified. The most common clinical manifestations were oral ulcerations (56.8%), genital ulcerations (37%), uveitis (24.7%), arthritis (22.2%), skin lesions (13.6%), and deep vein thrombosis (9.88%). Significant differences in high-density lipoprotein, hemoglobin, Creactive protein, and albumin levels were associated with the age, sex, and nationality of the patients, respectively. Similarly, body mass index was significantly associated with C-reactive protein (p = 0.004), alanine aminotransferase (p = 0.023), aspartate aminotransferase (p = 0.003), and gamma-glutamyl aminotransferase (p = 0.034) levels. Conclusion: This study demonstrated that there are significant associations between demographic factors, laboratory parameters, and BD activity.

Ophthalmologic Disease in Behçet's Syndrome

2025-06-09

İlknur Tuğal-Tutkun , Yusuf Yazıcı | CRC Press eBooks

Identification of immune-associated genes and single-cell sequencing analysis in diagnosing Behçet’s disease

2025-06-06

Qiyi Yu , Yidong Zhang , Yifan Wu +2 more | Scientific Reports

Behçet's Disease (BD) is diagnosed as continuing systematic inflammation, lacking clarified etiology and pertinent detection of clinical usage. We screened bulk-seq and single-cell seq data of BD from GEO database, … Behçet's Disease (BD) is diagnosed as continuing systematic inflammation, lacking clarified etiology and pertinent detection of clinical usage. We screened bulk-seq and single-cell seq data of BD from GEO database, then investigated immune infiltration landscape in BD and further explored main immune cells in a single-cell resolution. It was inferred that monocytes might be the trigger cell of BD occurrence and development. On the other hand, we performed differentially expression analysis and weighted correlation network analysis, and intersected the selected genes with immune-related genes from ImmPort database. After KEGG/GO-enrichment, Protein-Protein Interaction network and miRNA-mRNA regulatory network were constructed. Meanwhile, we utilized the Least Absolute Shrinkage and Selection Operator to filtrate 5 significant intersected genes for BD's diagnosis (NRTN, TRDJ1, IGLV4-69, PDIA2 and AVPR1A), then drew Operating Characteristic curve to analyze diagnosis value. For experimental validation, the BD mice models were constructed and qRT-PCR experiment was performed to validate the expression level of selected genes. In conclusion, we investigated the immune landscape in BD and explored the role of monocyte in BD occurrence and development. Through gene selection and machine learning, five new potential diagnostic biomarkers were discovered and validated in both external dataset and experiment assay levels.

An Overview of Novel Formulations For Ocular Viral Infections: Focused On Nanomedicines

2025-06-06

Shalu Verma , Nidhi Nainwal , Divya Juyal | Drug Development and Industrial Pharmacy

Ocular viral infections are a common cause of blindness globally. Many ocular viral infections are mistakenly identified as bacterial infections. In these situations, treatment is initiated belatedly and fails to … Ocular viral infections are a common cause of blindness globally. Many ocular viral infections are mistakenly identified as bacterial infections. In these situations, treatment is initiated belatedly and fails to address the root cause of the infection, which frequently results in serious ocular complications like corneal infiltrates, conjunctival scarring, and decreased visual acuity. The efficacy of conventional treatments for viral infections suffers from poor bioavailability, which requires the development of novel methods of drug delivery, accurate diagnosis, and efficient treatment choices. As nanotechnology in medicine advances at a rapid pace, multifunctional nanosystems are being prioritized more and more to address the problems brought on by viral infections of the eyes offering targeted delivery, increased bioavailability and decreased systemic toxicity. This study delivers a thorough overview of the use of nanomedicines in the treatment of ocular viral infections, with a particular emphasis on how they may enhance the safety and efficacy of antiviral drugs. We address a range of nanocarrier systems, such as liposomes, nanoparticles, nanosuspension, proniosomes, in-situ gels, dendrimers, and nanogels, emphasizing their distinct characteristics that facilitate the effective transportation of antiviral drugs to ocular tissues. This article also highlighted the regulatory barriers of ocular nanoformulation. The transition of in-vitro studies to in-vivo and clinical models has been discussed. This review also highlights the Preclinical studies of ocular viral treatment, ocular nanotoxicology and advancement of ocular antiviral treatments in the form of patents, ongoing clinical trials and marketed formulations.

Pharmacotherapy for non-infectious uveitis: spotlight on phase III clinical trials of locally injected or implanted therapeutics and systemic immunomodulatory drugs

2025-06-05

Melissa K. Shields , Lísia Barros Ferreira , Syed Ali +4 more | Journal of Ophthalmic Inflammation and Infection

Panax notoginseng protects blood-retinal barrier by modulating microglia polarization via TLR4/MyD88/NF-κB pathway in endotoxin-induced uveitis

2025-06-05

Meizhong Peng , Shangqiu Ning , Yan Wu +7 more | International Immunopharmacology