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To describe the long-term prevalence of ocular complications and visual prognosis in patients with pediatric uveitis. Demographics, etiology and location of uveitis, type of complications, treatment and visual outcomes were recorded in 296 children at first examination and at 1-, 2-, 3-, 5- and 10-year time points. Αnterior uveitis represented 53.4% of cases, followed by intermediate (28.0%), posterior uveitis (11.1%) and panuveitis (7.4%). The leading diagnoses were idiopathic uveitis (31.1%), juvenile idiopathic arthritis (27.0%) and pars planitis (22.6%). Posterior synechiae was the most frequent complication of anterior uveitis and panuveitis, cystoid macular edema and disc edema of intermediate and posterior uveitis respectively. Posterior uveitis and panuveitis had more severe final vision loss (23.1% and 20% respectively). This study provides clinical characteristics and main complications in a longitudinal long-term follow-up of a large non-infectious pediatric uveitis Greek population. Early diagnosis and close monitoring remain of fundamental importance. [ABSTRACT FROM AUTHOR]

Bacground Idiopathic uveitis is the most common uveitis type and is viewed as an inconclusive diagnosis for patients as well as physicians. Human leukocyte antigen (HLA) typing helps in understanding the pathogenesis of several diseases. Limited knowledge is known regarding the association of HLA with idiopathic uveitis. Aim This was a cross-sectional observational case–control study evaluating HLA-A and HLA-B allelic and phenotypic frequencies in patients with idiopathic uveitis. Patients and methods HLA-A and HLA-B molecular typing by PCR-sequence-specific oligonucleotide probes and LIRAS interpretation software was performed for 60 patients with idiopathic uveitis and 60 controls recruited from Kasr Al-Ainy Hospitals, Cairo University. Results Anterior uveitis pattern was the most common (55%), followed by pan-uveitis (35%) and posterior uveitis (10%). Overall, 40% of patients had bilateral uveitis, whereas 60% had unilateral uveitis. A total of 23 HLA-A and 43 HLA-B variant alleles were detected. HLA-AFNx0109 and HLA-AFNx0110 allele and phenotype frequencies were significantly higher in the patient group (P=0.023 and 0.034, and P=0.013 and 0.029, respectively). HLA-BFNx0107 allele and phenotype frequencies were significantly higher in the control group (P=0.007 and 0.006, respectively). HLA-BFNx0127 and HLA-BFNx0105 were detected in 8.3 and 26.7%, respectively, with no significant difference. Conclusion HLA-AFNx0109 and HLA-AFNx0110 alleles were statistically significantly higher in patients with idiopathic uveitis. HLA-BFNx0107 might be a protective allele against disease development. Larger cohort needs to be tested to validate these findings. Routine HLA typing and proper follow-up for primarily diagnosed idiopathic uveitis is recommended as it might reveal secondary causes for uveitis, especially in patients with positive HLA-BFNx0127 and HLA-BFNx0105.

"Idiopathic" is the most common category of uveitis, representing cases in which a specific diagnosis has not been established despite work-up. Sarcoidosis is a systemic granulomatous disorder affecting multiple organs including the lungs, skin, kidneys, and eyes. We used microRNA (miRNA) microarrays to investigate serum miRNA profiles of patients with ocular sarcoidosis as diagnosed by specific criteria (diagnosed ocular sarcoidosis), and patients with idiopathic uveitis characterized by ocular manifestations of sarcoidosis (suspected ocular sarcoidosis). Principal component analysis (PCA) and hierarchical clustering showed that serum miRNA profiles of diagnosed ocular sarcoidosis and suspected ocular sarcoidosis were both clearly distinguishable from healthy controls. Furthermore, comparative analysis of the miRNA profiles showed highly similar patterns between diagnosed ocular sarcoidosis and suspected ocular sarcoidosis. Pathway analysis revealed common pathways were involved in the two groups, including those of WNT signaling and TGF-beta signaling. Our study demonstrated a high overlap of differentially expressed serum miRNAs in patients with diagnosed ocular sarcoidosis and suspected ocular sarcoidosis, suggesting that these groups share a similar underlying pathology and may represent possible variants of the disease. Characterization of serum miRNA profiles may provide an opportunity for earlier diagnosis and treatment, and may inform more accurate clinical prognosis in patients with an ocular sarcoidosis phenotype. [ABSTRACT FROM AUTHOR]

Objective: To investigate the characteristics, evolution, and visual outcome of non-infectious uveitis. Methodology: Records of 201 patients with non-infectious uveitis (136 (67.7%) males and 84 (41.8%) juvenile-onset (≤ 16 years)) were retrospectively reviewed. Data were analyzed through Kruskal–Wallis and Mann–Whitney, chi-square (χ2) tests, and logistic regression. Results: The median disease and follow-up durations were 36 (interquartile range (IQR) 24–70) and 24 (IQR 10–36) months, respectively. Fifty-eight (28.9%) patients had persistently idiopathic uveitis, and 143 (71.1%) were associated with rheumatic diseases, of whom uveitis heralded, coincided with, and succeeded the rheumatic manifestation(s) in 62/143 (43.4%), 37/143 (25.9%), and 44/143 (30.7%) patients, respectively. Established rheumatic diseases were Behçet's disease (103/201 (51.2%)), juvenile idiopathic arthritis (13/201 (6.5%)), sarcoidosis (8/201 (4%)), seronegative spondyloarthropathy (7/201 (3.5%)), and Vogt-Koyanagi-Harada (7/201 (3.5%)), and other diagnoses were present in 5/201 (2.5%) patients. Patients with idiopathic uveitis were characterized by a juvenile-onset (p < 0.001), lower male predominance (p = 0.01), prevalent granulomatous (p < 0.001), and anterior (p = 0.001) uveitis. The median visual acuity at last visit was 0.3 (IQR 0.05–0.6). Visual loss was present in 45/201 (22.3%) patients (36/201 (17.9%) unilateral and 9/201 (4.4%) bilateral). Apart from a longer disease duration (p = 0.002), lower educational level (p = 0.03), and prevalent panuveitis (p < 0.001), visual loss was not associated with any other studied ocular or extra-ocular characteristics. Conclusion: Behçet's disease (51.2%) and idiopathic uveitis (28.9%) were the most prevalent causes of non-infectious uveitis in our study. Visual loss (22.3%) was associated with a longer disease duration, lower education level, and prevalent panuveitis. Key Points • Most common causes of uveitis referred to rheumatologists were Behçet's disease and idiopathic uveitis. • Several rheumatic diseases initially presented only with uveitis, more commonly in adult and male patients. • Panuveitis was more frequent among patients with an established rheumatic disease, whereas granulomatous uveitis was uncommon. • Longer disease duration and presence of panuveitis were independently associated with visual loss. [ABSTRACT FROM AUTHOR]

Purpose: To compare the thickness of retinal nerve fi ber layer (RNFL) in HLA-B27 positive and HLA-B27 negative acute anterior uveitis (AAU) patients with active inflammation. Methods: Thirty-seven patients with HLA-B27-positive AAU and 32 patients with HLA-B27-negative AAU were analyzed. Clinical characteristics, HLAB27 typing and retinal nerve fi ber layer thickness measurements were recorded. All data were compared with 35 age and sex matched healthy subjects. ROC analysis was used to determine the RNFL threshold in patients with HLA B27 positive AAU. Results: There was no significant difference between the HLA-B27 (-) group and the control group in terms of RNFL thickness in the temporal, temporal inferior and nasal inferior quadrants. (P = 0.293, p = 0.064, p = 0.033, respectively) In all other quadrants, RNFL thickness of the HLA-B27 (+) and HLA-B27 (-) groups was thicker than the control group. RNFL thicknesses in temporal superior, nasal superior and nasal inferior quadrants were significantly thicker in the HLA-B27 (+) group compared to the HLA-B27 (-) group. (P = 0.008, p = 0.012, p = 0.016, respectively) According to ROC analysis, RNFL thresholds were 139 µm, 111.50 µm and 110.50 µm in temporal superior, nasal superior and nasal inferior quadrants, respectively. A significant and positive correlation was found between RNFL and anterior chamber cell counts in all quadrant AAU patients. Conclusion: RNFL thickness increases more in HLA-B27 positive patients than in negative patients. RNFL thickness above the threshold values determined by ROC analysis may indicate HLA-B27 positivity. [ABSTRACT FROM AUTHOR]

Vogt-Koyanagi-Harada (VKH) disease is an idiopathic immune-related sickness that affects multiple systems and melanocytes in organs such as the uvea, ear, and meninges. The primary cause of activity is cellular immunological responses. Vogt-Koyanagi disease is identified primarily by skin abnormalities and anterior uveitis. Harada's illness is distinguished by neurological symptoms and exudative retinal detachments, which are associated with the HLA-DR4 and HLA-DRw53 genes. Pigmented races, such as Hispanics and Native Americans, are more likely to have VKH disease. Clinical features are blurred vision, floaters, alopecia, vitiligo, diffuse choroidal inflammation with disc edema, and exudative retinal detachment. Differential diagnoses include posterior scleritis, uveal effusion syndrome, central serous chorioretinopathy, and sympathetic ophthalmitis. The investigations used are optical coherence tomography (OCT), fundus fluorescein angiography (FA), and B-scan ultrasonography (USG). Treatment is done by using systemic steroids, cycloplegics, and immunosuppressants., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Tayal et al.)

Aim: To study patterns of uveitis in Indian children and compare with data sets published earlier in the literature.Methods: Consecutive patients below 16 years of age presenting to the uvea clinic of a tertiary eye care center were included prospectively through the period of July 2009-August 2013. Children with retinal vasculitis, exogenous endophthalmitis and masquerade syndromes were excluded from analysis. Uveitis was classified as per the nomenclature system adopted by the International Uveitis Study Group. Hemogram, Mantoux test and chest X-ray were done for each patient, along with tailored investigations and pediatric review as per clinical profile. Clinical pattern and etiology were the main outcome measures.Results: One hundred and thirty-four children were analyzed. Anterior uveitis (40%) was the commonest pattern followed by intermediate uveitis (25%), panuveitis (18%) and posterior uveitis (17%). Bilateral disease was present in 54%, 15% had infectious uveitis, 10% had granulomatous uveitis and 54% had idiopathic uveitis. Complications were present in half of the patients. Juvenile idiopathic arthritis (22), followed by toxoplasmosis (10) and tuberculosis (5), was the commonest etiology. Intermediate uveitis, non-granulomatous inflammation and older onset of disease had the high odds ratio of having idiopathic disease.Conclusion: Patterns of pediatric uveitis can vary between regions from even within the same geopolitical region. Anterior uveitis is commonest, and juvenile idiopathic arthritis and toxoplasmosis are the most frequent etiologies. Diagnosis of pediatric ocular tuberculosis is more difficult than in adults and needs better and well-defined criteria. [ABSTRACT FROM AUTHOR]

Objectives: Idiopathic uveitis is the most common form of uveitis in most countries. Uveitis affects about 40--80% of patients with Behçet's disease (BD). Class I, HLA-B5, and its subclass B51 allele have the strongest association with BD, but its role in idiopathic uveitis is unclear. The aim of this study was to determine the frequency of HLA-B5, HLA-B51 and HLA-B27 in patients with idiopathic uveitis, BD and the control group. Material and methods: Forty-eight patients with idiopathic uveitis, 62 patients with BD, and 49 control subjects were compared. The presence of HLA-B5, HLA-B51 and HLA-B27 was checked by reviewing the charts of patients with idiopathic uveitis and Behçet's disease referred to the rheumatologic center of Shiraz University of Medical Sciences. The control group consisted of a sex-matched normal control population, among which HLA typing was done. Results: HLA-B5 was significantly higher in patients with idiopathic uveitis and BD compared to the control group (p = 0.029 and 0.0001). It was significantly higher in patients with BD compared to those with idiopathic uveitis (p = 0.001). The difference of HLA-B51 between the groups was not statistically significant although it was higher in the BD group than the controls. The presence of HLA-B51 was significantly associated with ocular involvement in the BD group (p = 0.013). HLA-B27 was the least common type of HLA in all groups. Conclusions: HLA-B5 was the most common, compared to HLA-B51 and HLA-27, in patients with idiopathic uveitis and BD. There was an association between HLA-B51 positivity and ocular involvement in patients with BD. It seems that the pattern of HLA in our patients with idiopathic uveitis was different from that observed in some other studies. [ABSTRACT FROM AUTHOR]

Objective: Uveitis is one of the important manifestations of Behcet disease (BD). High sensitive C-reactive protein (hs-CRP) is an acute phase reactant, which may increase in BD. There are controversies in different studies regarding the relation between hs-CRP and uveitis in BD. This study aimed to determine serum values of hs-CRP and its relationship with uveitis in BD. Material and Methods: A total of 94 patients, divided into 3 groups (Behcet's uveitis (n = 31), Behcet's without uveitis (n = 37) and idiopathic uveitis (n = 26), participated in the study. Serum levels of hs-CRP were compared among those groups. Results: The mean serum hs-CRP level was 6.49 ± 3.27 mg/L (BD with uveitis), 6.41 ± 3.85 mg/L (BD without uveitis) and 6.97±3.9 mg/L (idiopathic uveitis. Tukey test was done among groups and there were no significant differences between them (P = 0.69). Conclusion: Our results revealed no significant difference among the 3 groups regarding the mean serum hs-CRP level. However, further studies are warranted in this regard. [ABSTRACT FROM AUTHOR]

Objectives: The present study aimed to determine the pattern and cause of noninfectious uveitis in rheumatology practice. The secondary objective was to identify the pattern of treatment and outcomes., Materials and Methods: This retrospective cross-sectional study was conducted in the Department of Rheumatology, National Hospital and Medical Centre, Lahore, Pakistan. After receiving consent, electronic medical records (EMRs) of all patients with a diagnosis of noninfectious uveitis (NIU) from November 2019 to January 2023 were reviewed, and a total of 52 patients labeled as having noninfectious uveitis were identified. The collected data included age at diagnosis, anatomical location of uveitis, associated systemic disease, used medications, and outcomes. All cases had been diagnosed and assessed mutually by a rheumatologist and an ophthalmologist using the International Uveitis Study Group classification system to classify the pattern of uveitis by location, clinical course, and laterality and rule out the possibility of other ophthalmologic diseases. Disease activity was defined using the Standardization of Uveitis Nomenclature (SUN) guidelines. Data was analyzed on SPSS Statistics version 23 (IBM Corp, Armonk, NY, USA)., Results: The mean age of the patients in this study was 36.02 ± 43.31 years, with 31 (59.6%) male patients. Anterior uveitis was the most common type observed among the patients at 55.8%, panuveitis was found in 25%, intermediate uveitis and posterior uveitis were seen in 9.6% each. Based on laterality, unilateral eye involvement was identified in 53.8% of patients. Spondyloarthritis (SpA) and idiopathic uveitis were observed in 34.6% and 28.8%, respectively. In this study, 28 (54.9%) patients were on conventional disease-modifying antirheumatic drugs (cDMARDDs), and 23 (45.1%) were on biological DMARDs. In the biologics group, 82% of patients were in remission in comparison to 60% in the cDMARDs group., Conclusion: To the best of our knowledge, this is the first report on noninfectious uveitis in the Pakistani population. The study concluded that anterior uveitis is the most common type of uveitis and is more common in males. Spondyloarthropathy is one of the most common underlying systemic diseases. Human leukocyte antigen (HLA)-B27 is associated more with uveitis. Biologics are more effective than cDMARDs in controlling the disease. Collaborative work between different specialties resulted in early diagnosis of underlying systemic disease, better management plans, and disease outcomes. To obtain further details on noninfectious uveitis, a population-based study is needed in Pakistan., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Younus et al.)

Objective: To analyze the prevalence of uveitis in patients treated at a tertiary ophthalmic service in Teresina, Piauí State, Brazil. Methods: This was a retrospective study based on medical records of patients treated at this service. Sex, age, origin, underlying diseases, anatomical diagnosis, clinical aspects and progression time were described. Results: 403 records were included, noting that 3.2 out of every 1,000 patients treated at the service had uveitis. The average age of patients was 42 years old, with both sexes (50.6% females and 49.4% males) affected similarly, and with 61.5% coming from the state capital, Teresina. The most common underlying disease was toxoplasmosis, followed by idiopathic uveitis with ocular manifestations only. As to the anatomical classification, 49.6% of them were posterior and only 3.5% were intermediate. Regarding the clinical aspects, 64% were granulomatous, 24.8% non-granulomatous, and 11.2% were not classifiable. According to the clinical progression, 41.4% were acute, followed by 30.8% chronic, 14.4% recurrent cases, and 13.4% of patients did not return for medical care. Conclusion: we found that for every 1,000 patients treated at the service, 3.2 had uveitis, with toxoplasmosis itself found to be an important source of uveitis. [ABSTRACT FROM AUTHOR]

Methods: This is a cross-sectional cohort study of patients with uveitis enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRAnet) registry. Clinical information including, demographic information, presenting features, disease complications, and medications were collected. Chi-square and Fisher's exact tests were used to assess for associations between medications and clinical characteristics. Results: Ninety-two children with idiopathic and 656 with juvenile idiopathic arthritis (JIA)-associated uveitis were identified. Indication (arthritis or uveitis) for medication use was not available for JIA patients; therefore, detailed analysis was limited to children with idiopathic uveitis. In this group, 94 % had received systemic steroids. Methotrexate (MTX) was used in 76 % of patients, with oral and subcutaneous forms given at similar rates. In multivariable analysis, non-Caucasians were more likely to be treated initially with subcutaneous MTX (P = 0.003). Of the 53 % of patients treated with a biologic DMARD, all received a tumor necrosis factor (TNF) inhibitor. TNF inhibitor use was associated with a higher frequency of cataracts (52 % vs 21 %; P = 0.001) and antinuclear antibody positivity (49 % vs 29 %; P = 0.04), although overall complication rates were not higher in these patients. Conclusion: Among idiopathic uveitis patients enrolled in the CARRAnet registry, MTX was the most commonly used DMARD, with subcutaneous and oral forms equally favored. Patients who received a TNF inhibitor were more likely to be ANA positive and have cataracts. [ABSTRACT FROM AUTHOR]

We created a registry to evaluate long term outcome, efficacy and adverse events for children treated wit TNF-α inhibitors in Switzerland. 106 patients (68 female/38 male) were included. 61 patients were treated with Etanercept (Enbrel®) and 45 with Infliximab (Remicade®). Concomitant treatment at baseline included corticosteroids in 26% and Methotrexate in 75% of the patients. Subjective disease activity three months after initiation of TNF-α was better in 81%, worse in 4% and stable in 15% of the patients. In total 24 adverse events in 21 patients were reported. Treatment with TNF-α inhibitors seems to be safe and effective for children and adolescents with rheumatologic diseases. [ABSTRACT FROM AUTHOR]

Uveitis is a clinical feature of the Blau syndrome, a disease linked to CARD15 (also referred to as NOD2) mutations. Three main mutations in this gene (R334W, R334Q and L469F) have been reported as Blau syndrome risk factors, a disease that manifests uveitis as one of its clinical features. However, little is known on the involvement of this gene in idiopathic uveitis. We thus sought to determine the frequency of these Blau-related CARD15 mutations in a cohort of Spanish patients with idiopathic uveitis. To this aim, 110 patients with idiopathic uveitis, followed at the Department of Ophtalmology of a tertiary hospital (Hospital Universitario Alcalá de Henares, Madrid. Spain) were enrolled. As a control population, 104 healthy subjects were used. DNA was extracted from blood samples and the Blau-related CARD15 mutations were analysed either by PCR-RFLP or direct DNA sequencing. None of the mutations studied was found in any of the individuals tested, whether diseased or healthy. It seems thus that Blau syndrome-related CARD15 mutations are not involved in idiopathic uveitis, a finding which allows us to suggest that the genetic aetiology of the idiopathic uveitis or the Blau-associated uveitis is different. [ABSTRACT FROM AUTHOR]

Retinal vasculitis is a major component of ocular inflammation that plays a role in retinal tissue damage in patients with idiopathic uveitis and Behçet's disease. Here we show that type 1 interferons (IFN α/β) were not detected in sera from normal individuals but were identified in up to 46% of the sera from retinal vasculitis patients. The predominant form of IFN observed was IFN-β, which was detected in 39% of Behçet's disease patients and 47% of idiopathic uveitis patients. Seven patients whose sera contained IFN-β were monitored prospectively. IFN-β was shown to be present for 6–12 months in all seven of the sera samples tested. Furthermore, the adhesion molecule profile identified in this study was strikingly different when Behçet's and uveitis patient sera were compared to sera from normal controls. Sera from Behçet's disease patients contained significantly elevated levels of the soluble adhesion molecules, sE-selectin and s-intracellular adhesion molecule-1 (sICAM-1), whereas sera from patients with idiopathic uveitis contained significantly increased sE-selectin. In vitro studies evaluating the cell source of these cytokines revealed that polyriboinosinic polyribocytidylic acid (poly I:C) activated retinal vascular endothelial cells produce sE-selectin, sICAM-1 and IFN-β. Production of these molecules was inhibited by pretreatment with anti-Toll-like receptor 3 (TLR-3) antibody. In conclusion, IFN-β, sE-selectin and sICAM-1 are elevated in patients with retinal vasculitis and are induced in retinal vascular endothelial cells in vitro by activating the innate immune system through TLR-3. Further analysis of innate immune signalling may prove to be a novel target for future studies on pathogenic mechanisms and therapeutic approaches in retinal vasculitis. [ABSTRACT FROM AUTHOR]

Objectives This study aims to describe the clinical, etiological, and treatment features of noninfectious uveitis in Jordanian children in a single center. Methods A retrospective, observational analysis of medical records of pediatric patients who were diagnosed with noninfectious uveitis from 2015 to 2020 at pediatric rheumatology and ophthalmology clinics at Queen Rania Children's Hospital, Amman, Jordan, was conducted. All patients were below 14 years of age at diagnosis. The collected data included age at diagnosis, anatomical location of uveitis, laterality, associated systemic disease, and used medications. Results Overall, 96 patients were included in this cohort (41 males and 55 females), with a mean age at diagnosis of 8.4±2.4 years. Anterior uveitis (44.8%) was the commonest anatomical location. Based on laterality, bilateral uveitis was reported in 59.3% of all patients. Idiopathic uveitis (46.9%) and juvenile idiopathic arthritis-associated uveitis (JIAU) (35.5%) were the most common diagnoses. Of the children with idiopathic uveitis, 47% had panuveitis, while 61.7% of the children with JIAU had chronic anterior uveitis. Posterior synechiae were the most common complication (12.5%). Patients with refractory uveitis received infliximab (29.1%) and adalimumab (4.1%). Conclusion To the best of our knowledge, this is the first report on noninfectious uveitis in Jordanian children. Compared with other regional and international published reports, JIAU and idiopathic uveitis were the most common diagnoses. To obtain more details on noninfectious uveitis characteristics, a population-based rather than a single-center study is needed in Jordan., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Alzyoud et al.)