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Indian Journal of Ophthalmology Jun 2024A 33-year-old male presented with unilateral painless vision loss with a history of sub-tenon steroid for the same. The fundus showed an elevated focus of...
A 33-year-old male presented with unilateral painless vision loss with a history of sub-tenon steroid for the same. The fundus showed an elevated focus of retinochoroiditis with vitritis. On investigating for the cause, polymerase chain reaction test on the anterior chamber tap was found to be positive for Toxoplasma. Such confusing and atypical cases usually produce a clinical dilemma and should be managed in a stepwise manner. Ancillary investigations usually provide a clue to the clinician and should be performed without any hesitation.
Topics: Humans; Male; Adult; Toxoplasmosis, Ocular; Toxoplasma; Polymerase Chain Reaction; Chorioretinitis; Fundus Oculi; Eye Infections, Parasitic; DNA, Protozoan; Diagnosis, Differential; Fluorescein Angiography
PubMed: 38804796
DOI: 10.4103/IJO.IJO_3341_23 -
Cureus Apr 2024The aim of this study was to explore the patterns of pediatric uveitis and the types of ocular complications of uveitis and to determine the possible risk factors...
AIM
The aim of this study was to explore the patterns of pediatric uveitis and the types of ocular complications of uveitis and to determine the possible risk factors associated with visual impairment.
METHOD
This was a cross-sectional study conducted at Queen Rania Children's Hospital between June 2020 and June 2023. All children diagnosed with uveitis were enrolled in the study. After collecting data from the patients and reviewing their medical records regarding age, gender, and past ocular and medical history, the patients were subjected to a detailed ophthalmic exam including best-corrected visual acuity (BCVA). Anterior segment exam using the slit lamp, intraocular pressure exam using Goldmann applanation tonometry, and posterior segment exam using 78 and 90 diopter Volk lenses were performed. Patients with other ocular diseases that affected visions not related to uveitis were excluded from the study.
RESULTS
A total of 82 children, accounting for 130 eyes, were enrolled in this study, with ages ranging from 2 to 16 years (mean age 10.5±4.3 years). Among them, 27 were males, constituting 32.9% of the participants. Unilateral uveitis was observed in 34 eyes, representing 26.2% of cases. The mean age of uveitis onset was 6.9±1.9 years, and the mean disease duration was 4.8±0.4 years. The majority of cases i.e. 90.8% (n = 74) were non-infectious, with 92.3% (n = 76) classified as non-granulomatous and 79.2% (n = 65) categorized as chronic. Anterior uveitis was the most prevalent site of inflammation in 70.8% of cases (n = 58), followed by panuveitis in 20.0% of cases (n = 16), intermediate uveitis in 6.2% of cases (n = 5), and posterior uveitis in 3.0% of cases (n = 2). The cause of uveitis could not be identified in 40.0% (n = 33) of cases. Juvenile idiopathic uveitis emerged as the most commonly known disorder associated with uveitis in 40.0% (n = 33) of cases. Complications were identified in 52.3% (n = 43) of cases, with posterior synechiae being the most prevalent; 26.9% (n = 22) demonstrated an improvement in BCVA, while 21.5% (n = 18) experienced a decline in BCVA relative to the initial assessment Conclusion: Pediatric uveitis tends to manifest as anterior, chronic, bilateral, and non-granulomatous. Higher frequencies of severe visual impairment are linked to panuveitis, infectious and granulomatous uveitis, early-onset, long-duration cases, and male gender. The use of biologics has a positive effect, significantly improving or preserving visual acuity.
PubMed: 38803751
DOI: 10.7759/cureus.59136 -
Frontiers in Immunology 2024Inflammation has been associated with an increased risk for cancer development, while innate immune system activation could counteract the risk for malignancies....
Risk for cancer development in familial Mediterranean fever and associated predisposing factors: an ambidirectional cohort study from the international AIDA Network registries.
OBJECTIVE
Inflammation has been associated with an increased risk for cancer development, while innate immune system activation could counteract the risk for malignancies. Familial Mediterranean fever (FMF) is a severe systemic inflammatory condition and also represents the archetype of innate immunity deregulation. Therefore, the aim of this study is to investigate the risk for cancer development in FMF.
METHODS
The risk ratio (RR) for malignancies was separately compared between FMF patients and fibromyalgia subjects, Still's disease patients and Behçet's disease patients. Clinical variables associated with cancer development in FMF patients were searched through binary logistic regression.
RESULTS
580 FMF patients and 102 fibromyalgia subjects, 1012 Behçet's disease patients and 497 Still's disease patients were enrolled. The RR for the occurrence of malignant neoplasms was 0.26 (95% Confidence Interval [CI.] 0.10-0.73, p=0.006) in patients with FMF compared to fibromyalgia subjects; the RR for the occurrence of malignant cancer was 0.51 (95% CI. 0.23-1.16, =0.10) in FMF compared to Still's disease and 0.60 (95% CI. 0.29-1.28, =0.18) in FMF compared to Behçet's disease. At logistic regression, the risk of occurrence of malignant neoplasms in FMF patients was associated with the age at disease onset (β1 = 0.039, 95% CI. 0.001-0.071, =0.02), the age at the diagnosis (β1 = 0.048, 95% CI. 0.039-0.085, =0.006), the age at the enrolment (β1 = 0.05, 95% CI. 0.007-0.068, =0.01), the number of attacks per year (β1 = 0.011, 95% CI. 0.001- 0.019, =0.008), the use of biotechnological agents (β1 = 1.77, 95% CI. 0.43-3.19, =0.009), the use of anti-IL-1 agents (β1 = 2.089, 95% CI. 0.7-3.5, =0.002).
CONCLUSIONS
The risk for cancer is reduced in Caucasic FMF patients; however, when malignant neoplasms occur, this is more frequent in FMF cases suffering from a severe disease phenotype and presenting a colchicine-resistant disease.
Topics: Humans; Familial Mediterranean Fever; Neoplasms; Male; Female; Adult; Registries; Middle Aged; Risk Factors; Cohort Studies; Young Adult; Fibromyalgia; Behcet Syndrome
PubMed: 38799474
DOI: 10.3389/fimmu.2024.1397890 -
American Journal of Ophthalmology Case... Sep 2024To describe ocular sarcoid-like reaction as a unique manifestation of paraneoplastic syndrome in the context of concurrent pulmonary sarcoid-like reaction and lung...
PURPOSE
To describe ocular sarcoid-like reaction as a unique manifestation of paraneoplastic syndrome in the context of concurrent pulmonary sarcoid-like reaction and lung adenocarcinoma.
METHODS
Single case report and narrative review.
RESULTS
A 59-year-old male patient presented with a year-long history of diminished vision and weight loss. Clinical examination revealed panuveitis and multiple chorioretinal lesions. A CT scan of the chest revealed mediastinal and hilar lymphadenopathy as well as a spiculated right lower lung nodule concerning for malignancy. Subsequent bronchoscopy and biopsy confirmed lung adenocarcinoma and non-caseating granulomas in sentinel lymph nodes.
CONCLUSION
Although pulmonary granulomatous reaction can be seen in the setting of lung malignancy, and ocular sarcoid-like reaction may present as a paraneoplastic manifestation of systemic malignancy, the presence of concomitant pulmonary and ocular sarcoid-like reactions distinguishes this case. The findings underscore the importance of a systemic workup for patients with concerning constitutional symptoms, as paraneoplastic syndromes and metastatic diseases may mimic uveitis. Recognition of paraneoplastic sarcoidosis as a potential clinical manifestation is essential, especially in patients with chronic illness indicators, necessitating a comprehensive evaluation for malignancy.
PubMed: 38799225
DOI: 10.1016/j.ajoc.2024.102076 -
Biomolecules Apr 2024Fundus autofluorescence (FAF) is a prompt and non-invasive imaging modality helpful in detecting pathological abnormalities within the retina and the choroid. This... (Review)
Review
Fundus autofluorescence (FAF) is a prompt and non-invasive imaging modality helpful in detecting pathological abnormalities within the retina and the choroid. This narrative review and case series provides an overview on the current application of FAF in posterior and panuveitis. The literature was reviewed for articles on lesion characteristics on FAF of specific posterior and panuveitis entities as well as benefits and limitations of FAF for diagnosing and monitoring disease. FAF characteristics are described for non-infectious and infectious uveitis forms as well as masquerade syndromes. Dependent on the uveitis entity, FAF is of diagnostic value in detecting disease and following the clinical course. Currently available FAF modalities which differ in excitation wavelengths can provide different pathological insights depending on disease entity and activity. Further studies on the comparison of FAF modalities and their individual value for uveitis diagnosis and monitoring are warranted.
Topics: Humans; Panuveitis; Fundus Oculi; Optical Imaging; Fluorescein Angiography
PubMed: 38785922
DOI: 10.3390/biom14050515 -
Journal of Cellular and Molecular... May 2024Behçet's disease (BD) is a complex autoimmune disorder impacting several organ systems. Although the involvement of abdominal aortic aneurysm (AAA) in BD is rare, it...
Behçet's disease (BD) is a complex autoimmune disorder impacting several organ systems. Although the involvement of abdominal aortic aneurysm (AAA) in BD is rare, it can be associated with severe consequences. In the present study, we identified diagnostic biomarkers in patients with BD having AAA. Mendelian randomization (MR) analysis was initially used to explore the potential causal association between BD and AAA. The Limma package, WGCNA, PPI and machine learning algorithms were employed to identify potential diagnostic genes. A receiver operating characteristic curve (ROC) for the nomogram was constructed to ascertain the diagnostic value of AAA in patients with BD. Finally, immune cell infiltration analyses and single-sample gene set enrichment analysis (ssGSEA) were conducted. The MR analysis indicated a suggestive association between BD and the risk of AAA (odds ratio [OR]: 1.0384, 95% confidence interval [CI]: 1.0081-1.0696, p = 0.0126). Three hub genes (CD247, CD2 and CCR7) were identified using the integrated bioinformatics analyses, which were subsequently utilised to construct a nomogram (area under the curve [AUC]: 0.982, 95% CI: 0.944-1.000). Finally, the immune cell infiltration assay revealed that dysregulation immune cells were positively correlated with the three hub genes. Our MR analyses revealed a higher susceptibility of patients with BD to AAA. We used a systematic approach to identify three potential hub genes (CD247, CD2 and CCR7) and developed a nomogram to assist in the diagnosis of AAA among patients with BD. In addition, immune cell infiltration analysis indicated the dysregulation in immune cell proportions.
Topics: Humans; Behcet Syndrome; Aortic Aneurysm, Abdominal; Mendelian Randomization Analysis; Computational Biology; Biomarkers; ROC Curve; Gene Regulatory Networks; Genetic Predisposition to Disease; Protein Interaction Maps; Nomograms; Receptors, CCR7
PubMed: 38785203
DOI: 10.1111/jcmm.18398 -
Journal of Neuroinflammation May 2024Behcet's disease (BD) is a rare but globally distributed vasculitis that primarily affects populations in the Mediterranean and Asian regions. Behcet's uveitis (BU) is a... (Review)
Review
Behcet's disease (BD) is a rare but globally distributed vasculitis that primarily affects populations in the Mediterranean and Asian regions. Behcet's uveitis (BU) is a common manifestation of BD, occurring in over two-thirds of the patients. BU is characterized by bilateral, chronic, recurrent, non-granulomatous uveitis in association with complications such as retinal ischemia and atrophy, optic atrophy, macular ischemia, macular edema, and further neovascular complications (vitreous hemorrhage, neovascular glaucoma). Although the etiology and pathogenesis of BU remain unclear, numerous studies reveal that genetic factors (such as HLA-B51), dysregulated immune responses of both the innate and adaptive immune systems, infections (such as streptococcus), and environmental factors (such as GDP) are all involved in its development. Innate immunity, including hyperactivity of neutrophils and γδT cells and elevated NK1/NK2 ratios, has been shown to play an essential role in this disease. Adaptive immune system disturbance, including homeostatic perturbations, Th1, Th17 overaction, and Treg cell dysfunction, is thought to be involved in BU pathogenesis. Treatment of BU requires a tailored approach based on the location, severity of inflammation, and systemic manifestations. The therapy aims to achieve rapid inflammation suppression, preservation of vision, and prevention of recurrence. Systemic corticosteroids combined with other immunosuppressive agents have been widely used to treat BU, and beneficial effects are observed in most patients. Recently, biologics have been shown to be effective in treating refractory BU cases. Novel therapeutic targets for treating BU include the LCK gene, Th17/Treg balance, JAK pathway inhibition, and cytokines such as IL-17 and RORγt. This article summarizes the recent studies on BU, especially in terms of pathogenesis, diagnostic criteria and classification, auxiliary examination, and treatment options. A better understanding of the significance of microbiome composition, genetic basis, and persistent immune mechanisms, as well as advancements in identifying new biomarkers and implementing objective quantitative detection of BU, may greatly contribute to improving the adequate management of BU patients.
Topics: Humans; Behcet Syndrome; Uveitis; Animals
PubMed: 38778397
DOI: 10.1186/s12974-024-03123-6 -
Cureus Apr 2024Uveitis is the inflammation of the uveal tract (i.e., iris, ciliary body, and choroid). Uveitis is categorized into the following three types based on the anatomical...
Uveitis is the inflammation of the uveal tract (i.e., iris, ciliary body, and choroid). Uveitis is categorized into the following three types based on the anatomical location of inflammation: anterior, intermediate, and posterior uveitis. Severe cases may lead to panuveitis, where all three layers may become inflamed potentially resulting in permanent vision loss. Uveitis can arise from different underlying disorders, including infectious causes or autoimmune disorders. Syphilis and Lyme disease are uncommon causes of uveitis. Eye involvement can occur at any stage in Lyme disease, characterized by diverse manifestations such as conjunctivitis, episcleritis, keratitis, uveitis, neuroretinitis, and retinal vasculitis. Patients may present with symptoms of blurred vision, eye pain or discomfort, visual floaters, headache, or intolerance to light. Patients can risk vision loss if not diagnosed and treated promptly.
PubMed: 38765401
DOI: 10.7759/cureus.58608 -
Arquivos Brasileiros de Oftalmologia 2024Sympathetic ophthalmia is a rare and potentially devastating bilateral diffuse granulomatous panuveitis. It is caused by surgical or non-surgical eye injuries and is an... (Review)
Review
Sympathetic ophthalmia is a rare and potentially devastating bilateral diffuse granulomatous panuveitis. It is caused by surgical or non-surgical eye injuries and is an uncommon and serious complication of trauma. It is diagnosed clinically and supported by imaging examinations such as ocular ultrasonography and optical coherence tomography. Its treatment consists of immunosuppressive therapy with steroids and sometimes steroid-sparing drugs, such as cyclosporine, azathioprine, cyclophosphamide, and mycophenolate mofetil. Fast and effective management with systemic immunosuppressive agents allows for disease control and achievement of good visual acuity in the sympathizing eye. By contrast, enucleation should be considered only in situations where the injured eye has no light perception or in the presence of severe trauma. In addition to a bibliographic review of this topic, we report six cases involving different immunosuppressive and surgical treatment modalities.
Topics: Humans; Ophthalmia, Sympathetic; Immunosuppressive Agents; Male; Female; Adult; Middle Aged; T-Lymphocytes; Tomography, Optical Coherence; Visual Acuity
PubMed: 38747753
DOI: 10.5935/0004-2749.2022-0142 -
The Israel Medical Association Journal... May 2024
Topics: Humans; Behcet Syndrome; Israel; Arabs; Early Diagnosis
PubMed: 38736352
DOI: No ID Found