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Clinical Immunology (Orlando, Fla.) Aug 2023Behçet's disease (BD) is a multi-system inflammatory disorder with vasculitic features. It does not suit any of the current pathogenesis-driven disease classifications... (Review)
Review
Behçet's disease (BD) is a multi-system inflammatory disorder with vasculitic features. It does not suit any of the current pathogenesis-driven disease classifications well, a unifying concept of its pathogenesis is not unanimously conceivable at present, and its etiology is obscure. Still, evidence from immunogenetic and other studies supports the notion of a complex-polygenic disease with robust innate effector responses, reconstitution of regulatory T cells upon successful treatment, and first clues to the role of an, as of yet, underexplored adaptive immune system and its antigen recognition receptors. Without an attempt to be comprehensive, this review aims to collect and organize impactful parts of this evidence in a way that allows the reader to appreciate the work done and define the efforts needed now. The focus is on literature and notions that drove the field into new directions, whether recent or more remote.
Topics: Humans; Behcet Syndrome; HLA-B51 Antigen; T-Lymphocytes, Regulatory; HLA-B Antigens
PubMed: 37295542
DOI: 10.1016/j.clim.2023.109661 -
Current Opinion in Rheumatology Jan 2024Epidemiology of vasculitides exhibit geographic variation and data from some parts of the world are still scarce. Increased recognition of these rare diseases and... (Review)
Review
PURPOSE OF REVIEW
Epidemiology of vasculitides exhibit geographic variation and data from some parts of the world are still scarce. Increased recognition of these rare diseases and improvement in diagnosis and patient care may lead to changes in their epidemiology. In this review, we aimed to highlight the most recent work on the epidemiology of systemic vasculitis.
RECENT FINDINGS
New data from countries where information on the epidemiology of giant cell arteritis, Takayasu arteritis and Behçet syndrome were limited have revealed that these conditions are not as rare as previously believed. The incidence rates during the coronavirus disease 2019 pandemic highlight the link between Kawasaki disease and respiratory pathogens. The use of different classification criteria hampers the comparison of true incidence and prevalence rates in antineutophil cytoplasmic antibody (ANCA)-associated vasculitis and its subtypes between geographies and over time.
SUMMARY
Recent studies have highlighted the epidemiology of vasculitides in different parts of the world and changing trends. Standardization of study design and disease definitions is needed to improve the reliability and comparability of the results.
Topics: Humans; Reproducibility of Results; Systemic Vasculitis; Mucocutaneous Lymph Node Syndrome; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Giant Cell Arteritis; Behcet Syndrome; Takayasu Arteritis
PubMed: 37800639
DOI: 10.1097/BOR.0000000000000983 -
Ocular Immunology and Inflammation Sep 2023To report and illustrate the main clinical presentations of posterior herpetic uveitis. (Review)
Review
PURPOSE
To report and illustrate the main clinical presentations of posterior herpetic uveitis.
METHODS
Narrative review.
RESULTS
The ocular manifestations of posterior herpetic uveitis include different clinical presentations. Herpes simplex and varicella zoster can cause acute retinal necrosis, progressive outer retinal necrosis, and non-necrotizing herpetic retinopathies. Cytomegalovirus has been associated with fulminant retinitis with confluent areas of retinal necrosis and retinal hemorrhages, indolent/granular retinitis, and frosted branch angiitis. These diverse clinical presentations are often associated with specific risk factors and different immunological profiles of the host.
CONCLUSIONS
Herpetic viruses can cause posterior uveitis, presenting various clinical findings. Specific ocular manifestations and the immunological status of the host can help to differentiate the various herpetic entities before laboratory tests confirm the diagnosis.
Topics: Humans; Herpesviridae Infections; Retinal Necrosis Syndrome, Acute; Retinitis; Retinal Diseases; Uveitis, Posterior; Necrosis
PubMed: 37364039
DOI: 10.1080/09273948.2023.2221338 -
Ophthalmology Dec 2023
Topics: Humans; Suppuration; Uveitis, Anterior; Eye Abnormalities
PubMed: 36849294
DOI: 10.1016/j.ophtha.2023.01.018 -
Ugeskrift For Laeger Nov 2023This is a case report of a 3-year-old boy who presented with unilateral anterior uveitis and tonic pupil following varicella-zoster virus (VZV) Infection. The patient...
This is a case report of a 3-year-old boy who presented with unilateral anterior uveitis and tonic pupil following varicella-zoster virus (VZV) Infection. The patient had red and irritated eyes and photophobia. Ophthalmological findings included anterior uveitis and tonic pupil accompanied by reduced vision and accommodation. An MRI of the cerebrum was normal. To ease the symptoms the patient was prescribed photophobia glasses with correction of hyperopia. Tonic pupil due to VZV infection is a rare complication, but may have long-term consequences, why patients with eye-involving VZV infection need to be examined by an ophthalmologist.
Topics: Male; Humans; Child; Child, Preschool; Chickenpox; Tonic Pupil; Photophobia; Herpesvirus 3, Human; Uveitis, Anterior; Acute Disease
PubMed: 38018730
DOI: No ID Found -
Graefe's Archive For Clinical and... Feb 2024To report the clinical and fluorescein angiographic (FA) features of demyelinating plaque-associated uveitis (DPU), a subset of uveitis in which patients have... (Observational Study)
Observational Study
PURPOSE
To report the clinical and fluorescein angiographic (FA) features of demyelinating plaque-associated uveitis (DPU), a subset of uveitis in which patients have demyelinating plaques on the brain/cervical magnetic resonance image (MRI) but do not meet the criteria for multiple sclerosis (MS).
METHODS
In this retrospective observational study, Persian Patients were diagnosed with DPU and included if (1) they never satisfied the MS criteria, (2) all other possible etiologies were excluded, and (3) they were followed for at least 2 years.
RESULTS
After a median follow-up of 3 years (interquartile range, 2.0-5.3), 8 out of 40 (20%) patients diagnosed with DPU were excluded as they subsequently met the MS criteria. Of remaining 32 patients studied, the mean age was 36.3±9.9 (range 20-56 years), and 30 (93.8%) were female. Twenty-four (75.0%) showed bilateral involvement and 27 (84.4%) had insidious-chronic course. Uveitis was classified as intermediate (with or without anterior uveitis) in 29 (90.6%) and isolated anterior in 3 (9.4%) patients. Nine (28.1%) patients had at least one systemic neurological complaint. Ocular findings were: granulomatous keratic precipitates in 43/44 (97.7%) eyes; snowballs in 25/52 (48.1%) eyes; snowbanks in 4/52 (7.7%) eyes; cystoid macular edema in 20/56 (35.7%) eyes; and optic neuritis in 5/56 (8.9%) eyes. Visual acuity was ≥ 20/40 in 39 eyes (69.6%) at presentation which improved to 46 eyes (81.2%) at 2-year follow up. The two most frequent findings in FA were optic disc leakage/staining in 44/52 (81.5%) eyes, and peripheral retinal perivascular leakage in 39/52 (76.9%) eyes, which in 14/52 (26.9%) eyes extended beyond the equator.
CONCLUSION
DPU usually presents as a bilateral chronic granulomatous intermediate and, less often, isolated anterior uveitis, especially in females. Most are neurologically asymptomatic. Visual outcome is generally favorable. In FA, peripheral retinal perivascular leakage is common. DPU patients have an increased tendency to develop MS and should be prohibited from anti-TNF treatment.
Topics: Humans; Female; Young Adult; Adult; Middle Aged; Male; Tumor Necrosis Factor Inhibitors; Uveitis; Uveitis, Anterior; Retina; Fluorescein Angiography; Retrospective Studies; Plaque, Atherosclerotic; Uveitis, Intermediate
PubMed: 37855958
DOI: 10.1007/s00417-023-06270-3 -
Clinical Immunology (Orlando, Fla.) Sep 2023
Topics: Humans; Behcet Syndrome
PubMed: 37517564
DOI: 10.1016/j.clim.2023.109711 -
The New England Journal of Medicine May 2024
Topics: Humans; Male; Behcet Syndrome; Immunosuppressive Agents; Thrombosis; Antibodies, Antiphospholipid; Anticoagulants
PubMed: 38718372
DOI: 10.1056/NEJMc2403905 -
The New England Journal of Medicine May 2024
Topics: Humans; Male; Behcet Syndrome; Immunosuppressive Agents; Inflammation; Thromboembolism; Antibodies, Antiphospholipid; Anticoagulants
PubMed: 38718373
DOI: 10.1056/NEJMc2403905 -
The New England Journal of Medicine May 2024
Topics: Humans; Male; Behcet Syndrome; Immunosuppressive Agents; Antibodies, Antiphospholipid; Thrombosis; Anticoagulants; Inflammation
PubMed: 38718371
DOI: 10.1056/NEJMc2403905