Humans ; Ulcer ; Intestinal Diseases ; Myelodysplastic Syndromes/genetics* ; Trisomy/genetics* ; Behcet Syndrome

Objective: To retrospectively investigate the clinical data, radiological characteristics, treatment, and outcome of patients with parenchymal neuro-Behcet's disease (P-NBD) with particular emphasis on dizziness. Methods: This was a cross-sectional study of clinical data from 25 patients with a confirmed diagnosis of P-NBD who were admitted to the Department of Neurology of the First Medical Center of Chinese People's Liberation Army General Hospital between 2010 and 2022. The median age of the population was 37 years (range: 17-85 years). Clinical data were retrospectively analyzed, including gender, age of onset, disease duration, clinical manifestations, serum immune indicators, cerebrospinal fluid (CSF) routine biochemical and cytokine levels, cranial and spinal magnetic resonance imaging (MRI) findings, treatment, and outcome. Results: The majority of patients were male (16 cases; 64.0%), the mean age of onset was (28±14) (range: 4-58 years), and the disease course was acute or subacute. Fever was the most common clinical presentation, and the complaint of dizziness was not uncommon (8/25 patients). Analysis of serum immune indices, including complement (C3 and C4), erythrocyte sedimentation rate, interleukin-1 (IL-1), IL-6, IL-8 and tumor necrotic factor-alpha were abnormal in 80.0% of patients (20/25). Most of the 16/25 patients who underwent lumbar puncture tests had normal intracranial pressure and increased CSF white cell count and protein [median values were 44 (15-380) ×10⁶/L and 0.73 (0.49-2.81) g/L, respectively]. Of the five patients who underwent CSF cytokine tests, four patients had abnormal results; of these, an elevated level of IL-6 was most common, followed by IL-1 and IL-8. The most common site of involvement in cranial MRI was the brainstem and basal ganglia (60.0% respectively), followed by white matter (48.0%) and the cortex (44.0%). Nine cases (36.0%) showed lesions with enhancement and six cases (24.0%) showed mass-like lesions. Three patients (12.0%) patients had lesions in the spinal cord, most frequently in the thoracic cord. All patients received immunological intervention therapy; during follow up, the majority had a favorable outcome. Conclusions: P-NBD is an autoimmune disease with multiple system involvement and diverse clinical manifestations. The symptom of dizziness is not uncommon and can be easily ignored. Early treatment with immunotherapy is important and can improve the outcome of these patients.
Humans ; Male ; Female ; Child, Preschool ; Child ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Aged ; Aged, 80 and over ; Behcet Syndrome/diagnosis* ; Interleukin-6 ; Retrospective Studies ; Cross-Sectional Studies ; Interleukin-8 ; Magnetic Resonance Imaging ; Neurology

Aging-induced changes in the immune system are associated with a higher incidence of infection and vaccination failure. Lymph nodes, which filter the lymph to identify and fight infections, play a central role in this process. However, careful characterization of the impact of aging on lymph nodes and associated autoimmune diseases is lacking. We combined single-cell RNA sequencing (scRNA-seq) with flow cytometry to delineate the immune cell atlas of cervical draining lymph nodes (CDLNs) of both young and old mice with or without experimental autoimmune uveitis (EAU). We found extensive and complicated changes in the cellular constituents of CDLNs during aging. When confronted with autoimmune challenges, old mice developed milder EAU compared to young mice. Within this EAU process, we highlighted that the pathogenicity of T helper 17 cells (Th17) was dampened, as shown by reduced GM-CSF secretion in old mice. The mitigated secretion of GM-CSF contributed to alleviation of IL-23 secretion by antigen-presenting cells (APCs) and may, in turn, weaken APCs' effects on facilitating the pathogenicity of Th17 cells. Meanwhile, our study further unveiled that aging downregulated GM-CSF secretion through reducing both the transcript and protein levels of IL-23R in Th17 cells from CDLNs. Overall, aging altered immune cell responses, especially through toning down Th17 cells, counteracting EAU challenge in old mice.
Aging ; Animals ; Autoimmune Diseases ; Disease Models, Animal ; Granulocyte-Macrophage Colony-Stimulating Factor/metabolism* ; Mice ; Mice, Inbred C57BL ; Th17 Cells/metabolism* ; Uveitis/pathology* ; Virulence

Humans ; Heart Ventricles ; Behcet Syndrome/complications* ; Heart ; Heart Diseases ; Ventricular Dysfunction, Left

Humans ; Behcet Syndrome/diagnostic imaging*

Behçet's disease (BD) is a systemic vasculitis characterized by recurrent acute vascular inflammation. There is currently limited epidemiological data in Southeast Asia. We discuss a 26-year-old woman who came to our institution for recurrent vulvar abscesses for a year. Physical examination showed labial, tongue, and vulvar ulcers. Pathergy test was negative and biopsy done were unremarkable . We diagnosed her clinically as a case of Behçet’s disease. We started her on colchicine 0.6 mg once daily and educated her on skin, oral, and perineal hygiene. On follow-up after one month, she noted resolution of her lesions. However, symptoms recurred in the last three months after discontinuing treatment. We used telemedicine to communicate due to the COVID-19 pandemic and lockdown, advising her to continue her medication. Timely diagnosis is essential in order to provide symptomatic relief and enhance patient education.
Behcet Syndrome ; Ulcer ; Vasculitis

OBJECTIVE: To evaluate the value of fundus autofluorescence (FAF) imaging combined with spectral domain optical coherence tomography (SD-OCT) in diagnosis, prognostic assessment and follow-up observation of acute Vogt-KoyanagiHarada (VKH) disease. METHODS: Clinical data were collected from 12 patients (23 eyes) with acute VKH disease treated in our hospital from May, 2018 to November, 2019, including detailed medical history, best corrected visual acuity (BCVA), and results of slit lamp biomicroscopy, fundus photography, SD-OCT, fundus fluorescein angiography (FFA) and FAF imaging.SDOCT and FAF imaging were repeated after a course of treatment and in follow-up examination, and the results were compared with those at the time of admission. RESULTS: VKH disease involved both eyes in 11 patients (91.7%).Fundus photography showed optic disc edema in 16 eyes (69.6%), and multiple retinal neuroepithelial detachment was detected by SD-OCT in all the involved eyes (100%).IN all the eyes, FFA revealed small and dense fluorescein leakage in the early stage and fluorescein accumulation in advanced stages of VHK disease to form multiple dye pooling in the areas of serous detachment.Hyperauto fluorescence was a common finding in FAF imaging (100%), and the area involved was consistent with that of fluorescein accumulation shown by FAF imaging.Ten eyes (43.5%) showed patches of relative hypoautofluorescence in the hyperauto fl uorescence areas, and granular hyperauto fl uorescence was found in the lesions in 4 eyes (17.4%).During the remission period of VKH disease, FAF imaging showed normal finding in 8 eyes (34.8%) and reduced areas (by 55.2%) and intensity (by 46.5%) of hyperautofluorescence in 9 eyes (39.1%).In 6 eyes (26.1%), only a few hyperautofluorescent spots scattered in the macula were observed.SD-OCT demonstrated significantly reduced (by 69.5% on average) or even disappearance of subretinal fluid in the eyes.The fluorescence intensity in FAF imaging showed a significant positive correlation with the volume of subretinal fluid detected by SD-OCT ( CONCLUSIONS The combination of fluorescein angiography, FAF imaging and SD-OCT can significantly improve the diagnostic accuracy of VKH disease.FAF imaging combined with SD-OCT provides an effective and noninvasive modality for evaluation of remission and monitoring the changes in VKH disease.
Acute Disease ; Fluorescein Angiography ; Follow-Up Studies ; Humans ; Retinal Detachment/diagnostic imaging* ; Tomography, Optical Coherence ; Uveomeningoencephalitic Syndrome/diagnostic imaging*

Humans ; Panuveitis/etiology* ; Retinal Detachment/etiology* ; Typhoid Fever/diagnosis* ; Visual Acuity

OBJECTIVE: This study explored the correlations between the use of complementary and integrative therapies (CITs) and symptoms among Turkish patients with familial Mediterranean fever (FMF). METHODS: This is a cross-sectional and descriptive study. The study was conducted with 1119 FMF patients who were registered to the social networking site for Behcet's and the FMF Patients Association (Befemder) in Turkey, between January 2018 and February 2019. Data were collected using an online survey, for which a three-part questionnaire was created using a Google form. Descriptive statistics, chi-square test and logistic regression analysis were used to analyze the data. RESULTS: It was determined that 53.2% of the individuals who participated in the research used various forms of CITs and that 32.8% used vitamin and mineral supplements (calcium, iron, and vitamin B12, C and D), 25.0% used nutritional supplements (fish oil and honey), and 24.6% used oral herbs (ginger, turmeric, green tea and rosemary) and mind-body methods (relaxation, respiration exercise and meditation). It was determined that the percentage of participants that used CITs was higher among women (odds ratio [OR] = 1.825; 95% confidence interval [CI] 1.421-2.344), those with joint pain (OR = 1.385; 95% CI 1.047-1.832), those with difficulty breathing (OR = 1.323; 95% CI 1.031-1.697), those with gastrointestinal symptoms (OR = 1.405; 95% CI 1.089-1.814) and those who had a family member with FMF (OR = 1.437; 95% CI 1.115-1.851). CONCLUSION More than half of the individuals used at least one type of CIT for symptom control.
Behcet Syndrome ; Cross-Sectional Studies ; Familial Mediterranean Fever/therapy* ; Female ; Humans ; Surveys and Questionnaires ; Turkey

This paper provides the summaries on nine (9) important and clinically relevant publications in the field of uveitis. The first is on the standardization of uveitis nomenclature, more popularly known by its acronym - SUN, which was a result of an international workshop participated by uveitis experts in 2004. Five (5) papers were large, multicenter, clinical trials that demonstrated safety and efficacy of two (2) corticosteroids delivery devices (dexamethasone implant [Ozurdex] and fluocinolone acetonide implant [RetisertTM]) and one (1) immunomodulatory drug (adalimumab). The POINT trial compared various delivery approaches when using corticosteroids for the treatment of uveitic macular edema. The FAST trial compared two (2) durable and commonly-prescribed steroidsparing immunosuppressants, methotrexate and mycophenolate mofetil, for the treatment of non-infectious uveitis. Lastly, the SITE study, which was a large retrospective cohort study, determined the risks of overall and malignancy-related deaths among patients with inflammatory eye diseases receiving systemic immunosuppressants. Findings of these studies provide basis and rationale for the care and management of patients with uveitis and lay the groundwork for future research.
Uveitis ; Clinical Trial ; Review ; Adrenal Cortex Hormones