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Molecular Vision 2023Acute anterior uveitis (AAU) is the most common extra-articular symptom of ankylosing spondylitis (AS). This study aims to reveal the cytokines and chemokines involved...
PURPOSE
Acute anterior uveitis (AAU) is the most common extra-articular symptom of ankylosing spondylitis (AS). This study aims to reveal the cytokines and chemokines involved in the immunopathogenesis of human leucocyte antigen (HLA)-B27 AS-associated AAU.
METHODS
Twenty-one HLA-B27 AS-associated AAU patients and 21 healthy controls (HCs) were recruited for this study. Serum cytokine concentrations in all 42 subjects were determined by the Meso Scale Discovery (MSD) electrochemiluminescence method. In each sample, 34 cytokines, 10 chemokines, eight angiogenesis mediators, and four vascular injury mediators were measured. The differences in cytokine and chemokine concentrations were compared between the two groups.
RESULTS
Concentrations of serum IL-3, TNF-α, IL-6, IL-17D, IL-22, IP10/CXCL10, MIP-3α/CCL20, sFlt-1/VEGFR-1, CRP, and MCP-4/CCL13 were significantly higher in patients with HL-B27 AS-associated AAU than in HCs ( < 0.05). In contrast, concentrations of serum IL-4, IL-8, MIP-1α/CCL3, Eotaxin-3/CCL26, PlGF, VEGF-C, and VEGF-D were significantly lower in patients with HL-B27 AS-associated AAU than in HCs ( < 0.05).
CONCLUSIONS
Significant differences were detected in the levels of several cytokines and chemokines in the serum of HLA-B27 AS-associated AAU compared with HCs. Some novel differential cytokines and chemokines that have not been reported in other kinds of uveitis were also identified. These results reveal the underlying pathogenesis of HLA-B27 AS-associated AAU and could potentially aid in clinical diagnosis.
Topics: Humans; Cytokines; Spondylitis, Ankylosing; HLA-B27 Antigen; Uveitis, Anterior; Chemokines; Acute Disease
PubMed: 38577559
DOI: No ID Found -
Reumatismo Sep 2023Adenosine deaminase 2 deficiency (DADA2) is a rare monogenic vasculopathy caused by loss-of-function homozygous or compound heterozygous mutations in ADA2, formerly...
Adenosine deaminase 2 deficiency (DADA2) is a rare monogenic vasculopathy caused by loss-of-function homozygous or compound heterozygous mutations in ADA2, formerly CECR1 (cat eye syndrome chromosome region 1) gene. The DADA2 phenotype is widely heterogeneous, and patients may present with fever, weight loss, livedo reticularis/racemosa, digital ischemia, cutaneous ulceration, peripheral neuropathy, abdominal pain, bowel perforation, and portal or nephrogenic hypertension. More specific manifestations include early-onset ischemic or hemorrhagic stroke, mild immunodeficiency and hypogammaglobinemia, cytopenia, and vision disturbances. Herein, we present the case of a young male with vasculitis associated with DADA2. The presence of HLA-B51 and the clinical features of this patient raised the question of similarities between ADA2 deficiency, Behçet's disease, and NOD2-associated diseases. Treatment of this rare monogenic disease is challenging and based on small case series. The long-term experience of this patient proved the difficulties of prednisone tapering and the lack of satisfactory therapeutic strategies.
Topics: Humans; Male; Behcet Syndrome; Adenosine Deaminase; Intercellular Signaling Peptides and Proteins; Vasculitis
PubMed: 37721348
DOI: 10.4081/reumatismo.2023.1578 -
BMC Ophthalmology Apr 2024To determine the prevalence, clinical characteristics, and independent predictors of uveitic macular edema (UME) in patients with intermediate, posterior and panuveitis.
BACKGROUND
To determine the prevalence, clinical characteristics, and independent predictors of uveitic macular edema (UME) in patients with intermediate, posterior and panuveitis.
METHODS
We retrospectively reviewed the records of patients with intermediate, posterior, and panuveitis who underwent macular assessment using optical coherence tomography between January 2015 and February 2020. The prevalence of UME and clinical characteristics of the patients were described. Predictors of UME were identified using multivariate regression analysis.
RESULTS
A total of 349 patients were included. The mean age was 41 years, female: male ratio was 1.3:1. The prevalence of UME was 51.9%. UME was found in 33.9%, 56.9%, and 54.1% of the intermediate, posterior, and panuveitis cases, respectively. Among patients with UME, 47% had infectious uveitis, 32.6% had idiopathic uveitis, and 20.4% had immune-mediated uveitis. Diffuse macular edema was the most frequently observed pattern (36.5%). Multivariate analysis showed that factors independently associated with UME included age at uveitis onset (adjusted odds ratio [aOR] 1.01, 95% confidence interval [CI] 1.00-1.03, P = 0.036), PU and panuveitis compared with intermediate uveitis (aOR 2.09, 95% CI 1.14-3.86, P = 0.018), and infectious uveitis compared with noninfectious uveitis (aOR 2.13, 95% CI 1.34-3.37, P = 0.001).
CONCLUSIONS
Increasing age at uveitis onset, posterior/panuveitis, and infectious etiology are predictive factors for UME in patients with intermediate, posterior and panuveitis.
Topics: Humans; Retrospective Studies; Male; Female; Macular Edema; Adult; Prevalence; Tomography, Optical Coherence; Middle Aged; Uveitis; Visual Acuity; Risk Factors; Young Adult; Singapore
PubMed: 38649909
DOI: 10.1186/s12886-024-03447-0 -
Clinical and Translational... Aug 2023Behçet's disease (BD) is a chronic systemic vasculitis characterized by oral and genital ulcers, uveitis, and skin lesions. Patients with BD may develop...
INTRODUCTION
Behçet's disease (BD) is a chronic systemic vasculitis characterized by oral and genital ulcers, uveitis, and skin lesions. Patients with BD may develop gastrointestinal (GI) disease; however, characterization of GI disease in American cohorts is lacking. In this article, we present clinical, endoscopic, and histopathologic GI findings in an American cohort of patients with BD.
METHODS
Patients with established BD were evaluated prospectively at the National Institutes of Health. Demographic and clinical data were collected including BD manifestations and GI symptoms. Endoscopy with histopathologic sampling was performed for both clinical and research indications with written consent.
RESULTS
Eighty-three patients were evaluated. The majority were female (83.1%) and white (75.9%). Mean age was 36 ± 14.8 years. GI symptoms were reported in 75% of cohort with nearly half of reporting abdominal pain (48.2%) and significant numbers reporting acid reflux, diarrhea, and nausea/vomiting. Esophagogastroduodenoscopy was performed in 37 patients; erythema and ulcers were the most common found abnormalities. Colonoscopy was performed in 32 patients with abnormalities including polyps, erythema, and ulcers. Endoscopy was normal in 27% of esophagogastroduodenoscopies and 47% of colonoscopies. Vascular congestion was demonstrated on the majority of random biopsies throughout the GI tract. Inflammation was not highly prevalent on random biopsies except in the stomach. Wireless capsule endoscopy was performed on 18 patients; ulcers and strictures were the most common abnormalities.
DISCUSSION
GI symptoms were common in this cohort of American patients with BD. Endoscopic examination was often normal; however, histopathologic examination demonstrated vascular congestion throughout the GI tract.
Topics: Humans; Male; Female; United States; Young Adult; Adult; Middle Aged; Behcet Syndrome; Ulcer; Gastrointestinal Diseases; Colonoscopy; Capsule Endoscopy
PubMed: 37114910
DOI: 10.14309/ctg.0000000000000591 -
Archives of Dermatological Research Jan 2024The primary objective of this study was to investigate the association between disease activity and serum and salivary procalcitonin (Pct) levels in patients with...
The primary objective of this study was to investigate the association between disease activity and serum and salivary procalcitonin (Pct) levels in patients with Behçet's disease (BD). The study included patients diagnosed with BD and age-matched healthy volunteers (N: 48, N: 32). Serum and salivary Pct levels were quantified using enzyme-linked immunosorbent assay (ELISA) in the laboratories of Diskapi Yildirim Beyazit Training and Research Hospital. No significant disparity was observed in serum and salivary Pct levels between the patient and control groups (p > 0.05). Furthermore, there was no noteworthy correlation between disease activity and serum and salivary Pct values (p > 0.05). However, the serum Pct level in patients with active oral ulcers was significantly elevated compared to those without active oral ulcers (p: 0.003). Serum Pct emerges as a valuable marker for monitoring oral aphthous ulcer attacks within the patient population.
Topics: Humans; Behcet Syndrome; Oral Ulcer; Enzyme-Linked Immunosorbent Assay; Healthy Volunteers
PubMed: 38236279
DOI: 10.1007/s00403-023-02805-0 -
BMC Neurology Sep 2023Neuro-Behçet's disease (NBD), characterized by isolated intracranial hypertension, is a rarely encountered condition, especially in children. In this study, we describe... (Review)
Review
BACKGROUND
Neuro-Behçet's disease (NBD), characterized by isolated intracranial hypertension, is a rarely encountered condition, especially in children. In this study, we describe the treatment of a pediatric patient with NBD, who exhibited isolated intracranial pressure elevation as indicated by the initial manifestation of diplopia and physical examination finding of papilledema.
CASE PRESENTATION
An 8-year-old boy was admitted to the hospital with a confirmed diagnosis of Behçet's disease (BD) over eight months. The patient also presented with the symptom of diplopia for three days. The evaluation of the patient's nervous system did not reveal any apparent abnormalities. The measurement of cerebrospinal fluid pressure yielded a reading of 470 mm HO. The examination of the fundus indicated papilledema, and imaging scans revealed evidence of focal demyelination. The symptoms of the child showed alleviation after the administration of mannitol, methylprednisolone, and azathioprine. Furthermore, this study involved a comprehensive analysis of 18 cases of NBD with isolated intracranial hypertension, comprising one case reported herein and 17 cases from the literature review. Three cases were children, and an equal distribution of males and females 9:9 was noted. The average age at the onset of symptoms was 24.7 years (8-38 years). Headache (90%) was the most commonly reported clinical manifestation, followed by blurred vision or diplopia (80%). The ocular manifestations included papilledema (100%), abducent nerve paralysis (20%) and local eye hemorrhages in the retina (30%). Notably, 88.9% of these ocular manifestations were relieved or cured after treatment.
CONCLUSION
This study presents the first reported case of NBD with isolated intracranial hypertension in the pediatric population of China. In a child with Bechet's disease presenting with features of raised intracranial pressure, it is important to be aware of neuro Bechet's presenting with intracranial hypertension without other neurological abnormalities. This will help make early diagnosis, institute treatment and prevent sequelae resulting from untreated raised intracranial pressure.
Topics: Male; Female; Child; Humans; Young Adult; Adult; Papilledema; Behcet Syndrome; Diplopia; Intracranial Hypertension; Awareness
PubMed: 37749518
DOI: 10.1186/s12883-023-03392-3 -
International Ophthalmology Dec 2023To evaluate the long-term visual outcomes of patients with uveitis undergoing cataract surgery and to identify possible factors influencing the visual prognosis and the...
PURPOSE
To evaluate the long-term visual outcomes of patients with uveitis undergoing cataract surgery and to identify possible factors influencing the visual prognosis and the development of postoperative complications.
METHODS
Retrospective study of all patients with uveitis who underwent cataract surgery between January 2015 and February 2020 in our tertiary referral center.
RESULTS
A total of 78 eyes from 78 patients were included in the study. The best-corrected visual acuity (BCVA) improved in 86% of patients, and a BCVA of 0.5 or better was achieved in 57 (73%) patients. A significant correlation was shown between the preoperative and postoperative BCVA (Spearman r = 0.521, p < 0.01). Final BCVA differed between diverse anatomical uveitis entities (p = 0.047), and anterior uveitis demonstrated the best outcomes. Chronic uveitis resulted in a worse final BCVA than acute recurrent uveitis (p = 0.001). The presence of CME any time before the surgery and intermediate uveitis were associated with worse visual prognosis, while systemic therapy for uveitis before surgery and iris manipulation during surgery were not related to visual outcomes. Postoperative development of cystoid macular edema (CME) was closely associated with preexisting CME (p < 0.001) and intermediate uveitis (p = 0.01).
CONCLUSIONS
Visual results of cataract surgery in patients with uveitis were beneficial, but limited visual outcomes were more frequently observed in patients with chronic uveitis and intermediate uveitis with a history of CME. In consequence, prevention, or adequate treatment of CME, especially in patients with intermediate uveitis, might result in better visual results of their cataract surgery.
Topics: Humans; Prognosis; Retrospective Studies; Phacoemulsification; Cataract; Lens Implantation, Intraocular; Uveitis; Uveitis, Intermediate; Iridocyclitis; Macular Edema; Treatment Outcome
PubMed: 37697081
DOI: 10.1007/s10792-023-02860-6 -
Graefe's Archive For Clinical and... Aug 2023To demonstrate different topographic distributions of multiple-evanescent white dot syndrome (MEWDS) and secondary MEWDS disease and to describe possible associations.
PURPOSE
To demonstrate different topographic distributions of multiple-evanescent white dot syndrome (MEWDS) and secondary MEWDS disease and to describe possible associations.
METHODS
Clinical evaluation and multimodal retinal imaging in 27 subjects with MEWDS (29 discrete episodes of MEWDS). Ophthalmic assessment included best-corrected visual acuity testing and multimodal retinal imaging with OCT, blue-light autofluorescence, fluorescein and indocyanine green angiography, fundus photography, and widefield pseudocolor and autofluorescence fundus imaging.
RESULTS
The topographic distribution of MEWDS lesions was centered on or around the optic disc (n = 17, 59%), centered on the macula (n = 7, 24%), sectoral (n = 2, 7%), or was indeterminate (n = 3, 10%). The MEWDS episodes either occurred in the absence ('primary MEWDS'; n = 14, 48%) or presence of concurrent chorioretinal pathology ('secondary MEWDS'; n = 15, 52%). In patients with the latter, MEWDS lesions were often centered around a coexisting chorioretinal lesion. The majority of patients in both groups experienced resolution of their symptoms and retinal changes on multimodal imaging by 3 months.
CONCLUSIONS
Distinct distributions of MEWDS lesions were identified. MEWDS may occur in tandem with other chorioretinal pathology, which may impact the topography of MEWDS lesions.
Topics: Humans; Retinal Diseases; Tomography, Optical Coherence; Retina; White Dot Syndromes; Fluorescein Angiography
PubMed: 36988677
DOI: 10.1007/s00417-023-06032-1 -
Medicina (Kaunas, Lithuania) Dec 2023Behçet's disease (BD) is a systemic vasculitis that frequently presents with a relapsing-remitting pattern. CNS involvement (Neuro-Behçet) is rare, affecting... (Review)
Review
Behçet's disease (BD) is a systemic vasculitis that frequently presents with a relapsing-remitting pattern. CNS involvement (Neuro-Behçet) is rare, affecting approximately 10% of patients. Its etiological mechanisms are not yet fully understood. The most commonly accepted hypothesis is that of a systemic inflammatory reaction triggered by an infectious agent or by an autoantigen, such as heat shock protein, in genetically predisposed individuals. Mycobacterium tuberculosis is known to be closely interconnected with BD, both affecting cell-mediated immunity to a certain extent and probably sharing a common genetic background. We present the case of a 34-year-old Caucasian woman who had been diagnosed with tuberculous meningitis 15 months prior, with significant neurological deficits and lesional burden on MRI with repeated relapses whenever treatment withdrawal was attempted. These relapses were initially considered as reactivation of tuberculous meningoencephalitis, and symptoms improved after a combination of antituberculous treatment and corticosteroid therapy. After the second relapse, the diagnosis was reconsidered, as new information emerged about oral and genital aphthous lesions, making us suspect a BD diagnosis. HLA B51 testing was positive, antituberculous treatment was stopped, and the patient was started on high doses of oral Cortisone and Azathioprine. Consequently, the evolution was favorable, with no further relapses and slow improvements in neurological deficits. To our knowledge, this is the first report of Neuro-Behçet's disease onset precipitated by tuberculous meningitis. We include a review of the available literature on this subject. Our case reinforces the fact that Mycobacterium tuberculosis infection can precipitate BD in genetically predisposed patients, and we recommend HLA B51 screening in patients with prolonged or relapsing meningoencephalitis, even if an infectious agent is apparently involved.
Topics: Female; Humans; Adult; Behcet Syndrome; HLA-B51 Antigen; Tuberculosis, Meningeal; Neoplasm Recurrence, Local; Meningoencephalitis; Recurrence
PubMed: 38138266
DOI: 10.3390/medicina59122163 -
Indian Journal of Ophthalmology Jul 2024To compare various ocular thermography parameters in posterior scleritis (PS), choroiditis (choroidal granuloma [CG], Vogt-Koyanagi-Harada [VKH] syndrome), central... (Comparative Study)
Comparative Study Observational Study
PURPOSE
To compare various ocular thermography parameters in posterior scleritis (PS), choroiditis (choroidal granuloma [CG], Vogt-Koyanagi-Harada [VKH] syndrome), central serous chorioretinopathy (CSCR), and healthy controls.
METHODS
This retrospective, observational, comparative study evaluated cases undergoing ocular thermography between April 2017 and October 2023. The study groups included cases of PS, CG, and VKH, while the control group comprised CSCR cases and healthy individuals. Various thermography parameters were assessed, which were as follows: Ocular surface temperature (OST), central corneal temperature (CCT), average scleral temperature (ST), nasal scleral temperature (nST), temperature difference between both the eyes (∆t), and difference between scleral and corneal temperatures (ST - CCT, nST - CCT).
RESULTS
It was found that ∆t was significantly higher in the PS group compared to the CG ( P = 0.005), CSCR ( P = 0.0001), and control groups (dilated control: P =0.006, undilated control: P = 0.0001). ST - CCT and nST - CCT were significantly higher in the undilated control and CSCR groups and significantly lower in the PS group. ST - CCT and nST - CCT showed less difference in the affected eyes compared to contralateral healthy eyes of PS and CG cases. OST, CCT, ST, and nST displayed statistically insignificant differences across all groups.
CONCLUSION
It is advisable to focus on temperature differences between the affected and healthy eyes, or the difference between the central corneal and scleral temperature of the affected eye, utilizing parameters such as ∆t, ST - CCT, and nST - CCT. These composite parameters offer a more effective approach than individual measurements like OST, CCT, ST, and nST. Thermography can serve as a screening tool to suspect and differentiate PS.
Topics: Humans; Scleritis; Retrospective Studies; Thermography; Female; Male; Adult; Middle Aged; Sclera; Choroiditis; Body Temperature
PubMed: 38454839
DOI: 10.4103/IJO.IJO_2830_23