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Acta Medica Portuguesa Sep 2023Behçet's disease is a relapsing multisystemic inflammatory syndrome characterized by recurrent oral and/or genital ulcers, uveitis, arthritis, skin lesions, and... (Review)
Review
Behçet's disease is a relapsing multisystemic inflammatory syndrome characterized by recurrent oral and/or genital ulcers, uveitis, arthritis, skin lesions, and gastrointestinal and neurological involvement. Neuro-Behçet corresponds to nervous system involvement and is one of the most severe complications of Behçet disease. It occurs in 3% to 30% of cases and is categorized into parenchymal (most common) or non-parenchymal disease. The most common manifestation of parenchymal neuro-Behçet is meningoencephalitis with involvement of the brainstem, where patients present with cranial neuropathies, encephalopathy, sensory-motor syndromes, epilepsy, or myelitis. The main non-parenchymal manifestation is cerebral venous thrombosis. Neuro-Behçet has a predominantly subacute course, with remission within weeks, or clinical progression in one third of the cases. The diagnosis is essentially clinical and diagnostic tests help to corroborate the suspicion, distinguish from differential diagnoses, and exclude complications. Brain magnetic resonance imaging allows the identification of acute lesions (hypointense or isointense on T2-weighted and hypointense on T1-weighted sequences) contrast-enhanced, and chronic lesions characterized by non-contrast enhanced small lesions and brainstem atrophy. If non-parenchymal involvement is suspected, cerebral veno-magnetic resonance imaging /computed tomography should be performed. Cerebrospinal fluid shows elevated proteinorachia and pleocytosis in parenchymal and no changes in non-parenchymal neuro-Behçet (except increased opening pressure). Outbursts of parenchymal disease should be treated with high dose intravenous corticosteroid therapy, with subsequent switch to oral corticoids, followed by biologic therapy, usually an anti-TNF. The treatment of cerebral venous thrombosis is controversial and may consist of a combination of corticosteroids and anticoagulation.
Topics: Humans; Behcet Syndrome; Tumor Necrosis Factor Inhibitors; Brain; Magnetic Resonance Imaging; Diagnosis, Differential; Adrenal Cortex Hormones; Venous Thrombosis
PubMed: 37345389
DOI: 10.20344/amp.19734 -
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 -
Behçet's Disease, Pathogenesis, Clinical Features, and Treatment Approaches: A Comprehensive Review.Medicina (Kaunas, Lithuania) Mar 2024Behçet's disease is a systemic inflammatory disorder of unknown etiology. The disease manifests with diverse clinical symptoms, most commonly recurrent oral and genital... (Review)
Review
Behçet's disease is a systemic inflammatory disorder of unknown etiology. The disease manifests with diverse clinical symptoms, most commonly recurrent oral and genital ulcers, skin lesions, and uveitis, though it can affect multiple organ systems. Diagnosis is primarily clinical due to the lack of a definitive diagnostic test, and management involves a multidisciplinary approach to control inflammation and manage symptoms. Current treatment strategies involve corticosteroids, immunosuppressive agents, and, increasingly, biological therapies. Behçet's disease exhibits a higher prevalence along the Silk Road, suggesting a role of environmental and genetic factors. Despite significant progress in understanding its clinical characteristics and treatment approaches, gaps remain in our understanding of its pathogenesis. Future research is needed to elucidate the disease's pathophysiology and optimize treatment strategies.
Topics: Humans; Adrenal Cortex Hormones; Behcet Syndrome; Immunosuppressive Agents
PubMed: 38674208
DOI: 10.3390/medicina60040562 -
Revista Espanola de Enfermedades... Jul 2023A 36-year-old man was admitted to the emergency department due to a 30-day history of abdominal distention and epigastralgia. He had described a non-intentional 10kg...
A 36-year-old man was admitted to the emergency department due to a 30-day history of abdominal distention and epigastralgia. He had described a non-intentional 10kg weight loss, dry cough, and fever 6 months before his admission. He had a history of tobacco and cocaine abuse and reported recurrent oral and genital ulcers. Physical examination showed an extensive area of venous collateral circulation on the abdominal wall, hepatomegaly, signs of a moderate ascites, and lower limb edema. Liver and renal function tests were normal. The ascitic fluid analysis did not show an inflammatory or infectious pattern. Upper flexible endoscopy revealed esophageal fine-caliber varices and colonoscopy showed an isolated terminal ileal ulcer. Abdominal imaging revealed hepatomegaly, voluminous ascites, and thrombosis of hepatic veins, inferior and superior vena cava (Figure 1). Infections and coagulation or lymphoproliferative disorders were excluded. Thereafter, the diagnosis of Budd-Chiari Syndrome in Behçet disease was established and immunosuppression treatment was started with good initial clinical evolution.
Topics: Male; Humans; Adult; Budd-Chiari Syndrome; Behcet Syndrome; Ascites; Hepatomegaly; Vena Cava, Superior; Vena Cava, Inferior
PubMed: 36263811
DOI: 10.17235/reed.2022.9267/2022 -
BMC Ophthalmology Jul 2023Sarcoidosis is an inflammatory disorder in which patients frequently develop ocular manifestations that precede systemic involvement, sometimes it even presents as an...
BACKGROUND
Sarcoidosis is an inflammatory disorder in which patients frequently develop ocular manifestations that precede systemic involvement, sometimes it even presents as an ocular isolated form of the disease. The purpose of this study is to report the ocular and systemic manifestations of sarcoidosis in a series of Mexican patients, as there is a low incidence of the disease in this population.
METHODS
A retrospective case series of patients with positive classification criteria for sarcoidosis who attended Asociacion Para Evitar la Ceguera en Mexico, IAP between 2011 and 2022. Descriptive statistics were used to report the clinical, laboratory, and imaging findings and treatment. Numerical results were presented using median values and first and third quartiles for distribution.
RESULTS
Fourteen patients were included in this study, 10 of them had definite ocular sarcoidosis (biopsy-proven), 4 had presumed ocular sarcoidosis. The median age of onset was 52 (34; 67), with a predominance of female patients (71.4%). Ten patients (71.4%) debuted with ocular manifestations. The most common forms of ocular involvement were bilateral anterior uveitis (50%) and panuveitis (28.6%). Median follow-up was 24 (13-49) months.
CONCLUSIONS
Sarcoidosis is a rare, underdiagnosed condition in Mexico and ocular involvement can be an early manifestation of the disease. Ophthalmologists should be alert to the signs of ocular sarcoidosis and collaborate with a multidisciplinary team to screen for systemic involvement if suspicion is high.
Topics: Humans; Female; Male; Retrospective Studies; Mexico; Sarcoidosis; Uveitis; Eye; Endophthalmitis
PubMed: 37474932
DOI: 10.1186/s12886-023-03081-2 -
Journal of Current Ophthalmology 2023To describe delayed-onset infectious endophthalmitis 4 months after intravitreal aflibercept injection.
PURPOSE
To describe delayed-onset infectious endophthalmitis 4 months after intravitreal aflibercept injection.
METHODS
An 80-year-old female was referred with signs and symptoms of clinical endophthalmitis 4 months after intravitreal injection of aflibercept for choroidal neovascularization. Noninfectious causes of panuveitis were excluded and she was diagnosed with delayed-onset postinjection infectious endophthalmitis. Vitreous and aqueous specimens were prepared and antibiotics (vancomycin and ceftazidime) were injected intravitreally.
RESULTS
Vitreous culture was positive for . During the 1 month after the antibiotic injections, symptoms and signs of the patient improved and became stable during the 6-month follow-ups.
CONCLUSIONS
Delayed-onset infectious endophthalmitis can be presented following intravitreal injections. Late presentation of uveitis in postinjected eyes needs complete investigations to rule out infectious endophthalmitis as an ophthalmic emergency.
PubMed: 38250491
DOI: 10.4103/joco.joco_134_22 -
International Journal of Ophthalmology 2024To determine the common causes and visual outcome after treatment among uveitis and scleritis patients.
AIM
To determine the common causes and visual outcome after treatment among uveitis and scleritis patients.
METHODS
This is a retrospective cohort observational study. All consecutive clinical records of patients with newly diagnosed uveitis and scleritis over a 4-year period, from Jan. 1, 2017 to Dec. 31, 2020, were analysed. Data was collected at the presentation and included a follow-up period of one year.
RESULTS
A total of 288 patients were recruited during the study period. Anterior uveitis was the most common anatomical diagnosis (50.0%) followed by panuveitis (25.0%), scleritis (13.5%), posterior uveitis (6.9%), and intermediate uveitis (4.5%). Viral Herpes was the most common cause of infectious cases, while Vogt-Koyanagi-Harada (VKH) disease and human leucocyte antigen (HLA) B27 spondyloarthropathy were the leading causes of identifiable non-infectious cases. Majority of patients presented with unilateral, non-granulomatous uveitis with an absence of hypopyon. Anatomical locations like posterior uveitis and panuveitis, and visual acuity worse than 3/60 at presentation were the factors associated with poor visual outcomes (<0.05). About 60% of patients had an identifiable cause for the uveitis and scleritis, with nearly equal distribution of infectious (=85, 29.5%) and non-infectious causes (=84, 29.2%). About 14.5% of patients were clinically blind at 1y of follow-up. The most common complication in our uveitis patients was glaucoma (47.5%), followed by cystoid macula oedema (18.9%) and cataract (13.9%).
CONCLUSION
Uveitis and scleritis are important causes of ocular morbidity. They are potentially blinding diseases which can have a good outcome if diagnosed and treated early.
PubMed: 38721518
DOI: 10.18240/ijo.2024.03.14 -
Ophthalmology and Therapy Aug 2023Scientific evidence of the effectiveness of the tumor necrosis factor inhibitor adalimumab (ADA) in pediatric patients with non-infectious non-anterior uveitis is still...
INTRODUCTION
Scientific evidence of the effectiveness of the tumor necrosis factor inhibitor adalimumab (ADA) in pediatric patients with non-infectious non-anterior uveitis is still limited. The aim of this study is to investigate the therapeutic role of ADA in a cohort of pediatric patients with non-anterior uveitis.
METHODS
This is an international multicenter study analyzing real-life data referred to pediatric patients treated with ADA for intermediate uveitis/pars planitis, posterior uveitis and panuveitis. Data were drawn from the AutoInflammatory Disease Alliance (AIDA) registry for patients with uveitis.
RESULTS
Twenty-one patients (36 affected eyes) were enrolled, and all patients benefited from ADA administration. In detail, 11 patients (19 affected eyes) did not experience further ocular inflammation after ADA introduction; 10 cases (17 affected eyes) showed a significant clinical improvement consisting of a decrease in severity and/or frequency of ocular relapses. The number of ocular flares dropped from 3.91 to 1.1 events/patient/year after ADA introduction (p = 0.0009); macular edema and retinal vasculitis were respectively observed in 18 eyes and 20 eyes at the start of ADA and in 4 eyes and 2 eyes at the last assessment. The mean daily glucocorticoid dosage significantly decreased from 26.8 ± 16.8 mg/day at the start of ADA to 6.25 ± 6.35 mg/day at the last assessment (p = 0.002). Intermediate uveitis/pars planitis (p = 0.01) and posterior uveitis (p = 0.03) were more frequently observed in patients with full response to ADA; panuveitis (p = 0.001) was significantly more frequent among patients continuing to experience uveitic flares. This could be related to a higher use of systemic glucocorticoids (p = 0.002) and conventional immunosuppressants (p = 0.007) at the start of ADA when treating intermediate uveitis/pars planitis. Regarding the safety profile, only one adverse event was reported during ADA treatment, consisting of the development of generalized adenopathy.
CONCLUSIONS
ADA proved to have an effective therapeutic role in all pediatric patients with non-anterior uveitis enrolled in the study. An overall glucocorticoid-sparing effect was observed despite the severity of cases enrolled. A more aggressive treatment of panuveitis and posterior uveitis at start of ADA could increase the likelihood of full response to therapy.
PubMed: 37166627
DOI: 10.1007/s40123-023-00712-1 -
Clinics in Dermatology 2023Adamantiades-Behçet disease is an inflammatory, vascular disease of unknown etiology. The disease is named after two physicians, Benediktos Adamantiades and Hulȗsi...
Adamantiades-Behçet disease is an inflammatory, vascular disease of unknown etiology. The disease is named after two physicians, Benediktos Adamantiades and Hulȗsi Behçet, who both made significant contributions to the study of the disease. It was probably first described by Hippocrates in 500 BCE. Adamantiades-Behçet disease is most common in the region encompassing the ancient trade route known as the Silk Road. In Turkey, the disease is estimated to affect 80 to 370 people per 100,000 inhabitants, and it is also the country with the highest incidence rate. The frequency of the disease associated with the clinical picture differs from the origin of the onset. The disease is characterized by recurrent aphthous ulcers of the mouth, genitals, skin lesions, and eye lesions. The disease process can also involve other organs, including the joints, nervous system, large vessels, heart, and gastrointestinal tract. Aphthous oral ulcers appear as the first harbinger of the disease and affect almost all patients (97%-99%). The scientific interest in Adamantiades-Behçet disease has increased exponentially in the past decade.
Topics: Humans; Behcet Syndrome; Ophthalmology; Dermatology; Stomatitis, Aphthous
PubMed: 37572969
DOI: 10.1016/j.clindermatol.2023.08.001 -
Pediatric Rheumatology Online Journal Oct 2023Pediatric uveitis is a severe inflammatory ocular condition that can lead to sight-threatening complications and can negatively impact quality of life. The retinal...
BACKGROUND
Pediatric uveitis is a severe inflammatory ocular condition that can lead to sight-threatening complications and can negatively impact quality of life. The retinal microcirculation is often affected in intermediate uveitis and panuveitis. Here, we examined the extraocular (i.e., systemic) microcirculation in pediatric uveitis cases and healthy controls using nailfold capillaroscopy (NFC).
METHODS
We performed NFC in 119 children with noninfectious uveitis and 25 healthy pediatric controls, and assessed the following parameters: capillary density (number of capillaries/mm), dilated capillaries (apex > 20 µm), avascular area, the presence of microhemorrhages, and capillary morphology. Differences in NFC parameters between cases and controls were calculated using regression analysis after adjusting for age and sex.
RESULTS
The mean (± SD) age of the patient group was 13.7 (± 3) years, with 56% females; 46%, 18%, and 36% of cases presented as anterior uveitis, intermediate uveitis, and panuveitis, respectively, with an overall mean disease duration of 4.7 (± 4.0) years. Compared to the control group, the pediatric uveitis cases had a significantly higher number of dilated capillaries/mm and a higher prevalence of ramified capillaries. Moreover, compared to the control group the intermediate uveitis cases had a significantly higher number of dilated capillaries, whereas the anterior uveitis cases had a lower capillary density and a higher prevalence of ramified capillaries.
CONCLUSIONS
Children with uveitis without systemic disease can present with changes in systemic microcirculation. These changes vary amongst the subtypes of uveitis.
Topics: Female; Humans; Child; Adolescent; Male; Microcirculation; Quality of Life; Nails; Uveitis; Uveitis, Anterior; Uveitis, Intermediate; Panuveitis; Microscopic Angioscopy
PubMed: 37784087
DOI: 10.1186/s12969-023-00896-7