scholarly journals Beneficial Effects of Apremilast on Genital Ulcers, Skin Lesions, and Arthritis in Patients With Behçet’s Disease: A Systematic Review and Meta-Analysis

2021 ◽  
Author(s):  
Yuki Iizuka ◽  
Kaoru Takase-Minegishi ◽  
Lisa Hirahara ◽  
Yohei Kirino ◽  
Yutaro Soejima ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Meta Analysis ◽  
Skin Lesions ◽  
Cochrane Library ◽  
Behcet's Disease ◽  
Beneficial Effects ◽  
Oral Ulcers ◽  
Genital Ulcers

Abstract Objective This study aimed to determine the clinical efficacy of apremilast for oral ulcers, extra-oral manifestations, and overall disease activity in patients with Behçet’s disease (BD). Methods A systematic literature search was performed in PubMed, EMBASE, Cochrane Library, and Web of Science Core Collection. Studies assessing the treatment effects of apremilast in BD were included. The odds ratios (ORs) of being symptom free for individual manifestations and mean difference (MD) of Behçet’s Disease Current Activity Form (BDCAF) scores were calculated with 95% confidence intervals (CIs) at 12 and 24 weeks using a random-model meta-analysis. Results Of 259 screened articles, eight were included. After 12 weeks of apremilast treatment the OR of symptom-free was as followings: oral ulcers, 45.76 (95% CI, 13.23–158.31); genital ulcers, 4.56 (95% CI, 2.47–8.44); erythema nodosum, 3.59 (95% CI, 1.11–11.61); pseudofolliculitis, 2.81 (95% CI, 1.29–6.15); and arthritis, 3.55 (95% CI, 1.71–7.40). Furthermore, BDCAF scores at 12 weeks were significantly reduced (MD=−1.38; −1.78 to −0.99). However, the proportion of oral-ulcer free patients increased at 24 weeks (OR=14.88; 4.81 to 46.07). Conclusion The currently accumulated data indicates an improvement in mucocutaneous and articular symptoms by short-term apremilast treatment in patients with BD.

scholarly journals Aortic thrombosis in a patient with a late diagnosis of Behcet's disease

2015 ◽  
Vol 14 (2) ◽  
pp. 193-196
Author(s):  
Ana Bittencourt Detanico ◽  
Marcelo Luiz Brandão ◽  
Ly de Freitas Fernandes ◽  
Carolina Parreira Ribeiro Camelo ◽  
Juliano Ricardo Santana dos Santos
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Thromboangiitis Obliterans ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Oral Ulcers ◽  
Arterial Involvement ◽  
Genital Ulcers

Behcet's disease is form of systemic vasculitis of unknown etiology. One surprising feature is that arterial involvement is less common in this disease than venous forms, accounting for 1 to 7% of patients. In 7 to 30% of cases the vascular symptoms precede the clinical diagnosis of Behcet. We describe the case of a patient with Thromboangiitis Obliterans who was treated with lumbar sympathectomy and an aortobiiliac bypass. Around 1 year later he was diagnosed with Behcet's disease after presenting with oral ulcers, genital ulcers and scaling lesions on the hands.

Ethiopathogenesis of Behçet's Disease

2018 ◽  
pp. 324-328
Keyword(s):  
Behçet’S Disease ◽  
Genetic Factors ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Skin Lesions ◽  
Th2 Cells ◽  
Innate And Adaptive Immunity ◽  
Behcet's Disease ◽  
Oral Ulcers ◽  
Th 1

Behçet’s disease (BD) is a chronic multisystem inflammatory disease characterized mainly by recurrent oral ulcers, ocular inflammation, genital ulcers, and skin lesions, presenting with remission and exacerbations. The pathogenesis of BD has not been fully elucidated since it has a broad spectrum of phenotypic heterogeneity and complex immunopathogenesis. BD has been considered that both environmental and genetic factors contribute to its onset and development. Recent advances in molecular biology and genetics show us that particular genes encompass both innate and adaptive immunity, and confirm the importance of the predominant polarization towards helper T cell (Th)1 versus Th2 cells, and the involvement of Th17 cells in BD.

scholarly journals Behcet’s disease: a review

2021 ◽  
Author(s):  
Daniela Ann Reyes-Weaver ◽  
Kevin Luis Plata-Jimenez ◽  
Raul Melo-Acevedo
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Age Of Onset ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Skin Lesions ◽  
Vascular Lesions ◽  
Severe Condition ◽  
Behcet's Disease ◽  
Oral Ulcers

Behcet's disease (BD) is a complex systemic vasculitis with an etiopathogenesis that remains unclear. It has a strong geographic association as well as a genetic propensity linked to the HLA-B51 factor and interactions between genetic and environmental factors. The typical age of onset is 25 to 40 years old and it is more common in men, who also have a more severe condition. Occlusive vasculitis is the hallmark of this condition, which can affect vessels of all diameters. Oral ulcers, genital ulcers, skin lesions, pathergy reaction as well as involvement of other systems and organs such as ophthalmic, neurological and vascular lesions, among others, are used to make the diagnosis. Uncontrolled neutrophil activation, activation of the humoral and cell immune systems, toxic proteins and infectious agents such as herpes simplex and streptococci are all involved in their pathophysiology. Due to the heterogeneity and several systems affected, the treatment is individualized and focused on treating each clinical manifestation.

scholarly journals Behçet's Disease with Multiorgan Infection: Alternative Treatment to Systemic Immunosuppressants

2021 ◽  
Vol 12 (2) ◽  
pp. 285
Author(s):  
Dian Hasanah
Keyword(s):  
Behçet’S Disease ◽  
Alternative Treatment ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Skin Lesions ◽  
Blood Leukocytes ◽  
Behcet's Disease ◽  
X Ray ◽  
Chest X Ray ◽  
Genital Ulcers

A B S T R A C T   Background. Behçet's disease is a rare systemic autoimmune vasculitis. The presence of infection makes it difficult to manage. Case. Ms. X, 19 years old, complained of mouth and genitalia ulcers and joints pain. She had typhoid fever two months before; and a week after recovery, she experienced reddish skin lesions on her legs. Her condition was weak and the pain visual analog score (VAS) was 9/10. Multiple oral ulcers were found in her oral cavity and vulva. Her lungs were hypersonor. There were hyperpigmented maculae in her lower extremities. Her ankle joints were painful in passive and active movements. Blood leukocytes: 13,210/µl, urine leukocytes: 20-23/hpf, ANA, anti-dsDNA and Pathergy tests were negative. The diagnosis of Behçet's disease was based on multiple oral and genital ulcers, history of erythema nodosum and arthritis. The patient was treated with topical triamcinolone. Joint ulcers and pain worsened; so that intravenous methylprednisolone was administer. Two days later, the patient had cough and oxygen desaturation. Chest X-ray showed emphysematous lungs and pneumonia. Methylprednisolone was stopped, ceftriaxone and levofloxacin were given, treatment for ulcers and arthritis was replaced with colchicine and rebamipide. Sucralfate is given for gargle and applied to the genital ulcers. On the seventh day, the ulcers were greatly reduced, joint pain resolved, cough subsided and VAS became 2/10. On the twelfth day, the chest X-ray was normal and ulcers were healed, ankle pain was suffered again accompanied by swelling. The patient was treated with methylprednisolone and azathioprine. Discussion. Treatment for this disease is immunosuppressants. In severe conditions with infection, colchicine can be an alternative treatment. Adjuvant treatment are rebamipide and/or sucralfate. Emphysematous lungs can be caused by alpha-1 antitrypsin deficiency or pulmonary vasculitis, which can be manifested in autoimmune diseases. Emphysematous lungs, leads us to follow up on the development to other autoimmune disorders such as lupus. Conclusion. Behçet's disease with infection can be successfully managed with colchicine, rebamipide and sucralfate.

scholarly journals POS1358 THE EFFECTS AND SAFETY OF APREMILAST AND CYTOKINE EXPRESSION IN BEHCET’S DISEASE PATIENTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 961.1-961
Author(s):  
Y. Ushio ◽  
R. Wakiya ◽  
K. Ueeda ◽  
T. Kameda ◽  
S. Nakashima ◽  
...  
Keyword(s):  
Psoriatic Arthritis ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Skin Lesions ◽  
Cytokine Expression ◽  
Behcet's Disease ◽  
Serum Cytokines ◽  
Oral Ulcers ◽  
The Mean ◽  
Genital Ulcers

Background:Apremilast, the small-molecule phosphodiesterase (PDE) -4 inhibitor, was approved for the treatment of recurrent oral ulcers associated with Behcet’s disease (BD) in Japan from September 2019, following the success of the phase 3 RELIEF study (1). However, the efficacy of apremilast on domains other than oral ulcers in BD patients is unclear. On the other hand, it has been reported that apremilast may decrease the production of proinflammatory cytokine and increase the production of anti-inflammatory mediators in psoriasis (PS) and psoriatic arthritis (PsA) (2).Objectives:To evaluate the effects and safty of apremilast on clinical symptoms and the changing of serum cytokine expression.Methods:BD patients who had treated with apremilast for active oral ulcers were included in the study. We investigated the improvement rate of oral and genital ulcers, skin lesions, arthritis. In addition, serum cytokines (IFN-γ, IL-10, IL-8, and TNF-α) before and after apremilast treatment were measured using a multiplex immunoassay (Luminex Assay, R&D Systems).Results:Fourteen patients (3 males and 11 females) were enrolled in this study. The mean age was 46.6 ± 13.0 years and the mean duration of disease was 10.2 ± 8.8 years. All patients had active oral ulcers, five had genital ulcers, six had skin lesions, and four had arthritis. Three months after the treatment with apremilast, oral ulcers improved in 13 patients (92.9%). The improvement rates of genital ulcers, skin lesions and arthritis were 60%, 25% and 25%, respectively. Changes in serum cytokines were different from those previously reported in PS. Adverse events were gastrointestinal symptoms such as nausea and diarrhea in 6 patients and sensorineural deafness in 1 patient. Medication was reduced in 2 patients, and discontinued in 1 patient due to nausea and diarrhea.Conclusion:Apremilast is useful not only for oral ulcers, but also for other lesions in BD patients. The effect of apremilast for other domain such as genital ulcers, skin lesions, arthritis was not comparable to that of active oral ulcers. Additionally, BD may have different cytokine profile from PS and PsA.References:[1]Hatemi G, Mahr A, Ishigatsubo Y, et al. Trial of Apremilast for Oral Ulcers in Behcet’s Syndrome. N Engl J Med. 2019;381(20):1918-28[2]Schafer P. Apremilast mechanism of action and application to psoriasis and psoriatic arthritis. Biochem Pharmacol. 2012;83(12):1583-1590Disclosure of Interests:None declared

scholarly journals The Use of Biologic Agents in the Treatment of Oral Lesions due to Pemphigus and Behcet's Disease: A Systematic Review

2020 ◽  
Vol 1 (1) ◽  
pp. 14-23
Author(s):  
Gerald E. Davis II ◽  
George Sarandev ◽  
Alexander T. Vaughan ◽  
Kamal Al-Eryani ◽  
Reyes Enciso
Keyword(s):  
Systematic Review ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Meta Analysis ◽  
Biologic Agents ◽  
Risk Of Bias ◽  
Cochrane Library ◽  
Behcet's Disease ◽  
Randomized Controlled

Background: Current treatments for pemphigus and Behcet's disease, such as corticosteroids, have long-term serious adverse effects. Objective: The objective of this systematic review was to evaluate the efficacy of biologic agents (biopharmaceuticals manufactured via a biological source) on the treatment of intraoral lesions associated with pemphigus and Behcet's disease compared to glucocorticoids or placebo. Methods: PubMed, Web of Science, Cochrane Library, and EMBASE were searched for randomized controlled studies up to January 2019. Bias was assessed with the risk of bias tool. Results: Out of 740 references retrieved, only four randomized controlled trials (RCTs) were included, comprised of a total of 158 subjects (138 pemphigus and 20 Behcet's disease). All studies were assessed at high risk of bias. Heterogeneity of data prevented the authors from performing a meta-analysis. Infliximab or rituximab with short-term prednisone showed higher safety and lowered cumulative prednisone dose than prednisone alone in the treatment of pemphigus. Subcutaneous injection of etanercept provided 45% of patients free of ulcers compared to 5% in the placebo group in one study with Behcet's disease; however, no difference was found in pemphigus patients. Conclusion: Though biological agents alone or in combination with prednisone showed favorable results in three RCTs compared to prednisone alone or placebo, a meta-analysis could not be undertaken due to high heterogeneity. Results are inconclusive, and larger, well-designed RCTs are needed.

scholarly journals Pathologic Features of Behçet's Disease in the Tubuler Gut

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Tuba Kara ◽  
Duygu Düşmez Apa
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Japanese Patients ◽  
Skin Lesions ◽  
Behcet's Disease ◽  
Oral Ulcers ◽  
Mediterranean Countries ◽  
Pathologic Features ◽  
Urgent Operation

Behçet's disease (BD) is a vasculitic disorder of relapsing acute inflammation characterized by recurrent oral ulcers, genital ulcers, uveitis, and skin lesions. The disease also affects other organs, including joints, the nervous system, blood vessels, and gastrointestinal (GI) system may also be involved and the lower GI tract is the mostly involved part, leading to severe morbidity. The frequency of GI involvement in BD varies among different ethnic groups. Although 50–60% of Japanese patients have GI disease, these manifestations are rare in patients from Mediterranean countries. The gastrointestinal manifestations of BD usually appear 4.5–6 years after the onset of the oral ulcers. The intestinal lesions are usually resistant to medical treatment and recur after surgery. The elementary lesion is apthous ulcer. Deep, round or oval ulcers with a punched-out appearance tend to perforate easily, so that many patients require urgent operation.

scholarly journals Retinal microvasculature features in patients with Behcet’s disease: a systematic review and meta-analysis

2022 ◽  
Vol 12 (1) ◽  
Author(s):  
Kai-bao Ji ◽  
Zhe Hu ◽  
Qing-lin Zhang ◽  
Hai-feng Mei ◽  
Yi-qiao Xing
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Meta Analysis ◽  
Cochrane Library ◽  
Control Group ◽  
Continuous Variables ◽  
Behcet's Disease ◽  
Retinal Microvasculature ◽  
The Status

AbstractThis meta-analysis aimed to analyze retinal microvasculature features in eyes with Behçet’s disease (BD) using optical coherence tomography angiography (OCTA). Electronic databases, including PubMed, Web of Science, Embase, and Cochrane Library, were comprehensively searched for published studies comparing retinal microvasculature characteristics between eyes with BD and controls. Continuous variables were calculated using the mean difference (MD) with 95% confidence interval (CI). Review Manager software (version 5.30) was used to conduct statistical analysis. A total of 13 eligible studies involving 599 eyes with BD and 622 control eyes were included in the meta-analysis. The pooled results showed that the macular whole enface superficial and deep vessel density (VD) values measured by OCTA were significantly lower in eyes with BD than in control eyes (superficial VD: MD = − 3.05, P < 0.00001; deep VD: MD = − 4.05, P = 0.0004). The foveal superficial and deep VD values were also significantly lower in the BD group than in the control group (superficial VD: MD = − 1.50, P = 0.009; deep VD: MD = − 4.25, −  = 0.03). Similarly, the analysis revealed a significant reduction in the parafoveal superficial and deep VD in eyes with BD than in control eyes (superficial VD: MD =  − 3.68, P < 0.00001; deep VD: MD = − 4.95, P = 0.0007). In addition, the superficial and deep foveal avascular zones (FAZs) were significantly larger in patients with BD than in controls (superficial FAZ: MD = 0.06, P = 0.02; deep FAZ: MD = 0.12, P = 0.03). The present meta-analysis found that macular whole enface VD, foveal VD, and parafoveal VD were lower in eyes with BD, and the FAZ was larger in patients with BD. The findings suggest that OCTA can assist clinicians in diagnosing and monitoring the status of patients with BD.

scholarly journals Psychological and neurocognitive impact of Behcet’s disease

2020 ◽  
pp. 001-008
Author(s):  
Fisher Caroline A
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Skin Lesions ◽  
Neurological Involvement ◽  
Future Research ◽  
Behcet's Disease ◽  
Genital Ulcers

Background: Behcet’s disease is a vasculitis, causing multisystem inflammation and resulting in oral and genital ulcers and eye and skin lesions. A proportion of suffers also have neurological involvement, termed neuro-Behcet’s disease. The purpose of this review was to investigate the psychological and neurocognitive sequelae associated with Behcet’s and neuro-Behcet’s disease and provide directions for future research.

scholarly journals Association between IL1B −511C/T polymorphism and Behçet’s disease: a meta-analysis

2021 ◽  
Vol 49 (2) ◽  
pp. 030006052097986
Author(s):  
Baogang Wang ◽  
Yihang Gao ◽  
Yuying Tan ◽  
Xiaolin Yang ◽  
Xiaxia Man
Keyword(s):  
Behçet’S Disease ◽  
Large Scale ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Meta Analysis ◽  
Recessive Model ◽  
Cochrane Library ◽  
China National Knowledge Infrastructure ◽  
Behcet's Disease ◽  
Hardy Weinberg Equilibrium

Background Many studies have investigated the relationship between the interleukin-1β gene ( IL1B) −511C/T polymorphism and the risk of Behçet’s disease (BD); however, the conclusions remain controversial. Methods In this study, we systemically retrieved relevant studies from the Chinese Biomedicine Database, China National Knowledge Infrastructure, Embase, Cochrane Library, and PubMed databases. We then calculated the odds ratios (ORs) and 95% confidence intervals (CIs) using the meta-package Stata version 12.0. Results The IL1B −511C/T polymorphism was not related to BD susceptibility using any of the tested models (C vs T: OR = 1.20, 95% CI = 0.97–1.49; CC vs TT: OR = 1.27, 95% CI = 0.95–1.70; CT vs TT: OR = 1.03, 95% CI = 0.781.36; dominant model: OR = 1.12, 95% CI = 0.87–1.46; recessive model: OR = 1.27, 95% CI = 0.89–1.82). Similarly, subgroup analysis including studies consistent with the Hardy–Weinberg equilibrium revealed no association between the IL1B polymorphism and BD susceptibility. Conclusion This meta-analysis indicates that the IL1B −511C/T polymorphism is unlikely to affect the risk of BD; however, further large-scale, carefully designed studies are needed to verify these results.

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