scholarly journals AB0723 JUVENILE BEHCET’S DISEASE: WHICH PARTICULARITIES?

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1393.1-1393
Author(s):  
O. Jomaa ◽  
O. Berriche ◽  
S. Arfa ◽  
A. Fraj ◽  
W. Romdhane ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Joint Damage ◽  
Platelet Aggregation Inhibitors ◽  
Neurological Damage ◽  
Poor Prognostic Factor ◽  
Behcet's Disease ◽  
Male Predominance

Background:Behcet’s disease (BD) is a systemic vasculitis that affects young adults aged between 20 and 30 years old. It is rare in childhood.Objectives:This work aims to analyze the clinical features of this form by comparing it with adult BD.Methods:Through a retrospective study including 192 cases with BD seen in The Internal Medicine Department at Tahar Sfar Hospital Mahdia TUNISA, we report 8 cases of juvenile BD (4.2%) that occurred under the age of 16 years.Results:There were 8 male. The average age of BD onset was 14 years [11, 16 years]. Genital aphthosis was noted in 5 patients. Ophthalmologic damage was observed in 4 patients, dominated by uveitis (75% of cases). No cases of blindness were observed. Joint damage was seen in 5 patients and vascular and neurological damage in 2 patients respectively. All patients received colchicine in addition to a platelet aggregation inhibitors at the moment of BD diagnosis. When comparing juvenile BD group with that of adults, we have noticied, the frequency of cutaneo-mucous and articular manifestations, the rarity of neurological damage and the absence of cardiac and digestive damage in the juvenile BD group.Conclusion:Juvenile BD is a rare form, with a male predominance. The younger age is not a poor prognostic factor. Early diagnosis and treatment can reduce the disease’s complications.References:[1]doi: 10.1007/s00296-018-4208-9Disclosure of Interests:None declared

scholarly journals Old and New Challenges in Uveitis Associated with Behçet’s Disease

2021 ◽  
Vol 10 (11) ◽  
pp. 2318
Author(s):  
Julie Gueudry ◽  
Mathilde Leclercq ◽  
David Saadoun ◽  
Bahram Bodaghi
Keyword(s):  
Behçet’S Disease ◽  
Structural Damage ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Retinal Vasculitis ◽  
Unknown Origin ◽  
Current Data ◽  
Ocular Involvement ◽  
Behcet's Disease

Behçet’s disease (BD) is a systemic vasculitis disease of unknown origin occurring in young people, which can be venous, arterial or both, classically occlusive. Ocular involvement is particularly frequent and severe; vascular occlusion secondary to retinal vasculitis may lead to rapid and severe loss of vision. Biologics have transformed the management of intraocular inflammation. However, the diagnosis of BD is still a major challenge. In the absence of a reliable biological marker, diagnosis is based on clinical diagnostic criteria and may be delayed after the appearance of the onset sign. However, therapeutic management of BD needs to be introduced early in order to control inflammation, to preserve visual function and to limit irreversible structural damage. The aim of this review is to provide current data on how innovations in clinical evaluation, investigations and treatments were able to improve the prognosis of uveitis associated with BD.

Mucocutaneous involvement in behçet’s disease

2020 ◽  
Vol 5 (5) ◽  
pp. 01-03
Author(s):  
Z. alaya ◽  
R amri ◽  
A. fraj ◽  
I. chaabane ◽  
W. Garbouj ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Chronic Inflammatory Disease ◽  
Behcet's Disease ◽  
Male Predominance ◽  
Skin Ulcers ◽  
Systemic Manifestations ◽  
Inflammatory Drugs ◽  
Perianal Ulcers

Behçet's disease is a chronic inflammatory disease characterized by its clinical polymorphism associating mucocutaneous involvement to systemic manifestations. The mucocutaneous lesions are considered the hallmark of the disease, being the most common symptoms presenting at the onset of disease. Our objective was to determine the characteristics of this skin involvement during Behçet's disease. We conducted a descriptive study over a period of 30 years, having collected all patients with Behçet's disease. These were 98 patients. A male predominance was observed in our studied population with a Sex Ratio of 2.5. The mean age at diagnosis was 34 years. Mucocutaneous involvement was observed in all patients. Oral aphthosis was constant and genital ulcers, were observed in 81 cases. The other mucocutaneous manifestations were: pseudofolliculitis (61 cases), erythema nodosum (7 cases), skin ulcers (4 cases), acneiform lesions (2 cases), perianal ulcers (1 case), skin ulceration (1 case) and erythema multiforme. (1 case). All of our patients were treated with colchicine. Corticosteroids and non-steroidal anti-inflammatory drugs were each indicated in one case for resistant forms.

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.

scholarly journals Effectiveness and Safety of Chinese Medicine Decoctions for Behcet’s Disease: A Systematic Review and Meta-Analysis

2021 ◽  
Vol 2021 ◽  
pp. 1-19
Author(s):  
Jingxian Yan ◽  
Yi Yan ◽  
Andrew Young ◽  
Zhiyong Yan ◽  
Zhimin Yan
Keyword(s):  
Chinese Medicine ◽  
Adverse Events ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Meta Analysis ◽  
Clinical Feature ◽  
Behcet's Disease ◽  
Secondary Outcomes

Background. Behcet’s disease (BD) is an autoimmune disease of systemic vasculitis with an unclear pathogenesis. Although western medicines remain the mainstay interventions, effectiveness and safety are significant challenges. Complementary and alternative medicine, including herbal medicine, are gaining more attention. Chinese medicine decoctions, which have been used for centuries, are the most common form of traditional therapies. Objective. The purpose of the review was to evaluate the effectiveness and safety of Chinese medicine decoctions in the treatment of BD. Methods. Randomized controlled trials (RCTs) for BD treatment with Chinese medicine decoctions were searched in six electronic databases until March 2021. Primary outcomes were total effective rate, recovery rate, and recurrence rate. Secondary outcomes were clinical feature scores (oral ulcers, eye lesions, genital ulcers, skin lesions, arthropathies, fever, and pathergy reactions) and laboratory index levels (erythrocyte sedimentation rate, C-reactive protein, and immunoglobulin A). The risk of bias was assessed with the Cochrane Handbook, and a meta-analysis was performed with RevMan 5.4.1. Results. Sixteen RCTs with 924 patients were included in the review. The meta-analysis indicated that Chinese medicine decoctions were effective for BD when compared with control groups for all the primary outcomes and 7/10 of the secondary outcomes. Adverse events were reported in 11 of the 16 RCTs, with the Chinese medicine decoctions possibly having fewer adverse events than western drugs. This review included a range of classical prescriptions. An additional meta-analysis of modified Gancao Xiexin Decoction for BD treatment was conducted. Gancao Xiexin decoction is also discussed as a representative prescription, as well as high-frequency herbs, and warrants further exploration for individualized medicine and pharmacology. Conclusion. Chinese medicine decoctions have the potential to be effective and safe for treating BD. However, additional well-designed RCTs are needed to confirm the findings because of the unsatisfactory quality of the included studies.

scholarly journals Isomer-Specific Monitoring of Sialylated N-Glycans Reveals Association of α2,3-Linked Sialic Acid Epitope With Behcet’s Disease

2021 ◽  
Vol 8 ◽  
Author(s):  
Nari Seo ◽  
Hyunjun Lee ◽  
Myung Jin Oh ◽  
Ga Hyeon Kim ◽  
Sang Gil Lee ◽  
...  
Keyword(s):  
Sialic Acid ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Multiple Reaction Monitoring ◽  
High Sensitivity ◽  
High Specificity ◽  
Unknown Etiology ◽  
Behcet's Disease

Behcet’s disease (BD) is an immune disease characterized by chronic and relapsing systemic vasculitis of unknown etiology, which can lead to blindness and even death. Despite continuous efforts to discover biomarkers for accurate and rapid diagnosis and optimal treatment of BD, there is still no signature marker with high sensitivity and high specificity. As the link between glycosylation and the immune system has been revealed, research on the immunological function of glycans is being actively conducted. In particular, sialic acids at the terminus of glycoconjugates are directly implicated in immune responses, cell–cell/pathogen interactions, and tumor progression. Therefore, changes in sialic acid epitope in the human body are spotlighted as a new indicator to monitor the onset and progression of immune diseases. Here, we performed global profiling of N-glycan compositions derived from the sera of 47 healthy donors and 47 BD patients using matrix-assisted laser desorption/ionization-time of flight mass spectrometry (MALDI-TOF MS) to preferentially determine BD target glycans. Then, three sialylated biantennary N-glycans were further subjected to the separation of linkage isomers and quantification using porous graphitized carbon-liquid chromatography (PGC-LC)/multiple reaction monitoring (MRM)-MS. We were able to successfully identify 11 isomers with sialic acid epitopes from the three glycan compositions consisting of Hex5HexNAc4NeuAc1, Hex5HexNAc4Fuc1NeuAc1, and Hex5HexNAc4NeuAc2. Among them, three isomers almost completely distinguished BD from control with high sensitivity and specificity with an area under the curve (AUC) of 0.945, suggesting the potential as novel BD biomarkers. In particular, it was confirmed that α2,3-sialic acid at the terminus of biantennary N-glycan was the epitope associated with BD. In this study, we present a novel approach to elucidating the association between BD and glycosylation by tracing isomeric structures containing sialic acid epitopes. Isomer-specific glycan profiling is suitable for analysis of large clinical cohorts and may facilitate the introduction of diagnostic assays for other immune diseases.

scholarly journals COVID-19 ON BEHÇET’S DISEASE PATIENT : SHED LIGHT ON SHADOWS (CASE REPORT)

2020 ◽  
Vol 8 (11) ◽  
pp. 49-52
Author(s):  
Hanane Ezzouine ◽  
Mehdi Simou ◽  
Aymane El Louadghiri ◽  
Mounir Louardi ◽  
Imane Khaoury ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Disease Patient ◽  
Family Type ◽  
Behcet's Disease ◽  
New Family ◽  
Arterial Involvement ◽  
Shed Light

A new family type of coronavirus (SARS-CoV-2) was first seen in Wuhan, China name coronavirus disease 2019 (COVID-19). COVID-19 primarily attacks the respiratory system, but several studies have shown that infection with SARS-COV-2 can cause thrombosis and have therefore considered COVID-19 to be a prothrombotic disease. Behçet's disease (MB) is a systemic vasculitis. Its ethiopathogeny is still poorly understood. Blood vessels of any caliber can be affected. Arterial involvement, such as thrombosis and / or aneurysm, is possible. This article reports the case of SARS-COV-2 infection in a patient with behcet's disease.

scholarly journals Behcet’s Disease in an Adult Male From Nepal: A Case Report

2021 ◽  
Author(s):  
Madan Basnet ◽  
Abisha Phudong ◽  
Kamal Gautam ◽  
Bishnu Pathak ◽  
Suman Gaire ◽  
...  
Keyword(s):  
Case Report ◽  
Blood Vessels ◽  
Behçet’S Disease ◽  
Adult Male ◽  
Acute Inflammation ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Skin Lesions ◽  
Behcet's Disease

Behcet’s Disease is a rare systemic vasculitis characterized by recurrent episodes of acute inflammation affecting blood vessels of all sizes. Symptoms include orogenital apthosis, cutaneous skin lesions, and uveitis. We present the case of a 38-year-old Nepalese man with Behcet’s Disease. In Nepal, Behcet’s Disease may still be under-reported.

scholarly journals Behçet’s Disease and Tuberculosis: A Complex Relationship

2020 ◽  
Author(s):  
Sara Mendonça Freitas ◽  
Joana Silva Marques ◽  
Ana Grilo ◽  
Rodolfo Gomes ◽  
Fernando Martos Gonçalves
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Gastrointestinal Involvement ◽  
Behcet's Disease ◽  
History Of ◽  
Systemic Manifestations ◽  
Work Up

Behçet’s disease (BD) is a systemic vasculitis characterized by recurrent orogenital ulceration and several systemic manifestations (such as gastrointestinal involvement, vascular disease or arthritis). The pathogenesis is still unknown but the trigger role of certain pathogens such as Mycobacterium tuberculosis is well documented. Furthermore, patients with BD are more susceptible to tuberculosis due to immunity defects. Here, we describe the case of a 70-year-old woman with a history of recurrent oral aphthae and inflammatory arthritis presenting with extensive thrombosis of left upper limb major veins, a positive HLA B51 genotype and colon ulceration; hence, BD diagnosis was made after excluding other causes. Simultaneously, the patient had cutaneous abscesses not associated with immunosuppressive therapy with continuous development, and after recurrent negative tuberculosis work-up, M. tuberculosis was isolated in an abscess culture.

Behçet's Disease from Rheumatology and Neurology Perspectives with Special Reference to Children

2019 ◽  
Vol 18 (04) ◽  
pp. 175-181
Author(s):  
Yaşar B. Turgut ◽  
Ayşe F. Tosun ◽  
Beste K. Yüzbaşı ◽  
Hasan Tunca
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
International Study ◽  
Skin Lesions ◽  
Disease Classification ◽  
Behcet's Disease ◽  
Male Predominance ◽  
Pediatric Age Group ◽  
Pathergy Test

AbstractBehçet's disease (BD) is an idiopathic chronic relapsing multisystemic vascular inflammatory disease. Although it is generally seen in young adults with male predominance, the onset age of the disease is the pediatric age group in only 4 to 26% of all cases of BD. According to the International Study Group for Behçet's Disease Classification Criteria, a diagnosis of BD requires recurrent oral aphthous ulcerations with two of the following: genital ulcerations, skin lesions, eye lesions, or a positive pathergy test. In this article, we extensively reviewed the current literature with respect to the rheumatological and neurological findings of BD in childhood.

Epidemiology, Prevalence, Incidence in Behçet's Disease

2018 ◽  
pp. 321-323
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Skin Lesions ◽  
Medical Community ◽  
Aphthous Ulcer ◽  
Ocular Involvement ◽  
Behcet's Disease ◽  
Ancient Silk Road

Behçet’s disease (BD) was first brought to the attention of the modern medical community by Hulusi Behçet in 1937. The disease is a systemic vasculitis characterized by a recurrent aphthous ulcer, genital ulcer, uveitis, and skin lesions. It is observed more common and severe throughout the ancient Silk Road from East Asia to the Mediterranean. Ocular involvement is reported in 25-75% of patients with BD. In most cases, it may progress to blindness if left untreated. The aim of this review is to investigate the epidemiology, prevalence, and incidence of BD.

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