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 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 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 Immunopathogenesis of Ocular Behçet’s Disease

2014 ◽  
Vol 2014 ◽  
pp. 1-13 ◽  
Author(s):  
Un Chul Park ◽  
Tae Wan Kim ◽  
Hyeong Gon Yu
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Skin Lesions ◽  
Unknown Etiology ◽  
Ocular Involvement ◽  
Inflammatory Disorder ◽  
General Hypothesis ◽  
Behcet's Disease

Behçet’s disease (BD) is a chronic recurrent systemic inflammatory disorder of unknown etiology characterized by oral and genital ulcerations, skin lesions, and uveitis. The ocular involvement of BD, or Behçet’s uveitis (BU), is characterized by panuveitis or posterior uveitis with occlusive retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous autoimmune uveitides, despite aggressive immunosuppression. Although pathogenesis of BD is unclear, researches have revealed that immunological aberrations may be the cornerstone of BD development. General hypothesis of BD pathogenesis is that inflammatory response is initiated by infectious agents or autoantigens in patients with predisposing genetic factors and perpetuated by both innate and acquired immunity. In addition, a network of immune mediators plays a substantial role in the inflammatory cascade. Recently, we found that the immunopathogenesis of BU is distinct from other autoimmune uveitides regarding intraocular effector cell profiles, maturation markers of dendritic cells, and the cytokine/chemokine environment. In addition, accumulating evidence indicates the involvement of Th17 cells in BD and BU. Recent studies on genetics and biologics therapies in refractory BU also support the immunological association with the pathogenesis of BU. In this review, we provide an overview of novel findings regarding the immunopathogenesis of BU.

scholarly journals A case of intestinal Behcet’s disease under Adalimumab

2017 ◽  
Vol 4 (3) ◽  
pp. 62
Author(s):  
David T. Dulaney ◽  
Wassem Juakiem ◽  
Katherine Cebe ◽  
Angelo H. Paredes
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Immunosuppressive Agents ◽  
African American Female ◽  
Behcet's Disease ◽  
Oral Ulcers ◽  
Adalimumab Therapy ◽  
Tnfα Inhibitors ◽  
Tnfα Inhibitor

Behcet’s disease (BD) is a multisystem mucocutaneous inflammatory condition characterized by recurrent genital and oral ulcers, ocular inflammation, and can involve the gastrointestinal tract. Treatment involves the usage of immunosuppressive agents to control the disease with glucocorticoids utilized for treatment of flares. Tumor necrosis factor inhibitors are frequently used to control the disease as well. We present the case of a 40 years old African American female presenting with intestinal BD that was refractory to adalimumab therapy. In conjunction with glucocorticoids, the patient’s intestinal disease was controlled with infliximab therapy. Currently, there have been no studies comparing the efficacy of TNFα inhibitors on the treatment of BD. Future studies are needed to compare the efficacy of TNFα inhibitor agents in the treatment of intestinal manifestations of BD.

scholarly journals Detection of Herpes Virus Genomes in Skin Lesions from Patients with Behçet's Disease and Other Related Inflammatory Diseases

2003 ◽  
Vol 83 (2) ◽  
pp. 124-127 ◽  
Author(s):  
MICHIKO TOJO ◽  
XUEYI ZHENG ◽  
HIROKATSU YANAGIHORI ◽  
NORITAKA OYAMA ◽  
KAZUO TAKAHASHI ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Herpes Virus ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Inflammatory Diseases ◽  
Skin Lesions ◽  
Behcet's Disease ◽  
Herpès Virus ◽  
Virus Genomes

Recurrent vulval ulceration: could it be Behçet's disease?

2012 ◽  
Vol 23 (9) ◽  
pp. 683-684
Author(s):  
S Patel ◽  
K Prime
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Disease ◽  
Holistic Approach ◽  
Nucleic Acid Amplification ◽  
Behcet's Disease ◽  
Oral Ulcers ◽  
Caribbean Woman ◽  
History Of

This report describes a young black Caribbean woman with a three-year history of recurrent genital ulceration initially diagnosed as genital herpes. This diagnosis had been made clinically by the general practitioner but never confirmed on culture or nucleic acid amplification testing; sequential treatment with aciclovir and famciclovir over a four-month period did not alleviate her genital symptoms. Presentation to our genitourinary (GU) medicine clinic identified painful aphthous genital and oral ulcers, erythema nodosum bilaterally and a history of eye irritation. A clinical diagnosis of Behçet's disease was made. Treatment with hydrocortisone mouth pellets and dermovate ointment was initiated in addition to dermatology and ophthalmology referral. This report reminds clinicians to consider alternative diagnoses in clinical practice when faced with patients who are resistant to treatment. It also highlights the importance of recognizing systemic disease and maintaining a holistic approach when treating patients.

Characteristics of T cell lines established from skin lesions of Behcet's disease

1997 ◽  
Vol 15 (1) ◽  
pp. 9-13 ◽  
Author(s):  
Mitsuru Mochizuki ◽  
Eishin Morita ◽  
Shoso Yamamoto ◽  
Seizo Yamana
Keyword(s):  
T Cell ◽  
Cell Lines ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Skin Lesions ◽  
Behcet's Disease ◽  
T Cell Lines

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.

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