scholarly journals Past and Present Behçet’s Disease Animal Models

2020 ◽  
Vol 21 (16) ◽  
pp. 1652-1663
Author(s):  
Jermilia Charles ◽  
Francis J. Castellino ◽  
Victoria A. Ploplis
Keyword(s):  
Herpes Simplex ◽  
Animal Models ◽  
Transgenic Mice ◽  
Autoinflammatory Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Herpes Simplex Type ◽  
Unknown Etiology ◽  
Disease Phenotype ◽  
Hsv 1

Behçet’s disease (BD) is presumably an autoinflammatory disease of unknown etiology for which several animal models have been described over the years. Agents and methods used for the development of these models have ranged from the herpes simplex type one virus (hsv-1) pathogen to the use of transgenic mice. Other models have also been used to investigate a possible autoimmune component. Each model possesses its own unique set of benefits and shortcomings, with no one model fully being able to recapitulate the disease phenotype. Here, we review the proposed models and provide commentary on their effectiveness and usefulness in studying the disease.

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.

Clinical Findings, Diagnosis, and Classification of Behçet's Disease

2018 ◽  
pp. 329-333
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Clinical Findings ◽  
Unknown Etiology ◽  
Ocular Involvement ◽  
Behcet's Disease ◽  
Diagnosis And Classification

Behçet’s Disease (BD) is a chronic multisystem vasculitis with unknown etiology, which is characterized by the triad of recurrent oral ulcers, genital ulcers, and iritis. The eye is one of the most commonly affected organs and typical ocular involvement is panuveitis with hypopyon and retinal vasculitis. The diagnosis is based on clinical evaluation including biomicroscopic and funduscopic examination. This article aims to review the clinical findings, diagnosis, and classification of BD.

scholarly journals Pulmonary embolism in Behcet’s disease: a case report

2020 ◽  
Vol 90 (4) ◽  
Author(s):  
Despoina Moumtzi ◽  
Marianna Kakoura
Keyword(s):  
Pulmonary Embolism ◽  
Differential Diagnosis ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Rare Complication ◽  
Pulmonary Involvement ◽  
Unknown Etiology ◽  
Disease Exacerbation ◽  
Behcet's Disease

Behcet’s disease (BD) is a vasculitis of unknown etiology. It is often correlated with thrombophilic factors such as V Leiden. Pulmonary involvement is reported in 1-10% of patients. The most common manifestations are pulmonary aneurysms while pulmonary embolism is a rare complication. A 41-year old man with BD and V Leiden heterozygosity complained of pleurodynia and fever. Pleurodynia deteriorated in the following days and PE was confirmed by CT angiography, without the presence of aneurysms. After the exclusion of the antiphospholipid syndrome, a therapeutic dose of apixaban was initiated. Two weeks later, pleurodynia relapsed in combination with pleural effusion unilaterally. These findings were attributed to disease exacerbation. For this reason, we decided to enhance the immunosuppressive therapy. Six months later, CTPA showed complete remission of the clots. Vasculitis predisposes to thrombosis with or without coexisting thrombophilia. Clinicians should include them in their differential diagnosis and provide personalized treatment, based on immunosuppressants.

scholarly journals The characteristic site-specific reactivation phenotypes of HSV-1 and HSV-2 depend upon the latency-associated transcript region.

1996 ◽  
Vol 184 (2) ◽  
pp. 659-664 ◽  
Author(s):  
T Yoshikawa ◽  
J M Hill ◽  
L R Stanberry ◽  
N Bourne ◽  
J F Kurawadwala ◽  
...  
Keyword(s):  
Herpes Simplex Virus ◽  
Herpes Simplex ◽  
Animal Models ◽  
Virus Type ◽  
Herpes Simplex Virus Type ◽  
Anatomic Site ◽  
Simplex Virus ◽  
Intertypic Recombinant ◽  
Hsv 1

After replication at sites of initial inoculation, herpes simplex virus type 1 and 2 (HSV-1 and HSV-2) establish lifelong latent infections of the sensory and autonomic neurons of the ganglia serving those sites. Periodically, the virus reactivates from these neurons, and travels centripetally along the neuronal axon to cause recurrent epithelial infection. The major clinically observed difference between infections with herpes simplex virus type 1 and type 2 is the anatomic site specificity of recurrence. HSV-1 reactivates most efficiently and frequently from trigeminal ganglia, causing recurrent ocular and oral-facial lesions, while HSV-2 reactivates primarily from sacral ganglia causing recurrent genital lesions. An intertypic recombinant virus was constructed and evaluated in animal models of recurrent ocular and genital herpes. Substitution of a 2.8-kbp region from the HSV-1 latency-associated transcript (LAT) for native HSV-2 sequences caused HSV-2 to reactivate with an HSV-1 phenotype in both animal models. The HSV-2 phenotype was restored by replacing the mutated sequences with wild-type HSV-2 LAT-region sequences. These sequences or their products must act specifically in the cellular environments of trigeminal and sacral neurons to promote the reactivation patterns characteristic of each virus.

scholarly journals Behcet’s Disease with Intracardiac Thrombus Presenting with Fever of Unknown Etiology

2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
Sajal Ajmani ◽  
Durga Prasanna Misra ◽  
Deep Chandh Raja ◽  
Namita Mohindra ◽  
Vikas Agarwal
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Thrombus Formation ◽  
Young Male ◽  
Unknown Etiology ◽  
High Dose ◽  
Intracardiac Thrombus ◽  
High Grade Fever ◽  
Behcet's Disease

A young male was referred to us for evaluation of fever of unknown origin (FUO). He had history of recurrent painful oral ulcers for one year and moderate to high grade fever, pustulopapular rash, and recurrent genital ulcers for 6 months and hemoptysis for 3 days. He was detected to have intracardiac thrombi and pulmonary arterial thrombosis along with underlying Behcet’s disease (BD). Patient responded to high dose prednisolone (1 mg/Kg/day) along with monthly parenteral cyclophosphamide therapy. This case highlights the fact that BD is an important cause for pulmonary artery vasculitis with intracardiac thrombus formation, and such patients can present with FUO.

scholarly journals Circulating herpes simplex type 1 (HSV-1)-specific CD8+ T cells do not access HSV-1 latently infected trigeminal ganglia

Herpesviridae ◽  
2011 ◽  
Vol 2 (1) ◽  
pp. 5 ◽  
Author(s):  
Susanne Himmelein ◽  
Anthony J St Leger ◽  
Jared E Knickelbein ◽  
Alexander Rowe ◽  
Michael L Freeman ◽  
...  
Keyword(s):  
T Cells ◽  
Herpes Simplex ◽  
Cd8 T Cells ◽  
Herpes Simplex Type ◽  
Trigeminal Ganglia ◽  
Latently Infected ◽  
Hsv 1

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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