scholarly journals Gender Differences in Behçet’s Disease Associated Uveitis

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Didar Ucar-Comlekoglu ◽  
Austin Fox ◽  
H. Nida Sen
Keyword(s):  
Gender Differences ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Vascular Involvement ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Male Patients

Behçet’s disease is a systemic vasculitis of unknown etiology, characterized by oral and genital ulceration, skin lesions, and uveitis as well as vascular, central nervous system, and gastrointestinal system involvement. It is prevalent in the Middle East, Mediterranean, and Eastern Asia. The aim of this review is to evaluate the gender differences in clinical manifestations of Behçet’s disease, treatment responses, mortality, and morbidity. Behçet’s disease has been reported to be more prevalent in males from certain geographic regions and particular ethnic groups; however, recent reports indicate more even gender distribution across the world. There are gender differences in clinical manifestations and severity of the disease. Ocular manifestations, vascular involvement, and neurologic symptoms are more frequently reported in male patients whereas oral and genital ulcers, skin lesions, and arthritis occur more frequently in female patients. The disease can have a more severe course in males, and overall mortality rate is significantly higher among young male patients.

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.

Vascular manifestations of Behçet's disease: report of a case and review of the literature

2011 ◽  
Vol 26 (6) ◽  
pp. 249-253 ◽  
Author(s):  
T Kotsis ◽  
K G Moulakakis ◽  
S Mylonas ◽  
V Andrikopoulos
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Inferior Vena ◽  
Recent Literature ◽  
Behçet's Disease ◽  
Vena Cava ◽  
Clinical Manifestations ◽  
Vascular Involvement ◽  
Thrombotic Occlusion ◽  
Behcet's Disease

Adamantiades–Behçet's disease (ABD) is a relapsing vasculitis of unknown aetiology and variable clinical manifestations. The syndrome can be presented in a myriad of ways and can involve nearly every organ. Although vascular involvement is not included among the ABD diagnostic criteria, it is a unique clinical manifestation in adults with a potentially devastating outcome. We report an ABD case, presenting with a thrombotic occlusion of the inferior vena cava. The authors review the recent literature, emphasizing the spectrum of vascular manifestations accompanying Behçet's disease.

scholarly journals Mucocutaneous Involvement in Behçet’s Disease: How Systemic Treatment Has Changed in the Last Decades and Future Perspectives

2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Cinzia Rotondo ◽  
Giuseppe Lopalco ◽  
Florenzo Iannone ◽  
Antonio Vitale ◽  
Rosaria Talarico ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Current State ◽  
Life Threatening ◽  
Symptom Complex ◽  
Appropriate Therapy

Behçet’s disease (BD) is a multisystemic disorder of unknown etiology characterized by the “triple symptom complex” consisting of recurrent oral aphthosis, genital ulcers, and chronic relapsing bilateral uveitis. Recurrent mucocutaneous lesions are generally considered the hallmark of the disease, being the most common symptoms presenting at the onset of disease. Although the improvement of knowledge about the pathogenetic mechanism added important changes in the treatment management of BD clinical manifestations, thus avoiding the appearance of serious life-threatening complications which are disease related, the mucocutaneous lesions are still the most nagging clinical manifestations to be treated. In this work we reviewed the current state of knowledge regarding the therapeutic approaches for mucocutaneous lesions of BD mainly based on controlled studies to provide a rational framework for selecting the appropriate therapy for treating these troublesome features of the disease.

scholarly journals Pathophysiology of the Behçet's Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-11 ◽  
Author(s):  
Ümit Türsen
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Vascular Involvement ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Multisystemic Disease ◽  
And Function ◽  
Systemic Associations ◽  
Immunological Alterations

Behçet's disease (BD) is a multisystemic disease of unknown etiology characterized by chronic relapsing oral-genital ulcers and uveitis. Multiple systemic associations including articular, gastrointestinal, cardiopulmonary, neurologic, and vascular involvement are also observed in BD. Although the etiopathogenesis of the disease remains unknown, increased neutrophil functions such as chemotaxis, phagocytosis, and excessive production of reactive oxygen species (ROS), including superoxide anion, which may be responsible for oxidative tissue damage seen in BD, and also immunological alterations, T lymphocyte abnormalities in both subpopulation and function have been considered to be correlated with the etiopathogenesis of BD. There is some clinical evidence suggesting that emotional stress and hormonal alterations can influence the course and disease activity of BD.

scholarly journals Behçet’s disease: Intracardiac thrombosis (a description of two cases and a review of literature)

2017 ◽  
Vol 89 (5) ◽  
pp. 79-82
Author(s):  
Z S Alekberova ◽  
P S Ovcharov ◽  
T A Lisitsyna ◽  
A V Volkov ◽  
T V Popkova
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Vena Cava ◽  
Skin Lesions ◽  
Left Ventricular ◽  
Aphthous Stomatitis ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Intracardiac Thrombosis

Behçet’s disease (BD) is systemic vasculitis of unknown etiology, which is more common in the countries located along the Great Silk Road. The disease is diagnosed if a patient has 4 key diagnostic signs: aphthous stomatitis, genital sores, and eye and skin lesions. Vascular diseases referred to as minor criteria for BD are characterized by the formation of aneurysms and thrombosis, predominantly in the venous bed. In venous disorders, a blood clot can form in any vessel, including caval, cerebral, pulmonary, and other veins. The paper describes two clinical cases of BD with intracardiac thrombosis. In one case, a 24-year-old male patient with a documented diagnosis of BD, echocardiography revealed a left ventricular spontaneous echo contrast phenomenon that disappeared due to immunosuppressive therapy. The other case was a 34-year-old female patient, in whom the diagnosis was based on the international disease criteria: aphthous stomatitis, skin lesions (pseudopustulosis, erythema nodosum), and genital sores. Computed tomographic angiography showed a 3.7×2.2-cm mass (thrombus) in the right atrium. In addition, blood clots were present in the hepatic and inferior vena cava. No abnormalities in the coagulation system were found in both cases.

scholarly journals Atypical Vascular Involvement in a Case of Behçet's Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-5
Author(s):  
Alejandro Rodríguez Morata ◽  
Ana Hidalgo Conde ◽  
Carlos de la Cruz Cosme ◽  
Susana Gómez Ramírez ◽  
Rafael Gómez Medialdea
Keyword(s):  
Venous Thrombosis ◽  
Behçet’S Disease ◽  
Cerebral Venous Thrombosis ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Young Patients ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Vascular Intervention ◽  
Behcet's Disease

Introduction. Behçet's disease (BD) is a form of vasculitis of unknown etiology which is rare in our environment. It is characterized by a variety of clinical manifestations and usually affects young adults. Recurrent oral and genital ulcers are a characteristic and extremely frequent symptom, but mortality is linked with more significant symptoms such as aortic pseudoaneurysm, pulmonary pseudoaneurysm, and cerebral venous thrombosis.Patient and Method. We present a case of a young male with atypical BD and severe polyvascular involvement (previous cerebral venous thrombosis and current peripheral venous thrombosis, acute ischemia, and peripheral arterial pseudoaneurysm) who required urgent surgical intervention due to a symptomatic external iliac pseudoaneurysm.Result. The pseudoaneurysm was successfully treated, we performed an iliofemoral bypass, and we treated it with steroids and immunosuppressive therapy.Conclusions. These rare clinical manifestations highlight the importance of considering BD in young patients, even in usual cases of vascular intervention, whether arterial or venous in nature.

scholarly journals Isolated Aortitis with multiple Aortic aneurysms and widespread venous, arterial and intracardiac thrombosis: A challenging Vascular Behcet’s Disease case management

2021 ◽  
pp. 195
Author(s):  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Sudden Onset ◽  
Aortic Aneurysms ◽  
Behcet's Disease ◽  
Disease Case ◽  
Intracardiac Thrombosis

In 1937, Hulusi Behcet’s brought to our attention the existence of a disease characterized by recurrent oral aphthae and any of several systemic manifestations including genital aphthae, ocular disease, skin lesions, gastrointestinal involvement, neurologic disease, vascular disease, or arthritis [1-3]. He became the first physician to describe this and the disease was henceforth named after his name. The aetiology of this disease remains unclear however it has been noticed that Behcet’s syndrome is remarkable for its systemic vasculitides which include its ability to involve blood vessels of all sizes (small, medium, and large) on both the arterial and venous sides of the circulation with occlusive nature [4]. This makes Vasculitis the major cause of the clinical manifestations of the disease with the involvement of almost any organ. We herein report an unusual case of a thirty-year-old Syrian man with no past medical illnesses. On presentation, he suffered from a sudden onset of shortness of breath and a history of hoarseness of voice for less than a week, and drastic weight loss of about thirty kgs within a month. Imaging confirmed the presence of pulmonary embolism, bilateral deep venous thrombosis, intracardiac thrombosis, popliteal artery thrombosis with thoracic, and abdominal aortic aneurysms. This case highlights the importance of having a high suspicion of the possibility of Behcet’s disease when multiple thrombi and aneurysms manifest in a patient on the silk root country. Thereby, stressing the importance of familiarizing ourselves with such unusual presentations of Behcet’s disease and the course of this unusual presentation with some learning points with a good literature review included below.

scholarly journals Severe Behçet’s disease equally affects both genders in Egyptian patients: A multicentre retrospective follow-up study

Reumatismo ◽  
2020 ◽  
Vol 71 (4) ◽  
pp. 218-225
Author(s):  
D.H.S. Attia ◽  
R.A. Abdel Noor
Keyword(s):  
Gender Differences ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Vascular Involvement ◽  
Venous Disease ◽  
Female Ratio ◽  
Behcet's Disease ◽  
Organ Systems ◽  
Egyptian Patients

Behçet’s disease is a relapsing multisystemic disease. Its highest prevalence is seen along the Silk Road. While several studies reported gender disparities, others didn’t. Scarce data are available about the Arabs and the gender differences detected in some ethnicities could not be applied to others. Our study aimed to detect gender differences among a cohort of adult Egyptian patients with Behçet’s disease. Medical files of 255 adult patients diagnosed with Behçet’s disease at the Rheumatology and Rehabilitation Department of Kasr Al-Aini Hospital, Cairo University and the Internal Medicine Department of Tanta University, between 2002 and 2018, were retrospectively reviewed. The demographic features, the cumulative clinical features, the use, if any, of an intravenous pulse of methylprednisolone and immunosuppressive/biological drugs, and the frequency of the cumulative damage to the different organ systems were described. The disease severity score was calculated as well. The study revealed a prominent male predominance; the male to female ratio was 6.7:1. Acne/pseudofolliculitis was more common in males (28.6% vs 13.2%, p=0.046); the same was observed regarding any vascular involvement and peripheral venous disease (36.3% vs 18.4%, p=0.03 and 30.4% vs 13.2%, p=0.03, respectively). On the other hand, encephalitis and cranial nerve lesions were more prevalent in females (15.8% vs 6%, p=0.03 and 10.5% vs 3.2%, p=0.04, respectively). Severe Behçet’s disease equally affects Egyptian males and females. BD should not be considered a benign disease in Egyptian females. The same quality of medical care should be equally offered to both genders.

scholarly journals Behçet’s disease in clinical practice

2013 ◽  
Vol 94 (4) ◽  
pp. 545-548
Author(s):  
R F Khamitov ◽  
L Y Palmova ◽  
Z N Yakupova ◽  
E R Khasanova
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Clinical Manifestations ◽  
Specific Treatment ◽  
Skin Lesions ◽  
Female Ratio ◽  
Behcet's Disease ◽  
Pathergy Test

Behçet’s disease is a chronic relapsing multisystemic vasculitis involving vessels of different diameter. The disease is characterized by involvement of skin and mucosa, eyes, gastrointestinal tract, joints, vessels, genitourinary system and neurological symptoms. According to data, male/female ratio is 2-10:1 The etiology of the disease is still unclear. Diagnosis is mainly based on thorough analysis of clinical manifestations. According to the International Study Group for Behçet’s Disease Diagnostic Criteria, combination of recurrent oral aphthosis and at least any two of the following: severe or scarring genital aphthosis, eye lesions (posterior uveitis, retinal vasculitis), skin lesions (nodular erythema, pseudofolliculitis, acne-like lesions) and positive pathergy test is needed to classify the patient as a patient with Behçet’s disease. Several clinical subtypes of Behçet’s disease are distinguished depending on predominant involvement of different organs and systems, including skin and mucosa, joints, eyes, or neurological manifestations. There are no specific laboratory tests for Behçet’s disease. The treatment approaches are variable depending on clinical manifestations. The effect of systemic corticosteroids, colchicine, azathioprine, chlorambucil, cyclophosphamide, sulfasalazine, methotrexate, cyclosporine, infliximab was proven. All the abovementioned requires the good clinical knowledge on Behçet’s disease from practicing doctors of different specialties, particularly - physicians. A case of highly active acute Behçet’s disease with nose, pharynx, eye and oral cavity mucosa, scrotum skin involvement is presented in the article. Questions of differential diagnosis are reviewed, specific treatment program with efficacy analysis is presented.

scholarly journals Pulmonary Nodules as an Initial Manifestation of Behçet’s Disease

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
M. Malekmohammad ◽  
A. Emamifar
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Artery Aneurysm ◽  
Skin Lesions ◽  
Vascular Involvement ◽  
Recurrent Fever ◽  
Initial Symptom ◽  
Initial Manifestation ◽  
Behcet's Disease

Behçet’s disease (BD) is a systemic vasculopathy, characterized by recurrent oral aphthae, genital ulcers, uveitis, and skin lesions. Although vascular involvement, including venous and arteries of any size, is a usual manifestation, cases with pulmonary thrombosis as the initial symptom are not common in the absence of pulmonary artery aneurysm (PAA). This report describes a 36-year-old man with recurrent fever, nonmassive hemoptysis, and persistent cough with lung nodules in CT scan who had undergone open lung biopsy. On the basis of morphological findings, BD was suggested and more precise evaluation confirmed the diagnosis.

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