Intracardiac thrombus in Behçet’s disease

Behçet’s disease (BD) is a multisystem inflammatory disorder. Intracardiac thrombus (ICT) formation is an uncommon but important complication of BD. Of the cases of Behçet’s disease, we selected those with ICT. All patients fulfilled the diagnostic criteria of the International Study Group of Behçet’s disease. The ICT in each case was confirmed by ultrasonography, computed tomography and MRI. Clinical features and laboratory parameters were determined. Among our 518 patients with BD, 8 were diagnosed as having intracardiac thrombus (ICT). All were male; the mean age at the time of the ICT diagnosis was 30.8 years. The main presenting symptoms were hemoptysis, chest pain, and dyspnea. It was associated with pulmonary artery aneurysm and vena cava thrombosis in 3 cases each, pulmonary embolism, and lower limbs deep venous thrombosis in 1 case each. The coexistence of other cardiac complications was as follows: pericarditis in 2 cases, myocarditis, endomyocardial fibrosis, and coronary arteritis with consequent myocardial infarction in one case each. In all cases, echocardiography was sufficient to reach the diagnosis. Chest computed tomography performed in all cases led to the diagnosis of associated pulmonary vasculo-Behçet lesions in 4 cases. All patients received colchicine, anticoagulation, and corticosteroids. Seven patients were on immunosuppressant agents (2 patients received azathioprine and 5 cyclophosphamide). Clinical remission with ICT resolution was observed in 5 cases. Combined immunosuppressive therapy with prednisone and cyclophosphamide might be needed to treat ICT due to BD.

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Computed tomography pulmonary angiography (CTPA) in Behçet’s disease patients: a remarkable gender gap and time to refine the treatment strategy

Clinical Rheumatology ◽

10.1007/s10067-021-05991-2 ◽

2021 ◽

Author(s):

Samar Tharwat ◽

Suzan S. ElAdle ◽

Abdel Hafeez Moshrif ◽

Faten Ismail ◽

Rawhya El-Shereef ◽

...

Keyword(s):

Computed Tomography ◽

Behçet’S Disease ◽

Gender Gap ◽

Treatment Strategy ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Pulmonary Angiography ◽

Computed Tomography Pulmonary Angiography ◽

Behcet's Disease

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Immunopathogenesis of Ocular Behçet’s Disease

Journal of Immunology Research ◽

10.1155/2014/653539 ◽

2014 ◽

Vol 2014 ◽

pp. 1-13 ◽

Cited By ~ 11

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.

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Intracardiac thrombus in a child with behçet’s disease

Pediatric Rheumatology ◽

10.1186/1546-0096-12-s1-p365 ◽

2014 ◽

Vol 12 (S1) ◽

Author(s):

Selçuk Yüksel ◽

Tülay Becerir ◽

Havva Evrengül ◽

Ali Koçyiğit ◽

Yasemin Işık Balcı ◽

...

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Intracardiac Thrombus ◽

Behcet's Disease

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

Case Reports in Immunology ◽

10.1155/2015/149359 ◽

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.

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A Darwinian view of Behçet's disease

Rheumatology and Immunology Research ◽

10.2478/rir-2021-0013 ◽

2021 ◽

Vol 2 (2) ◽

pp. 91-99

Author(s):

Rhodri Smith ◽

Robert J. Moots ◽

Mariam Murad ◽

Graham R. Wallace

Keyword(s):

Behçet’S Disease ◽

Complex Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Clinical Findings ◽

Unknown Etiology ◽

Inflammatory Disorder ◽

Protective Mechanisms ◽

Behcet's Disease ◽

Cns Lesions

Abstract Behçet’s disease (BD) is a multisystem inflammatory disorder of unknown etiology, characterized by oral and genital ulceration, with other complications including eye, skin, joint, and central nervous system (CNS) lesions. Diagnosis is based on clinical findings, which may differ between patients. There is a strong genetic basis for BD; however, only a few genes have been associated with the disease across the geographical spread of BD. In this article, we discuss the history and combination of genes involved in this complex disease in relation to the geographical range and present our view that the disease has developed from a Darwinian perspective, with different gene polymorphisms that affect the same biological pathway. Moreover, these mutations individually are protective mechanisms against the disease relevant to each region, which affected both archaic and modern humans.

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Neuro-Behçet’s Disease – Clinical Features, Diagnosis and Differential Diagnosis

European Neurological Review ◽

10.17925/enr.2018.13.2.93 ◽

2018 ◽

Vol 13 (2) ◽

pp. 93

Author(s):

Selen Ozyurt ◽

Petros Sfikakis ◽

Aksel Siva ◽

Cris S Constantinescu ◽

◽

...

Keyword(s):

Differential Diagnosis ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Case Reports ◽

Illustrative Case ◽

Inflammatory Disorder ◽

Behcet's Disease ◽

Multisystem Involvement ◽

The Central Nervous System

Background:Behçet’s disease is a relatively uncommon, inflammatory disorder with characteristic mucocutaneous lesions and multisystem involvement, of unknown aetiology; presumably a vascular autoinflammatory syndrome that develops under combined environmental and genetic influences. As neuro-Behçet’s disease affects the central nervous system in about 10% of cases and in ways that can mimic other neuroinflammatory conditions, awareness of its manifestations, significance, and management is important for neurologists. In March 2017, a mini-symposium at the 11th Congress of Controversies in Neurology in Athens, Greece, was dedicated to specific aspects of Behçet’s and neuro-Behçet’s disease. These included an introduction to Behçet’s disease, pathogenesis and treatment, an overview of its neurological manifestations (neuro-Behçet’s disease) and the differential diagnosis from other neuroinflammatory conditions. Illustrative case reports were used.Objectives:To provide a brief overview of neuro-Behçet’s disease that is informative for clinical neurological practice and that follows the structure of the 2017 mini-symposium.Data sources:Relevant recent comprehensive reviews of the subject and relevant original articles and case reports were provided by each speaker at the mini-symposium. This article contains some of these sources and some additions where necessary to emphasise specific points. References are also provided for more comprehensive recent reviews.Limitations:The mini-symposium was an opportunity for providing a brief update and overview of neuro-Behçet’s disease and to exchange ideas and experience among neurologists. As such, it was found to be helpful, but also limited in scope. This resultant article refers to comprehensive reviews on the topic but is not in itself a comprehensive systematic review.Conclusions:Neuro-Bechet’s disease comprises largely two forms, parenchymal and a non-parenchymal. These manifestations seldom overlap in the same individual and may reflect different pathogenetic mechanisms. The principles of treatment largely follow the principles of treating Bechet’s disease in general, with the mainstay being corticosteroids for exacerbations and immunosuppressive treatments for prevention of exacerbations. One notable exception is cyclosporine, which is typically avoided in neuro-Bechet’s disease. Anti-tumour necrosis factor biologicals play an increasing role in treatment. Distinguishing neuro-Behçet’s disease from other neuroinflammatory conditions, such as multiple sclerosis, is essential for both management and prognostic reasons.

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Decreasing incidence and severity of Behçet’s disease: a changing trend in epidemiological spectrum possibly associated with oral health

TURKISH JOURNAL OF MEDICAL SCIENCES ◽

10.3906/sag-2003-147 ◽

2020 ◽

Vol 50 (7) ◽

pp. 1587-1590

Author(s):

Gonca MUMCU ◽

Fatma ALİBAZ ÖNER ◽

Tülin ERGUN ◽

Haner DİRESKENELİ

Keyword(s):

Oral Health ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Epidemiological Studies ◽

Inflammatory Disorder ◽

Behcet's Disease ◽

Disease Spectrum ◽

Wide Range ◽

Ancient Silk Road

Behçet’s disease (BD) is a systemic and inflammatory disorder that is mainly present along the ancient Silk Road, from the Mediterranean Sea to East Asia. A wide range of prevalence figures (0.1–420/100,000) have been reported for BD, also among Turkish populations of similar genetic background living in different countries. Recently, a decline in the incidence of BD and a change of the disease spectrum to less-severe manifestations have been reported from Japan and Korea, two genetically homogenous, affluent populations with limited immigration. It was hypothesized that a decline in infectious diseases, especially dental/periodontal infections, associated with the improvement in oral health, could be a part of these changes in the disease expression. Further epidemiological studies in other populations might demonstrate whether there is a worldwide similar trend and may provide a better understanding of the triggering factors for the onset and course of BD.

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