0274: Cardiac involvement in Behcet’s disease

Behçet’s disease is a multisystem chronic inflammatory disease generally presenting as recurrent oral-genital ulcers and uveitis. Vascular involvement is a common presentation. However, cardiac involvement is rare in this disorder. In this case report, we present a large right ventricular thrombus detected both in computed tomography and echocardiography in a 32 year-old male patient with complaints of fever, haemoptysis and weight loss. Intracardiac thrombus showed both clinical and radiological regression with oral anticoagulant and immunosuppressive therapy.

Download Full-text

Cardiac Manifestations in Iranian Patients with Behçet’s Disease

The Journal of Tehran University Heart Center ◽

10.18502/jthc.v16i3.8187 ◽

2022 ◽

Author(s):

Soraya Shadmanfar ◽

Maryam Masoumi ◽

Fereydoun Davatchi ◽

Massoomeh Akhlaghi ◽

Seyedeh Tahereh Faezi ◽

...

Keyword(s):

Myocardial Infarction ◽

Heart Disease ◽

Ischemic Heart Disease ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Cardiac Involvement ◽

Behçet's Disease ◽

Ischemic Heart ◽

Behcet's Disease ◽

Cardiac Manifestations

Background: Behcet’s disease (BD) is a vasculitis with multisystem and multiorgan involvement. Cardiac involvement in BD is a rare complication with a poor outcome that manifests itself in different forms. In this study, we aimed to investigate cardiac involvement in BD. Methods: This is a retrospective study based on cardiac manifestations in BD according to the data of the Behçet’s Disease Unit, the Rheumatology Research Center, Tehran University of Medical Sciences, from registered patients from 1975 until June 2017. Cardiac manifestations consisted of pericarditis, myocardiopathy, myocardial infarction, stable ischemic heart disease, endomyocardial fibrosis, thrombosis, and valvular and coronary involvement. All the patients’ baseline and demographic data were recorded in a designed questionnaire. The laboratory workups, imaging, and pathological tests were also performed. Results: We studied 7650 patients with BD, of whom 51% were male. In the entire study population, 47 patients manifested cardiac involvement: valvular involvement in 6.1%, myocardial infarction in 23.4%, stable ischemic heart disease in 20%, pericarditis in 21.3%, intracardiac thrombosis in 2.1%, coronary aneurysm in 2.1%, heart failure in 12.8%, and dilated cardiomyopathy in 4.3%. Conclusion: The prevalence of cardiac involvement in our patients with BD was 0.6%. A multidisciplinary approach can reduce mortality and morbidity rates. Consequently, we suggest that echocardiography and other cardiac diagnostic tests be routinely considered for early diagnosis and subsequent treatment.

Download Full-text

Pediatric Behcet's Disease Patient with Cardiac Valvular Lesions: case report and a review of the literature

10.21203/rs.3.rs-1017088/v1 ◽

2021 ◽

Author(s):

Xin Yao ◽

Xin-Ning Wang ◽

Zhi-Xuan Zhou ◽

Sheng-Nan Li ◽

Jianming Lai

Keyword(s):

Surgical Treatment ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Cardiac Involvement ◽

Behçet's Disease ◽

Disease Patient ◽

Close Monitoring ◽

Primary Therapy ◽

Behcet's Disease ◽

Valvular Lesions

Abstract Background To explore the clinical characteristics, treatment regimen, and prognosis of cardiac valvular involvement in pediatric Behcet's disease. Methods Retrospective analysis was conducted on the clinical data obtained from one pediatric patient suffering from Behcet's disease with cardiac valvular involvement. Present study also reviewed the literature related to cardiac involvement in pediatric Behcet's disease. Results The disease onset in the female patient was reported at the age of 3 years, with clinical symptoms, including apthous ulcers, fever, perianal ulcers, and erythema nodosum, accompanied by a significant increase in the inflammatory markers. Echocardiography indicated that aortic valve (AV) lesions, which were absent initially, developed later, gradually exacerbated. Initially, she was diagnosed as "infectious disease" and "systemic inflammatory response syndrome", and antibiotic therapy proved ineffective. After an accurate diagnosis of Behcet's disease, the patient was given glucocorticoids, immunosuppressants, and biologics, which resulted in the relief of the symptoms; however, cardiac valvular lesions continued to exacerbate. Subsequent treatment included medical treatment and surgical treatment, and the patient's condition was stable at follow-up. Conclusions Cardiac valvular lesions in pediatric Behcet's disease are rare with insidious onset, enhancing the propensity of misdiagnosis or missed diagnosis. Main manifestations include recurrent oral ulceration, skin lesions, and fever. Cardiac involvement may occur early, yet the symptoms are insidious, requiring comprehensive physical examination and close monitoring of echocardiography. Glucocorticoids combined with immunosuppressants are the primary therapy for cardiac involvement in Behcet's disease. When cardiac valvular damage and dysfunction are observed, treatment regimens can be jointly recommended by multidisciplinary consultation to conduct the surgical treatment on time.

Download Full-text

SAT0276 CARDIAC MAGNETIC RESONANCE IMAGING IN PATIENTS WITH BEHCET’S DISEASE: A PILOT STUDY

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.5232 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1082.1-1083

Author(s):

A. A. Ahmed ◽

S. Tharwat ◽

N. M. Batouty ◽

S. D. Abd El-Halim

Keyword(s):

Magnetic Resonance Imaging ◽

Right Ventricular ◽

Behçet’S Disease ◽

Cardiac Mri ◽

Behcet’S Disease ◽

Cardiac Involvement ◽

Behçet's Disease ◽

Left Ventricular ◽

Resonance Imaging ◽

Behcet's Disease

Background:Behçet’s disease is a multisystemic vasculitis characterized by mucocutaneous, ocular, arthritic, and vascular manifestations.[1] However, the incidence and nature of cardiac involvement in Behcet’s disease have not been clearly documented yet.Objectives:This study aimed to assess the cardiac involvement in patients with Behçet’s disease using cardiac magnetic resonance imaging (MRI).Methods:This cross -sectional descriptive study was carried out on thirty consecutive patients with Behçet’s disease (21 males,9 females) with mean age 32.3±8.9 years and with no evidence of cardiac disease. They underwent cardiac MRI to determine morphological and functional changes of the heart and to detect areas of hyperenhancement after IV administration of gadolinium.Results:At least one abnormality on cardiac MRI was observed in 20/30 patients (66.67%). Myocardial oedema was observed in 3 patients (10.0%) and late gadolinium enhancement in only 1 patient (3.3%). Pericardial effusion was found in 3 patients (10.0 %), global hypokinesia in 6 patients (20.0%) and intra-cardiac thrombosis in only 1 patient (3.3%). Pulmonary artery was dilated in 4 patients (13.3%). Left ventricular and right ventricular end diastolic volume were altered in 4 patients (13.3 %) and 7 patients (23.3%) respectively. Also, left ventricular and right ventricular end systolic volume were abnormal in7 patients (23.3%) and 5 patients (16.7%) respectively. Moreover, there was aortic valve regurge in 2 patients (6.7%), tricuspid valve regurge in 9 patients (30%), and mitral valve regurge in 9 patients (30%).Dilated left main coronary artery in 2 patients (6.7%) and arythmogenic right ventricular dysplasia in only one patient 1 patient (3.3%).Conclusion:Behçet’s disease may cause cardiac abnormalities without clinical manifestations and cardiac MRI may represent a tool for early detection of these subtle abnormalities.References:[1]Geri, G., et al., Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature. Medicine, 2012.91(1): p. 25-34.Figure 1.Cardiac MRI in 32 year-old female patient with Behçet’s disease for 3 years.Acknowledgments: :The authors would like to acknowledge Ahmed El Bahy for his assistance with this studyDisclosure of Interests:None declared

Download Full-text

Cardiac involvement and superior vena caval obstruction in Behcet's disease.

Thorax ◽

10.1136/thx.33.3.375 ◽

1978 ◽

Vol 33 (3) ◽

pp. 375-377 ◽

Cited By ~ 9

Author(s):

N Roguin ◽

S Haim ◽

R Reshef ◽

E Peleg ◽

E Riss

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Cardiac Involvement ◽

Behçet's Disease ◽

Superior Vena ◽

Superior Vena Caval ◽

Behcet's Disease ◽

Superior Vena Caval Obstruction

Download Full-text

Treatment of Behçet's Disease: An Algorithmic Multidisciplinary Approach

Frontiers in Medicine ◽

10.3389/fmed.2021.624795 ◽

2021 ◽

Vol 8 ◽

Author(s):

Erkan Alpsoy ◽

Pietro Leccese ◽

Giacomo Emmi ◽

Shigeaki Ohno

Keyword(s):

Behçet’S Disease ◽

Multidisciplinary Approach ◽

Behcet’S Disease ◽

Cardiac Involvement ◽

Age Of Onset ◽

Behçet's Disease ◽

Immunosuppressive Drugs ◽

Unknown Etiology ◽

Multisystem Disease ◽

Behcet's Disease

Behçet's disease (BD) is a chronic, relapsing inflammatory, multisystem disease of unknown etiology. The disease has a wide clinical spectrum of mucocutaneous lesions and ocular, vascular, articular, neurologic, gastrointestinal and cardiac involvement. Although the number of effective drugs used in the disease's treatment has increased in recent years, BD is still associated with severe morbidity because of mainly mucocutaneous, articular and ocular symptoms and an increased mortality because of large vessel, neurological, gastrointestinal and cardiac involvement. Many factors are associated with a more serious course, such as male gender and a younger age of onset. While the severity of the disease is more pronounced in the first years of the disease, it decreases in most patients after the age of forties. The primary goal of treatment should be the prevention of irreversible organ damage. Therefore, early diagnosis and appropriate treatment and close follow-up are mandatory to reduce the morbidity and mortality of the disease. Treatment varies depending on the organ involved and the severity of the involvement. For all these reasons, the treatment should be personalized and arranged with a multidisciplinary approach according to the organs involved. Treatment is mainly based on suppression of the inflammatory attacks of the disease using local and systemic immunomodulatory and immunosuppressive drugs. In this review, based on the mainly controlled studies and personal experience in clinical practice and basic research in this field, we propose a stepwise, symptom-based, algorithmic approach for the management of BD with a holistic perspective.

Download Full-text