Behçet's Disease

Sakane, Tsuyoshi; Takeno, Mitsuhiro; Suzuki, Noboru; Inaba, Goro

doi:10.1056/nejm199910213411707

Cited by 1,614 publications

(1,354 citation statements)

References 33 publications

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“…However, in agreement with our data, 2 other studies reported no increase in mortality in patients with BD (42,43). Turning to morbidity, we looked specifically at inflammatory ocular disease, because this is both a frequent and a potentially serious complication of BD with a significant risk of visual loss (1,2). At the end of followup, all patients with and without eye disease were reevaluated by an ophthalmologist.…”

Section: Seventeen-year Study Of Behçet's Disease In Italysupporting

confidence: 83%

“…Behçet's disease (BD) is a vasculitis of unknown etiology characterized predominantly by recurrent oral and genital aphthae, uveitis, and a variety of skin lesions (1,2). BD has a distinct geographic distribution, its prevalence being much higher in countries along the ancient Silk Road, extending from Japan to the Mediterranean and Middle Eastern countries, than in northern Europe (3).…”

Section: Introductionmentioning

confidence: 99%

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Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: A seventeen‐year population‐based study

Salvarani¹,

Pipitone²,

Catanoso³

et al. 2007

Arthritis & Rheumatism

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Objective. To investigate the epidemiology and clinical course of Behçet's disease (BD) over a 17-year period in a defined area of northern Italy. Methods. All patients with incident BD diagnosed over a 17-year period (from January 1, 1988 to December 31, 2004) living in the Reggio Emilia area were identified through the following sources: physicians at Reggio Emilia Hospital, medical practitioners, and community-based specialists. We identified all patients registered in a centralized index and in the Reggio Emilia district database for rare diseases. Patients were followed up from the time of diagnosis until either their death or April 1, 2005. Results. Eighteen patients (9 men and 9 women) had complete BD. Mean ؎ SD age at diagnosis was 33 ؎ 7 years. The incidence rate of BD was 0.24 per 100,000. The prevalence of BD on January 1, 2005 was 3.8 per 100,000. No patients died during the followup period. Although all patients developed oral ulceration during the disease course, 22.2% had no oral lesions at disease onset. Eye disease occurred in 55.6%. Ocular disease was more common in men and appeared at disease onset or within the first few years of disease onset (median 3 years). Only 1 patient had loss of useful vision in at least 1 eye at the end of followup. In all affected patients, visual acuity improved once treatment was started. Conclusion. This population-based study is the first to report the prevalence and incidence of BD in Italy. In Italian patients, BD is nonfatal and the prognosis of eye disease is good.

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Section: Seventeen-year Study Of Behçet's Disease In Italysupporting

confidence: 83%

Section: Introductionmentioning

confidence: 99%

Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: A seventeen‐year population‐based study

Salvarani¹,

Pipitone²,

Catanoso³

et al. 2007

Arthritis & Rheumatism

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“…The prevalence of the HLA-B51 allele is high among patients with Behçet's disease who live in areas along the Silk Road, but not among white patients who live in Western countries (13%) (49,50). Turkey has the highest prevalence at 80 -370 cases per 100,000 populations.…”

Section: Discussionmentioning

confidence: 99%

Prevalence and incidence in patients with autoimmune rheumatic diseases: A nationwide population‐based study in Taiwan

See

Kuo

et al. 2013

Arthritis Care & Research

117

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“…1 It has been shown that the common histological lesion of all clinical manifestations appears to be vasculitis. 4,5 Many studies concerning the vascular involvement and the thrombotic Also, the finding of decreased plasminogen fibrinolytic activity in patients with active BD presents a possible disturbance in the blood coagulation mechanism in BD patients.…”

Section: Discussionmentioning

confidence: 99%

“…A large number of studies have shown a linkage between the disease and HLA-B51 and HLA-B27 antigens. [1][2][3][4] Vasculitis is the underlying pathological process, in contrast to the classic triad of recurrent urogenital ulcerations, with uveitis. 5,6 Vascular involvement in BD includes venous thrombosis, arterial occlusion, pulmonary artery, and aortic aneurysm formation.…”

mentioning

confidence: 99%

Cardiovascular Involvement in Behçet Disease: Clinical Implications

et al. 2015

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Beh?et disease (BD) is a multisystem disorder, with vasculitis as its underlying pathological process, in contrast to the classic triad of recurrent oral and genital ulcerations, with uveitis. Vascular involvement in BD includes venous thrombosis, arterial occlusion, and pulmonary artery and aortic aneurysm formation. Cardiac involvement is rare and often obscure. It includes intracardiac thrombi formation, and is associated with a poor prognosis. Our objectives are to describe two cases with BD, complicated with vascular and cardiac involvement, and to raise awareness of these rare complications, the needed routine surveillance, and thus to prevent inappropriate interventions, serious outcomes, and mortality. We present two male patients from the Mediterranean Basin with BD. The first was diagnosed early as a BD patient. The second was diagnosed at the time of cardiovascular (CV) involvement. We recommend that patients who are diagnosed, or even suspected of suffering from BD, especially in endemic areas along the Silk Route pathway, should be followed up routinely for CV involvement, even if rare, obscure, or with a bizarre presentation.

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Behçet's Disease

Cited by 1,614 publications

References 33 publications

Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: A seventeen‐year population‐based study

Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: A seventeen‐year population‐based study

Prevalence and incidence in patients with autoimmune rheumatic diseases: A nationwide population‐based study in Taiwan

Cardiovascular Involvement in Behçet Disease: Clinical Implications

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