Familial Behçet's Disease of Adult Age: A Report of 4 Cases from a Behçet Family

Gülbay, Banu Eriş; Acican, Turan; Diken, Özlem Erçen; Önen, Zeynep Pınar

doi:10.2169/internalmedicine.51.6858

Cited by 5 publications

(4 citation statements)

References 9 publications

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“…Immune fluorescence studies revealed IgM, IgG and β1 globulin on the vascular endothelial walls and the serum contained increased amounts of IgD, IgG, IgM, C1, C2, C3, C4 and immune complexes. The increased prevalence of the HLA-B5 tissue gene group suggests a genetic role as aetiological factor 13-15…”

Section: Discussionmentioning

confidence: 99%

Behcet's disease and cardiovascular involvement : our experience of asymptomatic Behcet's patients : cardiovascular topic

Uluşan¹,

Karadağ²,

Taşar³

et al. 2014

CVJA

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SummaryAbstractBehcet’s syndrome is a systemic inflammatory disease associated with vasculitis, and arterial, venous and cardiac disorders. Thirty-eight Behcet’s disease patients were examined prospectively with echocardiography, ultrasonography and computed tomography, and coagulation parameters were determined. Deep venous insufficiency was found in 16 patients, venous thrombosis in seven, one patient had iliac artery stenosis, three had carotid arterial intimal proliferation, two patients had aortic annulus dilatation, six had aortic valve insufficiency, and three had mitral valve insufficiency. None had coagulation defects. To decrease morbidity and mortality rates, a multidisciplinary approach is important for early diagnosis of cardiovascular involvement in Behcet’s disease.

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Section: Discussionmentioning

confidence: 99%

Behcet's disease and cardiovascular involvement : our experience of asymptomatic Behcet's patients : cardiovascular topic

Uluşan¹,

Karadağ²,

Taşar³

et al. 2014

CVJA

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“…Erythema-Nodosum-like Lesions Neutrophilic infiltrates, panniculitis, and fat necrosis [8]. Studies described such infiltrates as a differential factor from Erythema Nodosum.…”

Section: Genital Ulcerationmentioning

confidence: 99%

“…Although most cases are sporadic, which makes diagnosing and predicting them a lot more complex, familial clusters are reported [7]. Furthermore, in these cases, genetic anticipation has been observed [8]. The global prevalence 2017 reached 14.6 per 100,000, with even higher prevalence rates along the Silk Route, like Turkey, with 420 per 100,000 [9,2].…”

Section: Introductionmentioning

confidence: 99%

Advances and Challenges in Diagnosing Behçet’s Disease: A Comprehensive Review

Gomez Coral

2024

ARR

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Behçet's Disease (BD) presents significant diagnostic and management challenges due to its complex and multifaceted nature. Initially described in 1937, BD primarily affects populations along the Silk Road, with a prevalence of 14.6 per 100,000 globally and 420 per 100,000 in Turkey. Its autoimmune nature and lack of definitive etiology make diagnosis reliant on clinical manifestations, with recurrent oral ulcers being a hallmark symptom. Recent research has shed light on the genetic underpinnings of BD, with HLA-B*51 being a significant susceptibility locus. Immunological dysregulation involving T and B lymphocytes, dendritic cells, and various cytokines contribute to the pathogenesis. Clinical manifestations vary widely, affecting multiple organ systems, with mucocutaneous and ocular symptoms being the most common. Diagnosis is primarily clinical, aided by classification criteria such as the ISG and ICBD criteria, although challenges persist due to overlapping features with other autoimmune disorders. Multidisciplinary collaboration among specialists is crucial for accurate diagnosis and optimal management.

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“…Although BD has traditionally been considered sporadic there have been cases of familial clustering, suggesting a potential genetic predisposition to disease [14, 15]. Genetic anticipation, where the disease presents earlier in subsequent generations, is also evident [16]. …”

Section: Introductionmentioning

confidence: 99%

An update on the use of biologic therapies in the management of uveitis in Behçet’s disease: a comprehensive review

McNally

Damato

Murray

et al. 2017

Orphanet J Rare Dis

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ᅟBehçet’s disease (BD) is a systemic vasculitis characterised by a relapsing remitting course, affecting multiple organ systems. In the eye, it is a cause of potentially blinding inflammation in the form of uveitis. Management of uveitis in BD often requires the use of systemic immunosuppression, in order to reduce disease activity and prevent accumulation of irreversible damage. Whilst corticosteroids remain the mainstay of treatment, long-term use is limited by the development of adrenocorticotrophic side effects. There has therefore been significant interest in the use of corticosteroid-sparing immunosuppressive agents, and more recently, biologic therapies. Recent publications have demonstrated biologic therapy to have beneficial effects both on overall disease control, and quality of life for patients with BD. Widespread use of such agents is however limited, partly by the lack of high quality research evidence, and partly by the prohibitive cost of biologic treatments. In this review, we discuss the most recent research investigating the use of biologic therapy in uveitis due to BD, with consideration of health economics and quality of life outcomes.

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Familial Behçet's Disease of Adult Age: A Report of 4 Cases from a Behçet Family

Cited by 5 publications

References 9 publications

Behcet's disease and cardiovascular involvement : our experience of asymptomatic Behcet's patients : cardiovascular topic

Behcet's disease and cardiovascular involvement : our experience of asymptomatic Behcet's patients : cardiovascular topic

Advances and Challenges in Diagnosing Behçet’s Disease: A Comprehensive Review

An update on the use of biologic therapies in the management of uveitis in Behçet’s disease: a comprehensive review

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