Familial aggregation in Behçet’s disease: High frequency in siblings and parents of pediatric probands

Koné‐Paut, Isabelle; Geisler, Iris; Wechsler, B.; Özen, Seza; Özdoğan, Huri; Rozenbaum, Michael; Touitou, Isabelle

doi:10.1016/s0022-3476(99)70333-1

Cited by 128 publications

(57 citation statements)

References 11 publications

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“…Patients with BD who had affected relatives fulfill the disease criteria almost 10 years earlier on average than those of sporadic cases. Koné-Paut et al showed that the frequency of familial cases was significantly higher (p< 0.0001) in pediatric BD patients (12.3%) than in nonpediatric patients (2.2%) (8). In our group, all patients from the same family fulfilled the disease criteria when their age was over twenty years and thus they were described as adult patients.…”

Section: Discussionsupporting

confidence: 43%

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

et al. 2012

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A familial aggregation of Behçet's disease (BD) has long been noted. These studies have supported the direct role of HLA-B5 in the pathogenesis of BD. Despite the fact that familial clustering is characterized by genetic anticipation, accounting for the earlier disease onset in successive generations, we present two brothers and two cousins from the same family who were diagnosed when they were over twenty years old.We report these young adult patients to introduce the characteristics of familial aggregation of BD. In this article HLA-B*51 and Cw*16 positivities with adult onset were demonstrated.

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Section: Discussionsupporting

confidence: 43%

“…Also the mean age at the diagnosis of familial BD was almost 10 years earlier than in sporadic cases (8).…”

Section: Introductionmentioning

confidence: 84%

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

et al. 2012

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“…The prevalence of familial ABD is in the range of 1-18%. 8,9,[13][14][15][16][17][18][19] In a study from Turkey, the risk of disease in sisters was 11.4-52.5. 19 Among different genetic markers, the class I HLA-B5 and -B51 gene on chromosome 6p21 is in association with ABD.…”

Section: Discussionmentioning

confidence: 99%

Ocular involvement is associated with HLA-B51 in Adamantiades–Behçet's disease

Krause

Altenburg

et al. 2008

Eye

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Purpose To evaluate the association of HLA-B51 and ocular involvement in Adamantiades-Behçet's disease. Methods We retrospectively analysed all patients with Adamantiades-Behçet's disease examined in our Department of Ophthalmology since 1982. All patients fulfilled the criteria of the International Study Group for Behçet's disease. We included 140 patients (63 female and 77 male) with a mean follow-up of 6.4 years. Results The mean age at the first manifestation was 23 years; full disease was noted at 32 years. The mean age at the time of eye involvement was 30 years. Most of the patients were of Turkish (n ¼ 73) or German (n ¼ 34) origin. A total of 56% patients developed eye involvement. Forty-nine out of 76 HLA-B51-positive patients (64.5%) and 26 out of 60 HLA-B51-negative patients (43.3%; P ¼ 0.014) developed ocular involvement. Conclusion More than the half of the patients with Adamantiades-Behçet's disease evaluated in our department developed ocular involvement. There was a statistically significantly higher frequency of HLA-B51 in these patients.

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“…Although many cases of BS are sporadic, familial aggregation is observed with varing frequencies in different populations (more frequenct in juvenile patients) (42,43). The strongest genetic factor associated with the risk of Behçet syndrome is the HLA-B5/B51 allele carrier state, which is observed more frequently in familial cases compared with sporadic BS (44,45).…”

Section: Known Susceptibility Genes In Commonly Observed Diseases Witmentioning

confidence: 99%

Role of genetics in pediatric rheumatology

et al. 2017

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Pediatric rheumatology includes autoinflammatory monogenic diseases, autoinflammatory multifactorial diseases with complex inheritance, and diseases with uncertain clinical diagnosis or undefined conditions, even though they show signs of autoinflammation. Most of these diseases are systemic; it is important to diagnose patients promptly and definitively and to select proper treatment options based on the diagnoses. Clinical observation and acute-phase responses are usually sufficient for diagnosis; however, genetic analyses can provide supportive data for definite diagnosis and treatment, especially for rare monogenic diseases. As for multifactorial autoinflammatory diseases, susceptibility genes, and factors involved in the etiopathogenesis have not been fully identified. It is possible to identify disease genes and novel diseases, and lead to new treatment options by gene mapping studies and high-throughput screening strategies for multifactorial diseases and conditions with uncertain clinical characteristics. In this review, we discuss the three groups of autoinflammatory diseases and role of genetics in their diagnosis.

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Familial aggregation in Behçet’s disease: High frequency in siblings and parents of pediatric probands

Cited by 128 publications

References 11 publications

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

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

Ocular involvement is associated with HLA-B51 in Adamantiades–Behçet's disease

Role of genetics in pediatric rheumatology

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