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Familial aggregation in Behçet's Disease: Sibling history as a risk factor for Neuro-Behçet
Emine Büşra Ata1, İbrahim Yahya Çakır2, Asena Eken3
1Department of Internal Medicine, Division of Rheumatology, University of Health Sciences, Gulhane Training and Research Hospital, General Tevfik Sağlam Street, Ankara, 06100, Türkiye. eminebusraata@gmail.com.
Insights
Having an affected sibling triples the risk of neurological involvement in Behçet's disease (BD). Early recognition of neuro-Behçet is crucial for patients with a family history of BD.
Area of Science:
- Rheumatology
- Neurology
- Genetics
Background:
- Behçet's disease (BD) is a multisystem inflammatory disorder with genetic and environmental influences.
- Understanding familial aggregation's impact is key to pathogenesis.
Purpose of the Study:
- Investigate how a sibling history affects organ involvement and clinical features in Behçet's disease.
- Identify predictors of major organ involvement in BD patients with a family history.
Main Methods:
- Analyzed data from 891 Behçet's disease patients, stratified by family history (n=651) and affected sibling (n=70).
- Retrieved demographic, clinical, and laboratory data.
- Used logistic regression to identify predictors of major organ involvement, adjusting for covariates.
Main Results:
- Patients with an affected sibling showed significantly higher rates of neurological involvement (20% vs. 7.5%).
- A sibling history was associated with a threefold increased risk of neurological involvement (OR: 3.01).
- Demographics and smoking status were similar between groups.
Conclusions:
- An affected sibling increases the risk of neurological involvement in Behçet's disease by threefold.
- Early recognition of neuro-Behçet is critical in individuals with a family history.
- Clinicians should be vigilant for neurological manifestations in BD patients with affected siblings.
Background:
Behçet's disease (BD) is a multisystem inflammatory disorder in which both genetic predisposition and environmental factors contribute to its pathogenesis.
Aims:
This study aimed to investigate the impact of sibling history on organ involvement and clinical features in BD.
Methods:
A total of 891 patients diagnosed with Behçet's disease were enrolled in the study. Patients were stratified according to the presence and degree of familial aggregation: 651 had no family history of Behçet's disease (sporadic BD), while 240 reported a positive family history, of whom 70 had an affected sibling (BDw/SH). Demographic characteristics, clinical manifestations, and laboratory parameters were systematically retrieved from patients' medical records. Logistic regression analyses were performed to identify independent predictors of major organ involvement. Multivariable models were adjusted for relevant demographic and clinical covariates.
Results:
Age, sex, body mass index (BMI), and smoking status were similar in patients with a sibling history of Behçet's disease (n = 70) and those without family history (n = 651) (p > 0.05). Among the clinical manifestations, the frequency of neurological involvement was significantly higher in patients with a sibling history of Behçet's disease compared to those without family history ( 20% vs. 7.5%, p < 0.001), with a threefold increased risk in multivariate analysis (OR: 3.01, CI95%:1.54-5.88).
Conclusion:
These findings demonstrate that the presence of an affected sibling increases the risk of neurological involvement by 3-fold. Early recognition of neuro-Behçet is critical; hence, clinicians should remain alert to its possible neurological manifestations in individuals with a sibling history.
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