Background <p>Behçet’s disease (BD) is a multisystem inflammatory disorder in which both genetic predisposition and environmental factors contribute to its pathogenesis.</p> Aims <p>This study aimed to investigate the impact of sibling history on organ involvement and clinical features in BD.</p> Methods <p>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.</p> Results <p>Age, sex, body mass index (BMI), and smoking status were similar in patients with a sibling history of Behçet’s disease (<i>n</i> = 70) and those without family history (<i>n</i> = 651) (<i>p</i> &gt; 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%, <i>p</i> &lt; 0.001), with a threefold increased risk in multivariate analysis (OR: 3.01, CI95%:1.54–5.88).</p> Conclusion <p>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.</p>

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Familial aggregation in Behçet’s Disease: Sibling history as a risk factor for Neuro-Behçet

  • Emine Büşra Ata,
  • İbrahim Yahya Çakır,
  • Asena Eken,
  • Çetin Ergül,
  • Ebru Karaçalı,
  • Yunus Emre Dalkılıç,
  • Fatih Mehmet Doğan,
  • Emine Uslu,
  • Nuh Ataş,
  • Derya Yıldırım,
  • Duygu Tecer,
  • Güllü Sandal Uzun,
  • Aşkın Ateş,
  • Murat Turgay,
  • Mehmet Akif Öztürk,
  • Sedat Yılmaz,
  • Abdurrahman Tufan,
  • Hazan Karadeniz

摘要

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.