Autoinflammatory & rare systemic diseases
Distinct features of trisomy 8-associated autoinflammatory disease from Behçet's disease: case series and systematic review
Authors' conclusion
Trisomy 8-associated autoinflammatory disease patients were older and had poorer prognoses, more gastrointestinal involvement, and less frequent oral and genital ulcers and skin involvement, making BD diagnosis less likely.
Study context
- Population
- Patients with trisomy 8 and autoinflammatory features, compared with Behçet's disease (BD) patients from the Yokohama City University Registry88 articles, 181 published + 6 own patients
- Participants
- 187 vs 657
- Comparison
- Trisomy 8-associated autoinflammatory disease vs BD
- TRISOMY 8n = 187
- BDn = 657reference
- Outcome
- Clinical features of trisomy 8 patients
Methodological detailscomparison with BD registry cohort, Four online databases searched; 1,542 articles screened
- Comparison with BD registry cohort
- Four online databases searched; 1,542 articles screened
Pooled result
As reported in the abstract
- Fever (most common symptom in trisomy 8)
- 85.8%
- Oral ulcers in trisomy 8
- 80.8%
- Gastrointestinal lesions in trisomy 8
- 76.1%
- Genital ulcers in trisomy 8
- 54.7%
- Skin lesions in trisomy 8
- 53.7%
Further pooled results
| Outcome | OR (95% CI) | |
|---|---|---|
| Mortality | 11.74 (5.94–22.82) | |
| 0.050.21520Odds ratio (log scale) |
Original publication
Distinct features of trisomy 8-associated autoinflammatory disease from Behçet's disease: case series and systematic review. Clin Exp Rheumatol. Published 9 August 2024. doi:10. 55563/ clinexprheumatol/ 8j7rbr
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