Autoinflammatory & rare systemic diseases

Distinct features of trisomy 8-associated autoinflammatory disease from Behçet's disease: case series and systematic review

Clin Exp RheumatolPublished 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

OutcomeOR (95% CI)
Mortality11.74 (5.94–22.82)

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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