A review of patient data from the International AutoInflammatory Disease Alliance (AIDA) Network registry identifying those with mucocutaneous Behçet’s disease (BD) may progres to major organ involvement (MOI)m especially at later stages.

From the AIDA network, 328 BD patients with an onset of mucocutaneous manifestations were enrolled. 

A total of 82 patients (25%) developed MOI over the entire follow-up period. 

A significantly reduced risk of MOI was seen in those with minor oral aphthosis and no major oral aphthosis (OR 0.41; 0.22-0.79, p = 0.008). 

Conversely, a significantly higher risk of future MOI was seen in those with both major and minor oral aphthosis (OR 12.76; 1.44-113, p = 0.02). MOI was also increased with:

• Major oral aphthosis plus genital aphthosis (OR: 2.49, 1.1-5.6, p = 0.03)
• Major oral aphthosis plus pseudofolliculitis (OR: 2.9, 95%CI: 1.15-7.4, p = 0.02)
• Major oral aphthosis plus both genital aphthosis and pseudofolliculitis (OR: 3.73, 1.22-11.4, p = 0.02)
• Positive family history for BD was associated with MOI (OR: 2.85, 1.08-7.58, p = 0.03).

Major organinvolvement occurs in a minority of BD patients, especially those with a positive family history and the presence of major oral aphthosis combined with minor oral aphthosis, genital aphthosis or pseudofolliculitis.