Are clusters of patients with distinct clinical expression present in Behçet's disease?

Studies from Israel and Turkey have proposed that patient clusters with discriminating clinical features may exist in Behçet's disease (BD); such clusters could help to better understand pathogenetic mechanisms and guide therapeutic decisions. Herein, we searched for specific associations between each disease manifestation to all other manifestations in Greek patients with BD.

Specific clinical features were retrospectively recorded in 142 consecutive patients (80 men) fulfilling the International Study Group criteria, seen between 2000-2008 in our Departments (mean follow-up of 37 months). All possible associations between distinct clinical features were examined; further analysis in relation to HLA-B51 status and pathergy test positivity, available in 89 patients, was performed.

No significant associations between various manifestations of BD were found, either among all patients, or among men or women analysed separately. Uveitis was present more frequently in men, but not women, who were HLA-B51 carriers (p<0.02). A positive pathergy reaction was associated with oral ulcers (p<0.001) and central nervous involvement (p=0.008) in women, and folliculitis in men (p=0.046).

In contrast to studies from other countries, no subgroups of patients with distinct positive or negative associations between clinical features were found. HLA-B51 may have some prognostic significance in men only. Whether differences in disease expression between geographical areas may reflect different triggers of pathogenetic mechanisms operating among ethnic groups could be further explored in comparative studies.