Gallizzi R, et al. - This study details the baseline data of an Italian cohort of patients with as having Behçet’s disease (BD) or probable BD. The most frequent clinical manifestation was recurrent oral aphtosis, followed by ocular involvement. A more frequent occurrence of gastrointestinal lesions, as opposed to genital ulcers, was reported in Italy than in non-European countries. Skin, ocular and vascular manifestations had a higher frequency in males and genital ulcers in females. The presence of constitutional symptoms was reported in 44.5% and recurrent fever in one-third of the population.

Methods

• In this study, researchers described the baseline data of the first national epidemiological study on children coming from 16 Italian Pediatric Rheumatologic Centers diagnosed by the treating physicians as having Behçet’s Disease.
• They gathered data on demographic characteristics, clinical features and therapy.
• Thereafter, a comparison was performed between researchers' findings and those of international pediatric cohort studies.
• They also performed a retrospective assessment of the ability to diagnose BD using ISG, ICBD and, for the first time, the new PEDBD criteria.

Results

• A total of 110 patients (62 M, 48F) were included in this study, with average age at onset being 8.34±4.11 years.
• The following frequencies of signs/symptoms were documented: recurrent oral aphtosis 94.5%, genital ulcers 33.6%, ocular 43.6%, gastrointestinal 42.7%, musculoskeletal 42.7%, neurological 30.9% and vascular involvement 10%.
• As per the data, 32 patients (29.1%) fulfilled ISG, 78 (70.9%) ICBD, 50 (45.5%) PEDBD criteria and 31 (28%) didn’t fulfill any of them.
• Colchicine and corticosteroids, followed by immunosuppressants, were reported to be most frequently used treatments.
• For the treatment of severe organ involvement, biologic therapy (anti TNF-α and anti-IL-1) was given to 4 patients.