Paediatric neuro-Behçet's disease: multi-centre observational study

Behçet's disease (BD) is a multisystemic inflammatory disease, and its neurologic involvement represents serious manifestations. This study aimed to elucidate the neurologic manifestations of BD in a large paediatric population and to describe its outcomes and prognosis.

This retrospective multicentre study included 75 paediatric patients diagnosed with neuro-Behçet's disease (NBD). Demographic data, clinical features, neuroimaging findings, treatments and disability conditions were reviewed.

Neurologic manifestations were observed in 52 patients (69.3%) at initial presentation and in 23 patients (30.7%) at a median of 14 months (8-30) after diagnosis. The distribution of NBD subgroups was as follows: 56 patients (74.7%) with nonparenchymal, 15 patients (20%) with parenchymal and 4 patients (5.3%) with mixed forms. Headache, observed in 70 patients (93.3%), was the most common neurological symptom, being significantly more frequent in the non-parenchymal form (P = 0.028). Paresis, sensory loss and mood/mental health problems were significantly more frequent in the parenchymal form (P = 0.034, P = 0.016, P = 0.005, respectively). Neuroimaging revealed parenchymal lesions in 19 patients (25.3%), and vascular lesions were in 55 patients (73.3%). Azathioprine was the most commonly used immunosuppressor therapy (78.7%). The median follow-up duration of patients was 30 months (17.5-52.5), during which significant clinical improvement was observed, with no deaths reported.

Paediatric NBD remains a significant issue in paediatric rheumatology. The non-parenchymal subgroup is the predominant form in children. Paediatric NBD should be confirmed and classified by MRI. The early aggressive immunosuppressive therapy provides clinical improvement. Activity and disability indexes are valuable for treatment and follow-up.