A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry

Abstract

BackgroundBehçet’s disease is a rare multi-systemic inflammatory disease with unknown etiology which involves principally oral and genital mucosa, skin and eyes. Average age at onset of the disease is about 25-30 years, but it may be diagnosed before the age of 16. It is not very rare in Italy, even though there are limited data concerning epidemiology. Aim of this study is to describe the baseline data of an Italian cohort of patients with as having BD or probable BD.MethodsWe 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. Data on demographic characteristics, clinical features and therapy were collected. We then compared our findings to those of international pediatric cohort studies and also retrospectively evaluated the ability to diagnose BD using ISG, ICBD and, for the first time, the new PEDBD criteria.ResultsThe study included 110 patients (62 M, 48F). Average age at onset was 8.34±4.11 years. The frequencies of signs/symptoms were: 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%. Thirty-two patients (29.1%) fulfilled ISG, 78 (70.9%) ICBD, 50 (45.5%) PEDBD criteria and 31 (28%) didn’t fulfill any of them. The most frequently used treatments were colchicine and corticosteroids followed by immunosuppressants. Four patients received biologic therapy (anti TNF-α and anti-IL-1) to treat severe organ involvement.ConclusionsRecurrent oral aphtosis was the most frequent clinical manifestation, followed by ocular involvement. Gastrointestinal lesions were more frequent in Italy than in non-European countries as opposed to genital ulcers. Skin, ocular and vascular manifestations had a higher frequency in males and genital ulcers in females. Constitutional symptoms were present in 44.5% and recurrent fever in one third of our population.

Clinical manifestations and anti-TNF alpha therapy of juvenile Behçet’s disease in Taiwan

“…Other BD classification criteria have been developed for more robust diagnoses. The ICBD have been validated in JBD patients with sensitivities that range from 70 to 80%, but specificity data are absent [22, 23]. The PEDBD criteria were developed for children, and they have a sensitivity of 73.5% and a specificity of 97.7% [4, 21].…”

Adaptation to Mediterranea

“…This is a rare, sporadic, multi-systemic disease with undetermined cause. The main clinical features are constitutional symptoms and recurrent fever, oral aphthous, genital ulcers, with gastrointestinal, musculoskeletal, neurological, and vascular involvement [54].…”

Correction to: A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry

“…Following publication of the original article [ 1 ], the authors reported that the names of two institutional authors – EUROFEVER and the Paediatric Rheumatology International Trials Organisation (PRINTO) – had been unintentionally omitted in the final online version of the manuscript. The corrected author list is shown in this Correction.…”