POS0081\u2005CLINICAL FEATURES OF PEDIATRIC BEHÇET’S DISEASE IN CHINA

Abstract

Behçet’s disease (BD) is a systemic inflammatory disease involving primarily the oral and genital mucosa, skin and eyes. Some patients exhibit broader involvement with multi-organ system inflammation, causing significant morbidity and mortality. With much of the literature focused on adult-onset BD, the clinical spectrum of BD in children is not well studied. The diagnosis of pediatric BD is difficult to make due to its rarity in childhood, the paucity of experience among pediatricians and the lack of validated diagnostic criteria.We aim to describe the clinical characteristic of children with Behçet’s disease in China.We performed a cross-sectional cohort analysis of patients with BD recruited between June 2017 and May 2020. We enrolled a total of 848 patients with pediatric BD (onset prior to age 18 years) and 1531 patients with adult-onset BD. Clinical data were collected by retrospective chart review.From a cohort of 848 patients with pediatric BD (412 males, 436 females), the average age of disease onset was 11.59 ± 4.11 years and the earliest case was appeared the first symptom at 7 months of age. Clinical manifestations in this group included recurrent oral aphtosis (100%), genital ulcers (77.71%), ocular involvement (30.19%), skin involvement (63.56%), neurological involvement (4.60%), gastrointestinal involvement (20.05%), vascular involvement (9.79%), heart valve involvement (3.66%), arthritis / arthralgia (32.43%), fever (0.35%) and positive pathergy test (21.11%; Table 1). The prevalence of these clinical findings was largely similar to that of adult BD patients with the exception of mild differences in the frequency of gastrointestinal, heart valve, joint involvement and fever. However, the time from symptom onset to diagnosis was significantly longer in children with BD compared to adults (13.34±9.13 years vs. 6.73±33.21 years, P<0.001). The most common initial symptom in pediatric BD was recurrent oral aphtosis (91.57% vs. 86.28% in adults, P<0.001).In the pediatric group, clinical manifestations also differed between males and females. In addition to oral ulcers, the most common clinical manifestations in male patients were ocular involvement (42.23%), vascular involvement (15.53%), heart valve involvement (5.10%) and epididymitis (9.95%). Among female patients, the frequency of genital ulcers is significantly higher than that of men (86.24% vs. 68.69%, P<0.001).We described the clinical characteristics of a large cohort of pediatric BD patients in China. While the clinical manifestations are largely similar to adult BD, the time from symptom onset to diagnosis is significantly delayed in pediatric patients. Our study highlights the need to improve awareness of BD among pediatric providers to facilitate rapid diagnosis and treatment in children.[1]Yildiz et al. Pediatric Behçet’s disease- clinical aspects and current concepts. Eur J Rheumatol 2020; 7(Suppl 1): S38-S47.[2]Koné-Paut. Behçet’s disease in children, an overview. Pediatric Rheumatology (2016) 14:10.Table 1.Comparison of clinical features of BD in children and adultsChildren(n=848)Adult(n=1531)PAge of first symptom (y)11.59±4.1127.08±7.91Sex(M/F)412/436Years of diagnosis (y)13.34±9.136.73±33.21<0.001Recurrent oral aphthosis848 (100%)1518 (99.15%)0.007Genital Ulcers659 (77.71%)1891152 (75.24%)3790.176Ocular signs256 (30.19%)592508 (33.18%)10230.134Cutaneous signs539 (63.56%)3091020 (66.62%)5110.132Neurological signs39 (4.60%)80982 (5.36%)14490.421Gastrointestinal signs170 (20.05%)678244 (15.94%)13870.001Vascular signs83 (9.79%)765162 (10.58%)13690.542Heart valve signs31 (3.66%)817110 (7.18%)1421<0.001Joint signs275 (32.43%)573612 (39.97%)919<0.001Fever3 (0.35%)84522 (1.44%)15090.013Positive pathergy test179 (21.11%)669347 (22.66%)11840.381China Behcet’ Disease Patients Assossiation.None declared.