Related Experiment Videos
Behçet disease in children: a nationwide retrospective survey in Japan
1Department of Pediatrics, Koshigaya Hospital of Dokkyo University School of Medicine, Saitama, Japan.
Insights
This study investigated Behçet disease in Japanese children, finding gastrointestinal symptoms more common than in adults, while ocular issues were less frequent. Early diagnosis remains challenging due to delayed symptom manifestation.
Area of Science:
- Pediatrics
- Rheumatology
- Immunology
Background:
- Behçet disease is a rare multisystem inflammatory disorder.
- Understanding childhood Behçet disease is crucial for early diagnosis and management.
- Limited data exists on the epidemiology and clinical characteristics of Behçet disease in Japanese children.
Purpose of the Study:
- To determine the incidence of Behçet disease in Japanese children.
- To compare the clinical features of childhood Behçet disease with adult cases.
- To highlight diagnostic challenges in pediatric Behçet disease.
Main Methods:
- A nationwide survey of 1290 Japanese hospitals was conducted.
- Questionnaires were distributed to pediatric departments to identify cases.
- Data from 31 confirmed childhood Behçet disease cases were analyzed.
Main Results:
- Fifty-one potential cases were reported, with 31 meeting diagnostic criteria.
- Gastrointestinal symptoms were more prevalent in children than adults.
- Ocular complications were less frequent in childhood Behçet disease.
- Oral ulcers (100%) and skin lesions (55%) were common during the disease course.
Conclusions:
- Childhood Behçet disease presents distinct clinical features compared to adults, notably more gastrointestinal involvement.
- The diagnostic delay in pediatric Behçet disease underscores the need for specific diagnostic criteria.
- Further research on childhood Behçet disease is warranted to improve diagnostic accuracy and patient outcomes.
Abstract:
The purpose of the present study was to evaluate the incidence of Behçet disease among Japanese children and to compare their clinical features with adult patients. A first questionnaire was sent to the department of pediatrics of 1290 hospitals in Japan. Fifty-one cases of Behçet disease among children under 16 years of age were reported. From a second survey, 31 cases satisfied the criteria of the Behçet's Syndrome Research Committee of Japan. Of 31 patients (14 boys and 17 girls), three cases were the complete type, 24 cases were the incomplete type, and four cases were the possible type. As to the specific type of the disease, 10 cases were of the entero-Behçet type, two were a combination of the entero-vasculo-Behçet type, and four were the neuro-Behçet type. In the first 6 months after onset, oral ulcers were observed in 77% of cases, genital ulcers in 45%, uveitis in 10%, and skin lesions were observed in 39% of cases. During the course of the disease, the frequency of oral ulcers was 100%, genital ulcers, 58%; ocular complications, 29%; and skin lesions, 55%. Gastrointestinal signs and symptoms were more frequent in childhood Behçet disease than in adults, while ocular complications were less frequent. The diagnosis of Behçet disease in children is difficult because of the long interval before the onset of enough manifestations to satisfy the diagnostic criteria. Specific diagnostic criteria are necessary, based on a discussion of the many childhood cases of Behçet disease.