Behçet disease in children: A nationwide retrospective survey in Japan

Abstract

The purpose of the present study was to evaluate the incidence of BehFet 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 Behget disease among children under 16 years of age were reported. From a second survey, 31 cases satisfied the criteria of the BehGet'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-BehGet type, two were a combination of the entero-vasculo-Behqet type, and four were the neuro-BehGet 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 Behqet disease than in adults, while ocular complications were less frequent. The diagnosis of Behqet 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 Behget disease.

Uveitis in juvenile Behçet's disease: clinical course and visual outcome compared with adult patients

“…These recurrent attacks occurred in 9/13 (70%) paediatric group and in 8/16, (50%) adult group (NS). In the paediatric group, the mean number of acute exacerbations before remission was 4.1±2.7 (range, [1][2][3][4][5][6][7][8], and the mean interval between exacerbations (corrected for variable duration of followup) was 1.4 ± 0.8 years (range, 4 months to 2.5 years). The mean number of attacks in the adult group was 2.3 ± 1.5.…”

“…The cutoff age of 16 years was based on earlier studies of paediatric uveitis. [1][2][3]8 The research described adhered to the tenets of the Declaration of Helsinki and was approved by the Institutional Review Board.…”

“…The disease is uncommon in childhood, though it may occasionally present in incomplete form before the age of 16 years. [1][2][3] Because the disease is characterized by exacerbations and remissions, the complete form may be expressed only in adulthood. 4 Uveitis is one of the major criteria for diagnosis of BD, occurring in 70 to 95% of patients.…”

ABSTRACTS – Symposium

“…Ramnath MISRA (1) , Rajeev SINGH (2) , Sudhir SINHA (3) , A K. SASANY (4) , Amit SINGH This abstract will focus on identifying candidate bacterial antigents to which host T cell respond to and the cytokine profile of patients with reactive arthritis (ReA)/undifferentiated spondyloarthropathy (uSpA). We have previously shown that synovial fluid mononuclear cells (SFMC) from ReA and uSpA patients show proliferation to bacterial antigens that trigger ReA, i.e.…”

“…In the RADIATE trial patients who had experienced prior TNFinhibitor therapy received tocilizumab at 8 mg/kg plus MTX and had 50%, 29% and 12% ACR 20, 50 and 70 responses (3).…”