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.

To estimate the number of children with rheumatic diseases, a questionnaire was distributed to the pediatrics department of 1290 hospitals in Japan in June 1994. From this survey, 1606 cases with juvenile rheumatoid arthritis (JRA), 906 cases with systemic lupus erythematosus (SLE), 320 cases with dermatomyositis/polymyositis (DMPM), 28 cases with scleroderma (PSS), 70 cases with Sjogren's syndrome (Sjs), 93 cases with mixed connective tissue disease (MCTD), 25 cases with aortitis syndrome, 20 cases with polyarteritis (PN) and 51 cases with Behqet disease were reported. The crude annual incidence rates per 100000 among the childhood population were estimated as JRA, 0.83; SLE, 0.47; DMPM, 0.16; PSS, 0.01; Sjs, 0.04; MCTD, 0.05; aortitis syndrome, 0.01; PN, 0.01; and Behset disease, 0.03. The present study reveals that there are more children with rheumatic diseases than are estimajed from the reported cases in the literature and the number of children who are receiving Assistance Medical Costs Insurance covered by the Japanese government.

The purpose of the present study was to investigate the incidence of juvenile rheumatoid arthritis (JRA) among Japanese children and to evaluate the clinical features of this disease. A questionnaire was sent to the department of pediatrics of 1290 hospitals in Japan, in 1994, asking for the number of rheumatic patients during the past 10 years. Subsequently, a second questionnaire was sent asking for the type of onset, clinical features, treatment, and other details. The results of 570 cases were obtained. Of these, 310 cases (54%) were the systemic onset type, 140 cases were the polyarticular onset type (25%), and 120 cases (21%) were the pauciarticular onset type. Hence, in the present series of children, the proportion of the pauciarticular type was less than the other two types of JRA. In the laboratory findings of the systemic onset type, hyperfenitinemia and thrombocytosis were noted, in addition to leukocytosis, positive Creactive protein (CRP) and accentuated erythrocyte sedimentation rate (ESR). The rheumatoid factor was positive in 50% of patients with the polyarticular onset type. Chronic uveitis was recognized in 13 cases (10.80/0) of the pauciarticular onset type. In four girls, uveitis started before the onset of arthritis. Nonsteroidal anti-inflammatory drugs were used in almost one-third of cases, and methotrexate (MTX) was used in 12.8% of cases. The quality of life of children with JRA was disturbed in almost 20% of cases. Therefore, for the early and definitive diagnosis of the systemic type of JRA, diagnostic procedures including thrombocyte counts and serum ferritin level, should be performed. In order to obtain good results and to avoid side effects, a protocol for the use of disease modifying anti-rheumatic drugs and immunosuppressants, especially for the use of MTX, must be established.