Clinical features of Japanese children and adolescents with systemic lupus erythematosus: Results of 1980–1994 survey

Abstract

Marked advances have been made in the past decade in the management of adults with systemic lupus erythematosus (SLE). Therefore, a nationwide retrospective survey was conducted between 1980 and 1994 to investigate the clinical manifestations of SLE in Japanese children and adolescents. Questionnaires were sent to 340 hospitals. Of 405 patients reported by 176 hospitals, 373 patients, diagnosed by the criteria established by the Pediatric Study Group of the Japanese Ministry of Health and Welfare in 1985, were enrolled in the study. Forty‐nine of the 354 patients (13.8%) had relatives with a connective tissue disease within the third degree of consanguinity. The frequent manifestations in 373 patients were the presence of antinuclear antibody (98.9%), immunologic disorders (93.0%), hypocomplementemia (87.1%), malar rash (79.6%) and fever (74.0%). Lupus nephritis was present in 148 of the 309 patients (47.9%) at their first visit to a clinic, and 261 of the 373 patients (70.0%) developed renal involvement during the observation period. Of 370 patients, 92 patients (24.9%) exhibited central nervous system lupus. Of 368 patients, 192 patients (52.2%) were treated by methylprednisolone pulse therapy and 148 patients (40.2%) received immunosuppressants in combination with steroid therapy at some stage during the observation period. Survival rate at 5 years from onset was 95.9%. Management of infection, coagulopathies, and central nervous system involvement is essential to improve the prognosis of SLE in Japanese children and adolescents.

Epidemiology treatment and outcome of childhood systemic lupus erythematosus in Egypt

“…Alsalloum et al [40] reported that mean AI was 8.2 (range 0. [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] in Saudi LN children with class III and IV disease. In the study of Bogdanovic et al [28], AI was 9 (range 2-14) in patients with class IV LN.…”

“…Many investigators have described the features of childhood SLE among different ethnic and geographical groups [6,7,8,9,10,11,12]. Studies of SLE among Arab children are few [13,14,15,16], and there is only one study of Egyptian children [17].…”

Long term prognosis of children born to lupus patients

“…Three of them (one boy (9), two girls (8, 10)) had become negative again by the time of the third investigation. One girl became positive at 14 years old (12). One boy was positive at 5 years of age and had become negative by 11 years of age (11).…”

“…This ratio is similar to those demonstrated in familial cases of SLE and SLE in childhood. 12 This discrepancy between the sexes might be considered as due to hormonal, environmental, behavioural, or genetic effects. Hormonal effects must exclude oestrogen because serum oestrogen levels are the same for boys and girls.…”

Long-term remission in three patients with childhood-onset systemic lupus erythematosus

“…11,12 Additionally, in Japan, we have applied those therapies even in cases complicated only with hypocomplementemia, because this condition reflects the disease activity in childhood SLE. 16 In the past, the goals of long-term management in patients with SLE were to suppress the disease activity with a minimum of side effects, and to improve the quality of life in patients with SLE. However, ultimately, it is of course our wish that such patients can, if possible, live without medication.…”