ENGLISH SUMMARYVinje O, Flatø B. The epidemiology of juvenile systemic lupus erythematosus.Nor J Epidemiol 2008; 18 (1): 83-85.There are major uncertainties concerning the frequency of juvenile systemic lupus erythematosus in the different populations in the world. The incidence is reported to be between 0.36 and 0.9/1,00,000 per year. However, there exist great differences between different ethnic populations. The frequency is particularly high in African blacks and some Asian populations, and least frequent in white Caucasians. The disease is very infrequent before the age of 5. The great majority gets the disease after the age of 8, median age about 12 years. There is a female overrepresentation, most prominent in the older age group. The risk of early death has decreased significantly during the latest 40-50 years. Published data from the 1950s and 1960s yields 5-year survival rates of only 17.5 to 60%. Five-and 10 years survival rates are now approaching 100% and 90%. How it will turn out after 20, 30, 40 and 50 years of disease is unknown. Severe kidney affection, particularly grade 4 or 5 glomerulonephritis is historically most closely associated with early death. But also CNS disease, high general disease activity, severe infection and low social status increase the risk. The risk of severe infection, sepsis, seems to parallel the increase of effective treatment. Irreversible organ damage in different organs also seems to have increased in the latest decade, and might be associated with increasing survival, often as a result of more aggressive medical treatment.JSLE er en kronisk, fluktuerende, autoimmun, flerorgansykdom av ukjent årsak. Den kjennetegnes ved at det opptrer betennelse av blodkar og bindevev i en rekke organer med varierende lokalisering og alvorlighetsgrad. Positiv ANA er nesten alltid til stede, mens positiv nativt anti-DNA og SM-antigen har høy spesifisitet for sykdommen.
KLASSIFIKASJON
ACR-kriteria 1982 (1)• Sommerfuglutslett
INSIDENS OG PREVALENSDet finnes sparsomt med data om insidens og prevalens av SLE hos barn.
Insidens