Epidemiologi ved sklerodermi hos barn

Sammendrag

Localized scleroderma is an infrequent disease in children, but still ten times more frequent than systemic sclerosis. The incidence is probably 5-10/100,000 per year. The disease is classified into five subgroups. Paediatric rheumatologists report linear scleroderma as the most frequent subgroup whereas plaque morphea is found most frequently by dermatologists, linear scleroderma being in second place. The risk of transforming into systemic sclerosis is minimal. The mortality risk is not found increased compared with the normal population. Systemic sclerosis is the most infrequent systemic connective tissue disease in children, the incidence is less than 1/1,000,000 per year. The outcome in children is considered to be generally better than in adults. However, deaths is caused most frequently by heart failure with or without pulmonal hypertension, but also by renal or respiratory insuffiency, CNS disease and sepsis. Cumulative survival after 5, 10, 15 and 20 years of disease is reported to be 89%, 80%, 74% and 69%. For the age group less than 16 years the median age of death is reported to be 10.4 years, range 5 to 15 years. The disease in the children who die is often rapidly progressing with early involvement of internal organs.