Outcome in juvenile onset systemic lupus erythematosus.
OBJECTIVE
Over the past 2 decades, there has been a marked improvement in survival among patients with juvenile-onset systemic lupus erythematosus. As a result of the increased life expectancy, children and adolescents with systemic lupus erythematosus are now faced with considerable morbidity resulting from sequelae of disease activity, side effects of medications, and comorbid conditions. This morbidity affects physical and psychosocial well-being. Therefore, the need is increasing for monitoring the development of irreversible organ damage and the effect of the disease and its treatment on daily life. This review summarizes the recent advances in the investigation on survival, accumulated damage, and health-related quality of life in patients with juvenile-onset systemic lupus erythematosus.


RESULTS
The 5-year survival rate of patients with juvenile-onset systemic lupus erythematosus approaches 100%, and the 10-year survival rate is close to 90%. The development of cumulative organ damage has been observed in 50-60% of patients. Children and adolescents with systemic lupus erythematosus have been found to have poorer health-related quality of life, particularly in the physical domain, and lower socioeconomic achievements than their healthy peers.


CONCLUSIONS
The prolongation of the life span of patients with juvenile-onset systemic lupus erythematosus has been accompanied by a substantial risk of damage accumulation and has not been paralleled by an improvement in health-related quality of life. This problem highlights the need of measuring cumulative organ damage and health-related quality of life in the long-term follow-up of patients with juvenile-onset systemic lupus erythematosus and of designing new treatments and treatment strategies that are aimed not only at improving control of disease activity but also at minimizing the development of nonreversible damage.
Ravelli
Angelo
A
Dipartimento di Pediatria, Università di Genova, Unità Operativa Pediatria II, Istituto di Ricovero e Cura a Carattere Scientifico G. Gaslini, Italy. angeloravelli@ospedale-gaslini.ge.it
Ruperto
Nicolino
N
Martini
Alberto
A
D017668:Age of Onset; D002648:Child; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000401:mortality* / Q000503:physiopathology*; D011788:Quality of Life