Juvenile onset SLE can be life threatening during childhood and adolescence, can cause significant organ damage with subsequent long-term implications, and will continue to require monitoring and treatment into adult life. Therefore, this disease almost certainly has a significant long-term impact on affected children and adolescents. However, outcomes can be optimized with careful attention to factors relating to lupus disease activity, medication side effects, and healthy lifestyle choices (Table 3).
Mortality of juvenile onset SLE, as measured in the "short term" (5- and 10-year survival), has most certainly improved greatly over the last decades. Survival for children and adolescents with SLE is excellent in most pediatric rheumatology centers, with recent data showing 5-year survival of 100%, and 10-year survival of 85% in a cohort of patients followed in the British Columbia Children's Hospital Pediatric Rheumatology Program. (9). In a cohort of pediatric SLE patients with nephritis followed in Toronto
Table 3 Modifiers for Improved Outcomes in SLE
Weight reduction Increased physical activity No smoking
Monitoring lipid levels and consideration of medical treatment Vigilant treatment of hypertension
Screening for bone density and treatment of patients with low density Aggressive treatment of lupus disease activity
Canada, survival was 94% at an average of 11 years follow-up (32). A report of a similar cohort of pediatric SLE patients with nephritis from Italy showed 75% patient survival at 10 years (37). These data compare with survival rates in the 1970's of less than 40% at 10 years for pediatric patients with SLE and renal disease, and 75% at 10 years for pediatric patients treated with immunosuppressive medications (33).
One method of measuring impact of SLE over time is to use a standardized tool which measures organ damage due to SLE and its treatments. The Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index (SDI) has been developed to measure irreversible damage in a variety of organ systems due to SLE or its treat-ments(34,35). This tool has been used in a number of outcome studies of children and adolescents with SLE, uniformly showing gradual increase in disease damage scores over time. Of interest, most studies show that children and adolescents appear to accumulate damage more quickly than adults with SLE (14,22,36,37), with higher scores after a similar period of disease. These data suggest that the long-term impact of SLE for adolescents will be significant over their lives, as the accumulated damage to kidneys, central nervous system, musculoskeletal system and other organs will certainly cause functional problems for these patients.
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