VIENNA — Nearly two-thirds of a cohort of childhood-onset systemic lupus erythematosus patients had evidence of irreversible organ damage after a decade of disease, Vibke Lilleby, M.D., said at the annual European congress of rheumatology.
The most frequently affected organ systems were the neuropsychiatric, in 28% of cases; renal, in 13%; and musculoskeletal, in 13%, according to Dr. Lilleby of the University of Oslo.
Her 71-patient series with childhood-onset SLE had a mean age of 12.5 years at lupus symptom onset and a 10.8-year disease duration. Their mean Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)—a validated measure of nonreversible organ damage—was 1.3.
The independent predictors of a higher SDI were ever having taken cyclophosphamide, hypertension, and disease duration. In contrast, cumulative corticosteroid dose, the presence of renal disease at diagnosis, and erythrocyte sedimentation rate at diagnosis were among the examined factors that did not predict subsequent organ damage.
The high rate of irreversible organ damage in this cohort is a byproduct of the greatly improved long-term prognosis for childhood-onset SLE during the past 4 decades. Patients who in former years would have had a poor life expectancy are surviving much longer, with an associated increase in multiorgan morbidity due to the disease process itself or to its treatment, she explained at the congress, sponsored by the European League Against Rheumatism.
In a separate comparative study involving 70 of the same Norwegian patients with childhood-onset SLE and an equal number of matched controls, Dr. Lilleby found that osteopenia was much more frequent in the childhood-onset SLE group. For example, 41% of them had osteopenia at the lumbar spine and 40% at the femoral neck vs. 7% and 6% of the controls.
Cumulative dose of corticosteroids was a strong predictor of reduced bone mass at these sites. Reduced bone mineral density at the lumbar spine was also associated with male gender.