Childhood-onset systemic lupus erythematosus - Clinical presentation and prognosis in 31 patients

Objective. To determine the clinical features at onset, the disease course, and prognostic factors in children with SLE. Methods. The medical records of 31 patients with childhood-onset SLE were reviewed. Signs and symptoms at onset and during the course of the disease were documented as well as survival and SLICC/ACR damage index. The disease course was compared to 135 consecutive adult-onset SLE patients. Results. Childhood-onset SLE most frequently presented with fatigue, arthritis, fever, weight loss, and malar rash. During follow-up, the frequency of the presence of malar rash, anemia, leukocytopenia, and anti-dsDNA antibodies was significantly higher in childhood-onset than in adult-onset patients. Mean SLICC/ACR damage index was 2.6 after 4.7 years of follow-up. The presence of arthritis, anemia, and seizures at the onset of disease resulted in a 2.6 to 3.9 times higher chance of a severe disease course. Conclusion. Patients with childhood-onset SLE suffer from substantial morbidity. Arthritis, anemia, and seizures at onset may be indicators of poor prognosis.