Childhood-onset Systemic Lupus Erythematosus: clinical presentation and prognosis in 31 patients

Abstract

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.