Mj. Hood et al., Childhood-onset systemic lupus erythematosus - Clinical presentation and prognosis in 31 patients, SC J RHEUM, 28(4), 1999, pp. 222-226
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 r
eviewed. Signs and symptoms at onset and during the course of the disease w
ere 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, arthri
tis, fever, weight loss, and malar rash. During follow-up, the frequency of
the presence of malar rash, anemia, leukocytopenia, and anti-dsDNA antibod
ies was significantly higher in childhood-onset than in adult-onset patient
s. Mean SLICC/ACR damage index was 2.6 after 4.7 years of follow-up. The pr
esence 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 morbi
dity. Arthritis, anemia, and seizures at onset may be indicators of poor pr
ognosis.