Objective: To determine the frequency of systemic lupus erythematosus (SLE)-associated clinical manifestations, autoantibodies, and HLA class II alleles in a large cohort of patients with childhood-onset SLE.
Methods: Eighty children with SLE onset before age 18 (27 before age 11) were studied for the frequency of renal, neuropsychiatric, and hematologic complications as well as for anti-native DNA, Ro, La, Sm, and U1 RNP autoantibodies. HLA-DR, DQ, and DP alleles were determined by oligotyping. The results were compared with findings in 213 adults with SLE onset at or after age 18 years.
Results: Renal involvement was more frequent in those with childhood-onset SLE, especially those with onset before age 11 (82%, compared with 53% in adults). Anti-U1 RNP was more common in American blacks with SLE onset before age 18. HLA-DRB1*0301, DQA1*0501, DQB1*0201 was more common in Caucasians and DRB1*1503, DRB5*0101, DQA1*0102, DQB1*0602 in American blacks, regardless of age at SLE onset. Anti-Sm autoantibodies were most highly associated with HLA-DQA1*0102 and DQB1*0602.
Conclusion: While childhood-onset SLE shares many immunogenetic and serologic similarities to adult-onset disease, important clinical differences nevertheless exist in children with this disease.