Childhood-onset systemic lupus erythematosus

Abstract

Objectives: To describe the initial clinicolaboratory manifestations and short-term outcome in a series of Nigerian children with systemic lupus erythematosus (SLE). Methods: A nonrandomized prospective study of consecutive cases of childhood-onset SLE. Baseline and follow-up clinicolaboratory data were collected and analyzed. Each patient was followed up for 12 months. Results: Eleven children were studied. There were seven girls (F:M, 1.75). Mean ages at lupus onset and diagnosis were 10.0 ± 2.53 years and 11.2 ± 2.53 years, respectively. Mean time at onset of renal disease following SLE symptoms onset was 1.22 ± 0.93 years. All cases were misdiagnosed prior to presentation; diagnosis was delayed in nine patients. Lupus activity was mild, moderate and severe in two, five and four patients, respectively. Hypertension (n=5), nephrotic syndrome (n=6), microerythrocyturia (n=6) and acute renal failure (n=7) were associated morbidities. Of the 27 presenting clinical features, 17 were nondiagnostic, while 10 were diagnostic. Fever (n=9) was a major nondiagnostic symptom; major diagnostic manifestations were lupus nephritis (n=11), arthritis (n=10) and serositis (n=7). Catastrophic antiphospholipid syndrome was diagnosed in three. The glomerular lesions were nonproliferative (n=1), focal (n=3) and diffuse (n=7) proliferative lupus nephritis. Complete remission rate at end-point was 71.4%. Fourteen percent of the patients relapsed. Renal survival and mortality rates were 86.0% and 30.0%, respectively. Conclusion: In this study, severe renal and extrarenal comorbidities were common; mortality rate was also high. High frequency of misdiagnosis and delayed diagnosis were probably responsible for these.