Childhood-onset systemic lupus erythematosus.
OBJECTIVE
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.


CONCLUSIONS
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.
Paediatric Nephrology and Hypertension Unit, Obafemi Awolowo University Teaching Hospitals Complex, PMB 5538, Ile-Ife, Osun State, Nigeria. yetundeolowu@yahoo.com
Wasiu
W
Olowu
D000293:Adolescent; D017668:Age of Onset; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D008297:Male; D009549:Nigeria / Q000453:epidemiology; D011239:Prednisolone / Q000627:therapeutic use; D011446:Prospective Studies
D000893:Anti-Inflammatory Agents; D003520:Cyclophosphamide; D011239:Prednisolone