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.
Olowu
Wasiu
W
Paediatric Nephrology and Hypertension Unit, Obafemi Awolowo University Teaching Hospitals Complex, PMB 5538, Ile-Ife, Osun State, Nigeria. yetundeolowu@yahoo.com
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