Young age at onset, renal involvement, and arterial hypertension are of adverse prognostic significance in juvenile systemic lupus erythematosus.
OBJECTIVE
To look for associations between mortality, clinical or laboratory data, and age at disease onset in systemic lupus erythematosus patients aged 16 years or younger at disease onset.


METHODS
The medical records of patients seen at the Clinics Hospital, State University of Campinas, São Paulo, Brazil, between 1979 and 1995 were reviewed retrospectively. All 59 included patients (48F/11M) fulfilled four or more American College of Rheumatology criteria for systemic lupus erythematosus. Patients with discold, drug-induced or neonatal lupus, or other systemic connective tissue diseases were excluded. Patients were studied individually then classified in three groups based on age at disease onset, as follows: Group I, < or = 9 years of age; Group II, 10-14 years of age; and Group III, 15-16 years of age. Clinical and laboratory abnormalities and mortality were compared in the three groups.


RESULTS
The most frequent clinical manifestations were joint symptoms (91.5%), renal involvement (71.1%), malar rash (61%), alopecia (61%), fever (59.3%) and photosensitivity (52.5%). Laboratory findings included antinuclear antibody in 94.9% of cases. LE cells in 71.1%, low serum complement in 65.3%, anti-DNA in 63.4%, hematuria in 62.7%, and proteinuria in 61%. The mortality rate was 23.7% (9F/5M) overall, 18.7% in females, and 45.4% in males (P = 0.07). The cause of death was infection in eight patients (57.1% of decedents), central nervous system disease in five (35.7%), and renal insufficiency in one (7.2%). Disease onset before 15 years of age (P = 0.026), renal involvement (P = 0.03), and arterial hypertension (P = 0.002) were predictive of mortality. Mortality was not influenced by gender or race.
Pediatrics Department, School of Medical Sciences, State University of Campinas, São Paulo, Brazil.
R
R
Marini
L T
LT
Costallat
D000293:Adolescent; D017677:Age Distribution; D017668:Age of Onset; D001938:Brazil / Q000453:epidemiology; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D006801:Humans; D006973:Hypertension / Q000175:diagnosis / Q000453:epidemiology*; D015994:Incidence; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis / Q000453:epidemiology*; D008181:Lupus Nephritis / Q000175:diagnosis / Q000453:epidemiology*; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D017678:Sex Distribution; D015996:Survival Rate