Although African children
with SLE may be at high risk for poor outcomes based on race, socioeconomic
status, and age, little research has investigated this population.  We have
initiated the first registry of this high risk pediatric SLE (pSLE) population
in South Africa (SA).  Here, we present the first comparison of treatment and
outcomes between a South African pSLE cohort (PULSE) and a North American pSLE
cohort (Childhood Arthritis and Rheumatology Research Alliance (CARRA)
Registry).

  Methods:    

We conducted a cross
sectional analysis (retrospective chart review) of pSLE patients in Cape Town, SA
from 1988-2014 meeting ACR criteria for pSLE.  Patient age, gender, race,
presenting features, clinical and serologic disease markers, and treatment were
recorded and compared to an established North American pSLE cohort.

  Results:

The SA cohort
includes 72 patients; mean age 11.5 years, 83% female. The SA cohort had increased
rates and severity of lupus nephritis and high disease activity at enrollment (Table
1) SA patients had increased use of cyclophosphamide (942% vs 29%),
methotrexate (30% vs 19%), and azathioprine (61% vs 16%). PSLE patients in the
CARRA cohort had increased use of antimalarials and mycophenolate mofetil (Table
2). The PULSE cohort had high rates of end organ damage with 63% having a
SLICC-DI score >0. Within the SA cohort, 13% went on to develop ESRD, of
which 9% required transplant, strikingly higher than North American peers. (Table
3)

  Conclusion:

The PULSE cohort is
the largest registry of pSLE patients in Africa to date. Children in SA receive
different therapy and demonstrate a striking increase in poor renal outcomes,
end stage renal disease, and irreversible organ damage compared to North
American peers. These differences may be due to treatment, health care access, racial
predisposition, or a combination of factors. Further prospective research is
required to determine the burden of pSLE in South Africa, and identify risk
factors for poorer outcome in this high risk population.

                                                Table
1. Features at Enrollment

                        Table
2. Treatment History at Enrollment

                        Table 3. Disease Damage at
Enrollment

*NR indicates not reported in this cohort.