Background/Purpose: In developed nations, SLE is more common and severe in people of African extraction than in Caucasians; however, the epidemiology of SLE in Africa is largely undetermined. Historically, the incidence of SLE in Africa was presumed to be low, but recent studies challenge this theory. In general, children present with higher disease activity, require more therapy, and accrue more organ damage than adult-onset patients. Although African children with SLE may be at high risk for poor outcomes, little research has investigated this population.  We have initiated the first prospective study of this high risk pediatric SLE (pSLE) population.  Here, we report the initial findings of the South African pSLE patients (PULSE cohort).

Methods: We conducted a retrospective chart review of pediatric and adult rheumatology and nephrology patients seen at 2 centers in Cape Town, South Africa from 1988-2014 meeting American College of Rheumatology criteria for pSLE.  Patient age, gender, race, and presenting features were recorded for the PULSE cohort and compared to previously described pSLE cohorts in South Africa and worldwide.

Results ; Initial review of patients yielded 68 patients (age 12.2; 83% female). The racial distribution was 65% colored, 26% black, 3% white, and 3% Asian/Indian. A much larger proportion of patients in our cohort are of colored or black race compared to a previously published South African cohort. Most patients presented with severe lupus nephritis (LN) (renal biopsy performed in 49%)). Of patients with LN, 83% presented with ISN class IV or higher.  Pediatric LN cohorts from developed nations report 6-7% progressing to end stage renal disease (ESRD), and reports from developing nations report 8-12%. Within the cohort, 13% went on to develop ESRD requiring transplant, strikingly higher than previously reported cohorts. Our cohort had severe disease at diagnosis (mean SLEDAI 20.4), compared to previously reported pSLE cohorts (SLEDAI 4-13). Also, the PULSE cohort had end organ damage with 63% of the cohort having a SLICC score >0 (mean SLICC 1.9), compared to only 23% in a previously reported US cohort of 221 pSLE patients.

Table 1. Demographic Information
	PULSE cohort South Africa 2014 n=68	Faller cohort (South Africa, 2005)  n=36	APPLE cohort (USA, 2010) N=221
Average age (years)	12	11.5	15.7
Median Age (years)	12.2	Not reported	15.5
% Female	83	61	83
% Colored	65	14	Not reported
% Black	26	38	27 (African Am)
% Indian/Asian	3	1	13
% White	3	42	51

Table 2. International Society of Nephrology/ Renal Pathology Society 2003 Classification of Lupus Nephritis
Class of Lupus Nephritis	PULSE cohort South Africa 2014 N=32	Toronto Cohort N=43
I Minimal Change	0 (0%)	0 (0%)
II Mesangial	3 (9%)	10 (23%)
III Focal Proliferative	2 (6%)	11 (26%)
IV Diffuse Proliferative	17 (53%)	17 (40%)
V Membranous	7 (21%)	5 (11%)
VI Advanced Sclerosis	3 (9%)	Not Reported

Conclusion: The PULSE cohort is the largest registry of pSLE patients in Africa to date. Preliminary findings show these children present with high disease activity and progress to end organ damage at higher rates than pSLE cohorts in developed nations.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pulse-pediatric-update-on-lupus-in-south-africa-epidemiology-and-management/