Background/Purpose: Ancestral background may contribute to increased disease morbidity in patients with pediatric lupus systemic erythematosus (pSLE) of non-European descent; however, there exists a paucity of information describing the effects of ancestry on the early disease processes of pSLE. We recently identified that pSLE patients of not uniquely European ancestry (nonEA) at a tertiary care center in Los Angeles, California, were more likely to present with more active disease during the first 12 months of diagnosis. We aim to assess whether children and adolescents with pSLE of nonEA ancestry have a more severe early disease course than their European American counterparts.

Methods: CARRAnet Registry data was obtained for 665 subjects with pSLE (diagnosis <18 years); 130 were further identified as diagnosed within 12 months prior to their baseline visit between 2010 and 2012. Demographic and socioeconomic data, ACR SLE criteria, and SLE Disease Activity Index (SLEDAI) were evaluated at baseline, and any renal biopsy results were recorded. Cumulative steroid exposure and use of additional immunosuppressive agents was also documented. Impact of disease was assessed, using ACR functional class, health-related quality of life, Childhood Health Assessment Questionnaire (CHAQ), and physician global assessment (PGA). Comparison of these data between self-reported Hispanic- (HA), Asian- (A), African- (AA), non-European (nonEA; including all subjects of mixed ancestry or of not uniquely European ancestry), and European- (EA) American patients with pSLE was conducted.

Results: Age at onset was slightly higher among HA, A, and nonEA patients when compared with EA pSLE (Table 1). All Non-EA pSLE groups were more likely to have renal involvement during year 1 than EA; similarly, HA, A, and nonEA were more likely to present with proteinuria than EA (22%, 30%, 17% vs. 12.5%). However, total ACR criteria and SLEDAI scores near diagnosis did not differ among all non-EA groups compared to EA (Table 1). HA, AA, and nonEA pSLE received more immunosuppressive agents in addition to steroids, and more A pSLE had >1 month of cumulative steroids compared to EA pSLE (Table 1). Furthermore, A pSLE patients had more active PGA scores than EA. Overall, the EA and all non-EA groups had comparable duration of symptoms prior to diagnosis despite lower rates of insurance coverage in HA and nonEA and more HA, AA, and nonEA subjects reporting poverty-level household incomes than EA (Table 1).

Conclusion: CARRAnet Registry pSLE patients of not uniquely European ancestry present at a slightly older age and potentially reflect a more active and refractory disease during the initial 12 months compared to EA counterparts. As the CARRAnet Registry continues to grow and additional follow-up data is collected, trends may emerge to further support ancestry-related differences in disease severity in the early stages of pSLE.

 

Table 1: Clinical and socio-economic characteristics of EA, nonEA, HA, AA, and A pSLE patients.

	EA	NonEA	p*	HA	p*		AA	p*	A		p*
Total pSLE Cohort, n	177	488		118			203		68
Female:Male	149:28	410:78		100:18			166:37		60:10
Age at diagnosis, Mean years (range)	12.6 (3-18)	12.7 (4-18)		12.3 (3-18)			12.8 (4-18)		12.8 (5-18)
Early disease cohort, n	32	98		36			40		10
Female:Male	30:2	118:12		33:3			37:3		9:1
Age at onset, mean years	12.5	13.3	0.07	14.3	0.02		12.6	0.8	14.2		0.06
Duration of symptoms, mean‡	5.9	6.1	0.4	6.6	0.2		6.1	0.4	5.8		0.5
Duration of diagnosis, mean‡	3.7	5.6	0.3	4.3	0.2		4.1	0.3	3.1		0.3
Duration of symptoms prior to diagnosis, mean	2.3	2.3	0.5	2.3	0.5		2.2	0.4	3.1		0.1
Clinical
ACR SLE Criteria, mean (range)	4.5 (1-8)	4.6 (1-8)	0.4	4.6 (1-6)		0.4	4.7 (2-8)	0.3	4.8 (2-7)	0.3
SLEDAI at baseline‡, median (range)	4 (0-32)	4 (0-34)	0.2	4 (0-26)		0.2	4 (0-34)	0.4	4 (0-17)	0.3
Arthritis, %	59	66	0.4	69		0.2	70	0.2	70	0.2
Renal involvement, %	38	52	0.03	47		0.2	50	0.07	70	<0.01
Cumulative steroid use, %†	88	82	0.4	81		0.3	75	0.03	100	<0.01
Immunosuppression, mean††	0.78	0.9	0.3	0.83		0.8	1.0	0.3	0.7	0.8
Quality of life
PGA, mean	2.5	2.8	0.3	2.5		0.5	2.9	0.3	3.75	0.04
CHAQ, mean	0.22	0.29	0.2	0.33		0.2	0.30	0.2	0.25	0.2
Sociodemographic
Household income <$25,000; %	0	20	<0.001	25		<0.001	23	<0.001	0		0.2
Insurance status, %	94	91	0.6	81		0.01	95	1	100		0.03
EA-European American; HA-Hispanic American; AA-African American; A-Asian American; nonEA-of mixed ancestry or not uniquely of EA descent, including HA, AA, and A groups; SLEDAI-SLE Disease Activity Index; PGA-physician Global Assessment; CHAQ-Childhood Health Assessment Questionnaire *p-values taken in comparison to EA values and considered significant at p< 0.05 ‡Values assessed at time of enrollment/baseline visit, which occurred within 12 months of symptom onset and 12 months of diagnosis † % of patients requiring cumulative prednisone >1 month ††Number of immunosuppressive agents required in addition to steroids and/or hydroxychloroquine

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/ancestral-group-differences-in-pediatric-sle-early-disease-severity-an-analysis-of-the-carranet-registry/