Background/Purpose: The aim of this study was to investigate differences in clinical features and outcomes including mortality between pediatric-onset systematic lupus erythematosus (pSLE) and adult-onset SLE (aSLE).

Methods: In total, 972 SLE patients were enrolled in the Hanyang BAE lupus cohort in Seoul, Korea, between February 1998 and December 2010. For mortality analysis, only 766 of the 972 SLE patients enrolled up to 2008 were included, because mortality data were prepared by data linkage with the available data from the Korean National Statistical Office. Mortality between the 2 groups was compared using the survival rates with Kaplan-Meier and the standardized mortality ratio (SMR). Multivariate linear regressions were used to determine the predictors of increased mortality.

Results: There were 111 (11.4%) pSLE patients and 861 (88.6%) aSLE patients. The female: male ratio in pSLE (3:1) is lower than that seen in aSLE (18:1). The duration of follow-up was similar in the two groups, with 5.7 years in the pSLE group and 5.6 years in the aSLE group. The frequency of malar rash, photosensitivity, renal disorder, neurologic disorder and immunologic disorder in ACR criteria were significantly higher in pSLE patients, whereas arthritis was more common in aSLE. The maximum value of the SLE Disease Activity Index (SLEDAI) scores (21.1 versus 9.6; p=0.0006), and the adjusted mean SLEDAI scores (AMS) during followup period were significantly higher in pSLE groups (5.2 versus 4.2; p=0.0006). The mean SDI scores were 0.9 in the pSLE group and 0.8 in the aSLE group, and pSLE patients were more frequently affected in neuropsychiatric (14.4% vs. 8.4%; p=0.022) and renal system (18% vs. 9.3%; p=0.006).

Among 766 SLE patients (4,530 person-years), a total of 29 cases of death were confirmed. In pSLE, lupus-related deaths were most frequent (57.1%) whereas, deaths related infection were most common in aSLE (32.0%). The cumulative probabilities of survival at 5, 10 years were 97.6%, 89.3% in pSLE, and 98.2%, 96.4% in aSLE (log rank; p=0.035). Compared with an age-, sex-, and calendar-matched general population, the SMR of patients with pSLE and aSLE were 50.0 (95% CI 20.1-103) and 4.8 (95% CI 3.1-7.4), respectively. In multivariate regression analysis, pediatric onset lupus, shorter disease duration, higher disease activity, and higher disease damage were identified as predictor of mortality. In particular, the presence of neuropsychiatric damage (HR 3.8 vs. 96.6; p=0.0097) and hemolytic anemia (HR 2.5 vs. 22.5; p=0.0155) increased the mortality significantly in pSLE. 

Conclusion: This study demonstrates that the patients with pSLE have higher rate of major organ involvement and worse clinical outcomes with higher mortality. In particular, special attention will be needed when combined with neuropsychiatric damage or hemolytic anemia in pSLE.

 

	Pediatric-onset         (N=111, 11.4%)	Adult-onset      (N=861, 88.6%)	P
Gender      Women	86(77.5%)	817(94.9%)	<0.0001
Age (yrs)
Age at Diagnosis	12.4 ± 2. 5 (5-15)	29.4 ± 9.6 (16-68)	<0.0001
Time(onset to diagnosis)	0.7 ± 1.7 (0-10.9)	2.0 ± 3.7 (0-30.2)	–
Follow up period	6.4 ± 3.8 (0-13.7)	6.3 ± 4.1 (0-13.7)	–
Disease duration        (at last followup)	9.5 ±4.5(1.2-18.8)	9.3 ± 5.2 (0-33.3)	–
SLEDAI score
At enrollment	5.6 ± 4.6 (0-28	5.4 ± 4.3 (0-22)	–
Maximum	21.1 ± 7.2 (0-45)	9.6 ± 5.3 (0-38)	0.0006
Adjusted mean SLEDAI	5.2 ± 2.9 (0-14.5)	4.2 ± 2.6 (0-18.4)	0.0006
SDI score
At last followup	1.0 ± 1.6 (0-8)	0.9 ±1.5 (0-9)	–
Renal SDI ≥ 1	20 (18.0%)	82 (9.5%)	0.0098
CNS SDI ≥ 1	17 (15.3%)	74 (8.6%)	0.0345
Mortality
SMR	50.0 (95% CI 20.1-103)	4.8 (95% CI 3.1-7.4)
5 years survival rates	97.6%	98.2%	0.035
10 years survival rates	89.3%	96.4%	0.035

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/difference-in-clinical-features-and-mortality-between-pediatric-onset-and-adult-onset-systemic-lupus-erythematosus/