Background/Purpose: This research explores whether delay of the childhood-onset SLE (cSLE) diagnosis until 4/11 ACR criteria are met affects patient outcome negatively

Methods: Institutional Review Board approval was obtained to retrospectively review the charts of 98 SLE patients seen in the rheumatology clinic at Nationwide Children’s Hospital over the past 24 years. All the patients were divided in to two groups, ‘definitive cSLE’ – who met the minimum 4/11 or more ACR criteria at presentation in rheumatology clinic and the ‘probable cSLE’ who did not meet the minimum criteria. Both the groups were assessed for disease severity, damage and gradient of damage. Appropriate statistical tests were used, i.e. Chi-Square test, Fisher’s Exact test, Univariate logistic regression and Wilcoxon two-sample test were utilized. All tests were conducted in SAS 9.2

Results: Out of 98 cSLE patients, 71 % were included in definitive cSLE (DcSLE) group while 29 % were included in probable cSLE (PcSLE) group. The mean time for PcSLE group to reach DcSLE status was 20.3 months There was no difference in the ethnic distribution (p=0.7370). PcSLE were more likely to have a higher male: female ratio (p=0.032), and were older at presentation that DcSLE (p=0.045). PcSLE patients were less likely to have internal organ involvement (7.1% vs. 25.7%), were less likely to be hospitalized and receive pulse steroids (P=0.0142) or oral steroids (0.0172) at presentation. PcSLE patients were less likely to be hospitalized to receive pulse steroids ever (p=0.0628), were less likely to have renal disease ever (p=0.0653) and nervous system disease ever (p=0.0182). PcSLE was more likely to receive hydroxychloroquine (p=0.050). The organ damage was assessed using SLICC/ACR damage index at 1, 5 and 10 years post diagnosis. The maximum damage was recorded within first 5 years of the diagnosis. Initial damage was predictive of later damage. D pSLE had higher disease damage scores at 5 and 10 years. We compared the gradient between the onsets of symptoms and the development of organ damage in the two groups. The PcSLE patients had significantly higher internal organ damage gradient as compared to DcSLE (p value=0.0169)

Conclusion: In our population, PcSLE patients had a significantly high gradient of damage than the DcSLE group. In spite of D pSLE being more severe diseases ever and more diseases damage, the disease damage progression was steeper and faster in PpSLE. This may be explained by the fact that PpSLE patients received a less intense treatment regimen at presentation than DcSLE group. It may be that PcSLE patients need just as early vigorous treatment as the children with DcSLE

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/children-with-probable-sle-by-acr-criteria-may-need-more-aggressive-lupus-treatment-early-in-the-disease-course/