Comment on: Long-term outcomes of childhood-onset systemic lupus erythematosus

Dear Editor, We read with great interest the recent article by Mirguet et al. [1] in which they retrospectively evaluated the long-term outcomes of childhood-onset SLE (cSLE) and identified the duration of follow-up and Sub-Saharan African ethnicity were associated with cumulative organ damage and disease activity. We noticed that 77 (55.8%) patients in the study were Caucasians, Africans accounted for 32 (24.2%) persons, while Asian and other ethnicity were only 29 (21.9%) persons. It is important to note that clinical features and disease progression of cSLE varied based on different ages of onset, ethnicity and gender [2–4]. We estimated that the primary data from the SOPHIE registry (ClinicalTrials ID: NCT03446339) may provide supplementary information on organ damage in Asian with cSLE (Table 1). Comparisons of demographic and clinical data in patients with cSLE between SOPHIE and PEDIALUP registry at last visit 13.4 ± 3.2 14.0 (12.0, 16.0) 12.8 ± 2.7 13.1 (10.9, 14.5) 15.8 ± 9.6 15.4 (9.6, 22.4) 13.4 ± 3.2 14.0 (12.0, 16.0) 12.8 ± 2.7 13.1 (10.9, 14.5) 15.8 ± 9.6 15.4 (9.6, 22.4) FU: follow-up; ESRD: end-stage renal disease. Comparisons of demographic and clinical data in patients with cSLE between SOPHIE and PEDIALUP registry at last visit 13.4 ± 3.2 14.0 (12.0, 16.0) 12.8 ± 2.7 13.1 (10.9, 14.5) 15.8 ± 9.6 15.4 (9.6, 22.4) 13.4 ± 3.2 14.0 (12.0, 16.0) 12.8 ± 2.7 13.1 (10.9, 14.5) 15.8 ± 9.6 15.4 (9.6, 22.4) FU: follow-up; ESRD: end-stage renal disease. A total of 124 patients with cSLE in our registry were included in the cross-sectional analysis (Table 1). The mean age of the study population was 38.3 ± 10.8 years. The mean age at diagnosis was 13.4 ± 3.2 years, which was comparable to the cited study. In the SOPHIE study, a higher proportion of patients (60.5% vs 36.9%) was diagnosed with cSLE between 1972 and 2015. And thus, a longer duration of follow-up in the SOPHIE registry (24.2 ± 10.7 years) than that of the PEDIALUP study (15.8 ± 9.6 years). Comparisons of clinical manifestations at the last follow-up were made between the two studies. Specifically, a total of 20 (16.1%) patients were identified with vascular disease (including aortic aneurysm, venous thrombosis and vasculitis) in the SOPHIE study, which was significantly higher when compared with the PEDIALUP study. Neurological manifestations including stroke, syncope and transient ischaemic attack were observed in 14 (11.3%) patients in the SOPHIE registry. However, the difference in neurological features was not significant between the two groups. The proportion of patients with systemic lupus erythematosus disease activity index (SLEDAI) scores ≥6 was similar between the two study populations, whereas a larger percentage of people with SLEDAI scores ≥8 in the PEDIALUP study (26.8%) compared with the SOPHIE registry (12.9%). Furthermore, we contrasted our results with study from the University of Toronto that had a comparable mean age of diagnosis [5]. The Toronto trial exhibited a greater incidence of cerebrovascular disease than ours indicated (26.6% vs 11.3%, P = 0.00). There were substantial differences in treatment approaches between the PEDIALUP study and SOPHIE. In the SOPHIE registry, a greater percentage of individuals underwent steroid therapy (78.2% vs 64.0%), whereas a lower number of patients used HCQ (62.9% vs 83.0%). Overall, those results suggested that the long-term outcome of cSLE in organ damage was a serious concern and can be varied with race and follow-up duration. Additionally, the authors demonstrated that the proportion of patients with a SLICC-DI score of ≥1 was significantly different across groups of follow-up <10 years, follow-up between 10 and 20 years and follow-up over 20 years. Whether age and disease duration have been included in the adjustment of the difference was not addressed in the manuscript. Thus, we proposed that an adjustment specific to the age and disease duration would be performed owing to patients with 10 years or above follow-up duration could be older and with longer disease duration compared with those patients with shorter follow-up duration. Tsakonas et al. [6] conducted a study on the long-term effects of HCQ withdrawal on SLE exacerbations. They reveal that patients randomized to continue HCQ treatment had a lower incidence of major flares compared with those who received a placebo. Recent advances in the management of SLE, especially the evolution of the combination therapy (biologics with standard care), have been proven in reducing disease activity and steroid use as well as prolong remission in patients with SLE [7, 8]. Future studies may focus on the evaluation of personalized treatment approaches including biologic therapies, dose adjustments, and medication adherence on the long-term outcomes for cSLE patients. The data will be shared on reasonable request to the corresponding author. The study was partially funded by the Chengdu Municipal Health Commission (NO 2023049). Disclosure statement: The authors have declared no conflict of interest.

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