Systemic lupus of pediatric onset in Afro-Caribbean children: a cohort study in the French West Indies and French Guiana

Abstract

Background Systemic diseases of pediatric onset are more frequent in the Afro-Caribbean population. We performed a study of patients followed in the French overseas departments of America (FOAD) for pediatric systemic lupus erythematosus (pSLE). The aims were to describe the clinical and biological specificities during childhood in this population. Methods A retrospective study was conducted between January 2000 and September 2021. Patients with pSLE were identified from multiple sources: computerized hospital archives, registry of referring pediatricians, adult specialists in internal medicine and the French National Registry for rare diseases. We studied SLE with pediatric onset defined by international criteria. Results Overall, 2148 patients were identified, of whom 54 were included. The average follow-up was 8.3 years (range: 0.3—25 years). We observed an increase in new diagnoses over time. At onset, pSLE patients had a median of 10 SLICC criteria (range: 4–12), and the median EULAR/ACR 2019 score was 38 (12—54). At onset, one third of patients had renal involvement, 15% had neurolupus and 41% cardiac involvement. During childhood, 54% had renal involvement, and 26% suffered from neurolupus. Patients suffered a median of 3 flares during childhood, and 26% had more than 5 flares. Patients with younger age at onset had worse outcomes than those who were older at diagnosis, i.e., more flares (median 5, p = 0.02) and requiring an average of 4 background therapies (p = 0.04). Conclusion The outcomes of Afro-Caribbean patients were similar to those in Western population, but with worse disease activity at onset. Further studies should be performed to identify the genetic and environmental factors in this population.

Juvenile Dermatomyositis in Afro-Caribbean children: a cohort study in the French West Indies

“…Overall, the outcomes of Afro-Caribbean children followed-up and treated in the FWI for JDM was comparable to North America and European countries. This florid symptomatology at diagnosis with a similar overall prognosis has previously been described as a characteristic of our population in another connective tissue disease namely juvenile systemic lupus [ 10 ]. In studies from North America or Africa, African descent seems to be associated with worse prognosis in JDM [ 3 , 11 , 12 ].…”

“…This incidence is similar to North America and European countries [ 19 ]. In contrast, the incidence of another connective tissue disease (lupus) is much more important in our Afro-Caribbean population in the FWI [ 10 ].…”

Description and outcomes of Afro-Caribbean children treated for multisystem inflammatory syndrome in the French West Indies

“…The French healthcare system is universal and free, thus the bias related to socioeconomic status and access to healthcare is likely to be less impactful in the French overseas Departments, and the outcomes of our patients are more in favor of socio-economic factors worsening the prognosis, rather than any ethnic susceptibility. This has already been shown for other auto-immune or inflammatory diseases in our pediatric population [ 11 , 12 ]. The number of MIS-C cases observed in Martinique, Guadeloupe and French Guiana was 40 over a 2.5-year period (incidence of 12 cases per 100 000 children).…”

“…The first documented case of MIS-C admitted to PICU in the FWI was in January 2021. The FWI have a combined population of approximately 330 000 children [ 11 , 12 ], the incidence of MIS-C cases was 12 per 100 000 children. Twenty-three were girls (57.5 %), the median age was 7 years (IQR 5–11).…”

Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus (jSLE)

“…To date, relatively few studies with limited patient numbers focused on NP-jSLE [ 19 , 29 – 34 ]. Based on these and following the general trend, NP-SLE may be more common and aggressive in jSLE (15–95%) when compared to adult-onset SLE (14–80%) [ 19 , 30 – 35 ]. Considering psychosocial aspects, morbidity and mortality associated with NP disease, timely diagnosis, outcome prediction and informed individualised treatment of NP-SLE are critical [ 36 ].…”