Systemic juvenile idiopathic arthritis in French Afro-Caribbean children, a retrospective cohort study

This retrospective study from the FOAD over a period of 22 years identified a cohort of 25 patients suffering from sJIA. This is the largest cohort of pediatric patients with this disease of Afro-Caribbean origin. The current international JIA classification has contributed to a more uniform classification of this disease, thus facilitating comparisons of diagnosis and epidemiology across countries and ethnic populations [5]. One of the strengths of this work is its multicenter nature, with the participation of all the referring pediatricians in the FOAD. Our methodology enabled us to analyze patient therapies by referring to the national registry for rare diseases, as well the registries of local referring clinicians and by exploring computerized hospital archives with wide inclusion criteria. This led to exhaustive identification of patients, and we cross-checked data from multiple sources to minimize the loss of patients and data. Thus, the prevalence in our study may have been underestimated, because of the memory bias of retrospective studies covering a long period. There is also potential for recruitment bias in our study, because our methodology mostly identifies patients who required hospitalization. Nevertheless, most patients with sJIA have a short initial hospitalization at disease onset.

Our study shows a stable incidence of sJIA in this population of Afro-Caribbean children over a 22-year period, at a rate of 0.4 patients per year per 100,000 children. This incidence is consistent with that described in Western countries [8, 9]. However, this contrasts with the rates of three main systemic diseases in the FOAD (namely systemic lupus, bowel disease and type 1 diabetes) with pediatric onset showing an increase in the number of cases over the years (unpublished personal data). The overall prognosis of the children in our cohort was similar to that of cohorts from Western countries [9, 10]. In the biotherapy era, with all biotherapies available in 2009 in the FOAD, more than two-thirds of children did not require biotherapy to achieve complete remission during childhood. None of our patients had ophthalmological involvement, which is consistent with the low incidence of uveitis in this subtype of JIA [11]. The proportion of patients with coronary involvement at onset was higher in our cohort. Coronary damage has previously been described in sJIA [12], but none of our patients had subsequent complications, such as later coronary artery damage, dilatation or relapse. The diagnosis of sJIA was made in the presence of evidence of persistent arthritis and/or MAS, and these patients did not have a more severe clinical or biological course. Our patients had few autoantibodies, which clearly differentiates this entity from other JIA or systemic disease [13]. Although we chose an international definition of MAS associated with sJIA [7] to avoid it simply reflecting the perception of the clinicians, we found a high percentage of MAS in our cohort, higher than previously described [14], which was not related to an increase in mortality. However, in our cohort most of the children who had MAS at some point during the course of their diseases required biotherapy for disease control. MAS complicating sJIA occurs more frequently during childhood than in adult onset Still’s disease [15]. Our patients were on therapy for a small part of the time they were being followed up by specialists, and a large majority of those who transitioned to adult care had been weaned from treatment for over a year. This probably illustrates the pediatricians’ doubts about the future outcomes of these patients [16]. Adult/child studies on the evolutionary profile of these patients are necessary to assess the risk of recurrence in adulthood.

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