DISCUSSION
Our patients shared many clinical characteristics with previously described patients. Previously identified demographic risk factors included younger age of disease onset, specifically less than 2 years of age, and presence of trisomy 21 2, 9. Seven of our patients (78%) were under school age at diagnosis of sJIA-LD, though our average age (4.6 years) was older than previously described. The majority presented within 2 years after initial diagnosis of sJIA. Of note, due to their mild presentation, one patient did present with sJIA-LD in early adolescence, almost 7 years following initial diagnosis of sJIA. One of the nine patients had trisomy 21 (11% compared to the pediatric prevalence of approximately 1 out of 700 per the CDC). Additionally, in our cohort, patients were more likely to be female and to self-identify as white. One prior study identified a female predominance 10, but our literature review did not identify other studies that showed race/ethnicity differences in sJIA-LD cohorts. Patients with sJIA-LD have high morbidity and mortality; at the time of this paper, none of our patients have passed from sJIA-LD.