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.