Evaluation of the presentation of systemic onset juvenile rheumatoid arthritis: data from the Pennsylvania Systemic Onset Juvenile Arthritis Registry (PASOJAR).
OBJECTIVE
To characterize the initial clinical and laboratory features of patients with systemic onset juvenile rheumatoid arthritis (soJRA) through a Web-based registry.


METHODS
Patients diagnosed with soJRA in the last 15 years at 3 medical centers in Pennsylvania were identified. Data were collected retrospectively using a Web-based interface in compliance with patient privacy standards. Inferential statistics were used to compare features of patients with and without macrophage activation syndrome.


RESULTS
We identified 136 patients; 88% of patients presented with arthritis (8% mono-, 45% oligo-, 47% polyarticular). The most common joints involved were the knee (68% of patients with arthritis), wrist (68%), and ankle (57%). The International League of Associations for Rheumatology criteria for systemic juvenile idiopathic arthritis (SJIA) identified only 30% of patients at presentation.


CONCLUSIONS
We successfully characterized the presenting features of a relatively rare disease, soJRA, through the use of a Web-based registry. Current classification criteria for SJIA may not be particularly sensitive for diagnosis at presentation.
Behrens
Edward M
EM
Department of Pediatrics, Division of Rheumatology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4318, USA. behrens@email.chop.edu
Beukelman
Timothy
T
Gallo
Lisa
L
Spangler
Julie
J
Rosenkranz
Margalit
M
Arkachaisri
Thaschawee
T
Ayala
Rosanne
R
Groh
Brandt
B
Finkel
Terri H
TH
Cron
Randy Q
RQ
D000293:Adolescent; D001171:Arthritis, Juvenile / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology; D015415:Biomarkers; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D020407:Internet; D008297:Male; D010414:Pennsylvania / Q000453:epidemiology; D012042:Registries
D015415:Biomarkers