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Evaluation of the Presentation of Systemic Onset Juvenile Rheumatoid Arthritis: Data from the Pennsylvania Systemic Onset Juvenile Arthritis Registry (PASOJAR)
EDWARD M. BEHRENS, TIMOTHY BEUKELMAN, LISA GALLO, JULIE SPANGLER, MARGALIT ROSENKRANZ, THASCHAWEE ARKACHAISRI, ROSANNE AYALA, BRANDT GROH, TERRI H. FINKEL, and RANDY Q. CRON
ABSTRACT. 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. Conclusion. 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. (First Release Dec 15 2007; J Rheumatol 2008;35:343-8) Key Indexing Terms:
SYSTEMIC ONSET JUVENILE RHEUMATOID ARTHRITIS
From the Department of Pediatrics, Division of Rheumatology, The Children's Hospital of Philadelphia, Philadelphia; Department of Pediatrics, Division of Rheumatology, Children's Hospital of Pittsburgh, Pittsburgh; and Department of Pediatrics, Division of Rheumatology, Pennsylvania State University Hershey Medical Center, Hershey, Pennsylvania, USA. Supported in part through grants from the Eastern, Central, and Western Pennsylvania Arthritis Foundation Chapters. Dr. Behrens was supported by grant number T32-HD0043021 from the National Institutes of Health. Dr. Beukelman was supported by gifts from Amgen, Berlex, Merck, Novartis, Pfizer, and Wyeth to the pharmacoepidemiology training program of the Center for Clinical Epidemiology and Biostatistics at the University of Pennsylvania School of Medicine. Dr. Cron was supported by grants from the Nickolett Family Awards Program for JRA Research and the Foerderer Fund. E.M. Behrens, MD; T. Beukelman, MD; L. Gallo; T.H. Finkel, MD, PhD; R.Q. Cron, MD, PhD, The Children's Hospital of Philadelphia, Department of Pediatrics, Division of Rheumatology; J. Spangler; M. Rosenkranz, MD; T. Arkachaisri, MD, Department of Pediatrics, Division of Rheumatology, Children's Hospital of Pittsburgh; R. Ayala; B. Groh, MD, Department of Pediatrics, Division of Rheumatology, Pennsylvania State University Hershey Medical Center. Address reprint requests to Dr. E.M. Behrens, Children's Hospital of Philadelphia, 3615 Civic Center Blvd., ARC 1102, Philadelphia, PA 19104-4318, USA. E-mail: behrens@email.chop.edu Accepted for publication September 20, 2007. |
