ARTHRITIS & RHEUMATOLOGY Vol. 68, No. 12, December 2016, pp 3023–3034 DOI 10.1002/art.39796 V C 2016, American College of Rheumatology Outcome and Trends in Treatment of Systemic Juvenile Idiopathic Arthritis in the German National Pediatric Rheumatologic Database, 2000–2013 Jens Klotsche, 1 Anna Raab, 1 Martina Niewerth, 2 Claudia Sengler, 2 Gerd Ganser, 3 Tilmann Kallinich, 4 Tim Niehues, 5 Markus Hufnagel, 6 Angelika Thon, 7 Toni Hospach, 8 Gerd Horneff, 9 and Kirsten Minden 1 Objective. To investigate the clinical presentation and medical treatment of patients with systemic juvenile idiopathic arthritis (JIA) during the first year of illness. Our study focused on 3-year outcomes in a subsample of patients who were followed up longitudinally. Methods. From 2000 to 2013, 597 patients with sys- temic JIA and a disease duration of £12 months were re- corded in the National Pediatric Rheumatologic Database. Among those patients, 3-year outcome data were available for 133. These data included the clinical Juvenile Arthritis Disease Activity Score in 10 joints (JADAS-10) and the physician’s global assessment score (on a numerical rating scale), as well as assessment of joint involvement, growth retardation, and patient-reported outcomes. Results. The median clinical JADAS-10 declined significantly, from 7 in 2000 to 2 in 2013, while the propor- tion of patients with inactive disease increased from 19% in 2000 to 41% in 2013. The rate of treatment with sys- temic glucocorticoids and disease-modifying antirheu- matic drugs (DMARDs) remained stable from 2000 to 2013. By 2013, the proportion of patients with systemic JIA who were treated with biologic DMARDs had increased to 20%. At 3-year follow-up, 72% of patients with systemic JIA had inactive disease, and 77% had no functional limi- tations. Growth retardation was associated with persis- tently high disease activity and continuing treatment with systemic glucocorticoids. At the 3-year follow-up, one- third of patients were still being treated with systemic glucocorticoids. Conclusion. The proportion of patients with inac- tive disease has increased over the past decade. Possible explanations may include improved access to specialized care, additional treatment options, and earlier or faster step-up treatment. However, challenges in the manage- ment of systemic JIA remain, as ~30% of patients con- tinue to present with ongoing active disease. Juvenile idiopathic arthritis (JIA) is the common term for all forms of arthritis that begin before age 16 years, persist for more than 6 weeks, and for which the eti- ology is unknown. Systemic juvenile idiopathic arthritis (JIA) is 1 of 7 categories of JIA that account for 5–15% of all JIA cases (1–4). The peak age at onset of systemic JIA is between 18 months and 24 months (4). Systemic JIA has a distinct clinical phenotype characterized by daily spiking fevers and is accompanied by a variety of other sys- temic signs, including evanescent erythematous skin rash, generalized lymphadenopathy, pericarditis, pleuritis, and hepatosplenomegaly (2). Despite its relative rareness, The National Pediatric Rheumatologic Database received a grant from the Federal Ministry of Education and Research in 2005. It is currently funded by the Children’s Arthritis Foundation (Kinder Rheumastiftung). 1 Jens Klotsche, PhD, Anna Raab, MD, Kirsten Minden, MD: German Rheumatism Research Centre Berlin and Charit e University Hospital Berlin, Berlin, Germany; 2 Martina Niewerth, MSc, Claudia Sengler, MD: German Rheumatism Research Centre Berlin, Berlin, Germany; 3 Gerd Ganser, MD: St. Josef-Stift Sendenhorst Hospital, Sendenhorst, Germany; 4 Tilmann Kallinich, MD: Charit e University Hospital Berlin, Berlin, Germany; 5 Tim Niehues, MD: HELIOS Klinikum Krefeld, Krefeld, Germany; 6 Markus Hufnagel, MD: University Medical Center Freiburg, Freiburg, Germany; 7 Angelika Thon, MD: Hannover Medical School, Hannover, Germany; 8 Toni Hospach, MD: Olgahospital, Clinical Center Stuttgart, Stuttgart, Germany; 9 Gerd Horneff, MD: Asklepios Clinic Sankt Augustin, St. Augustin, Germany. Drs. Klotsche and Raab contributed equally to this work. Dr. Hufnagel has received research grants from Novartis and Roche. Dr. Hospach has received consulting fees and/or speaking fees from Pfizer, Novartis, Roche, and AbbVie (less than $10,000 each). Dr. Horneff has received research grants from Pfizer, AbbVie, and Roche. Dr. Minden has received research grants from Pfizer and AbbVie. Address correspondence to Jens Klotsche, PhD, German Rheu- matism Research Centre, a Leibniz Institute, Chariteplatz 1, 10117 Berlin, Germany. E-mail: Jens.Klotsche@drfz.de. Submitted for publication November 11, 2015; accepted in revised form June 16, 2016. 3023