Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany

• DOI: 10.1186/s12969-018-0224-2
• Publication Date: 2018-01-22T12:21:04Z
• Authors: Claas H. Hinze; Dirk Holzinger; Elke Lainka; Johannes-Peter Haas; Fabian Speth; Tilmann Kallinich; Nikolaus Rieber; Markus Hufnagel; Annette F. Jansson; Christian Hedrich; Hanna Winowski; Thomas Berger; Ivan Foeldvari; Gerd Ganser; Anton Hospach; Hans-Iko Huppertz; Kirsten Mönkemöller; Ulrich Neudorf; Elisabeth Weißbar...; Helmut Wittkowski; Gerd Horneff; Dirk Foell
• Keywords: Systemic therapy

Systemic juvenile idiopathic arthritis (SJIA) is an autoinflammatory disease associated with chronic arthritis. Early diagnosis and effective therapy of SJIA desirable, so that complications are avoided. The PRO-KIND initiative the German Society for Pediatric Rheumatology (GKJR) aims to define consensus-based strategies harmonize diagnostic therapeutic approaches in Germany.We analyzed data on patients diagnosed from 3 national registries Germany. Subsequently, via online surveys teleconferences among pediatric rheumatologists a special expertise treatment SJIA, we identified current Those were harmonized formulation statements and, supported by findings literature search. Finally, in-person consensus conference using nominal group technique was held further modify consent statements.Up 50% Germany do not fulfill International League Associations (ILAR) classification criteria, mostly due absence Our suggest obligatory allowing probable SJIA. Malignant, infectious hereditary diseases should be considered before rendering There substantial variability initial Based registry data, most initially receive systemic glucocorticoids, however, increasingly substituted or accompanied biological agents, i.e. interleukin (IL)-1 IL-6 blockade (up 27.2% patients). We preferred therapies definitive including step-up patterns targets short-term (resolution fever, decrease C-reactive protein within 7 days), mid-term (improvement physician global active joint count at least JADAS-10 score maximally 5.4 4 weeks) long-term (glucocorticoid-free clinically inactive 6 12 months), explicit treat-to-target strategy.We developed regarding