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Pediatric Rheumatology
Published in: Pediatric Rheumatology 1/2018

Open Access 01-12-2018 | Research article

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

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 Weißbarth-Riedel, Helmut Wittkowski, Gerd Horneff, Dirk Foell, PRO-KIND SJIA project collaborators

Published in: Pediatric Rheumatology | Issue 1/2018

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Abstract

Background

Systemic juvenile idiopathic arthritis (SJIA) is an autoinflammatory disease associated with chronic arthritis. Early diagnosis and effective therapy of SJIA is desirable, so that complications are avoided. The PRO-KIND initiative of the German Society for Pediatric Rheumatology (GKJR) aims to define consensus-based strategies to harmonize diagnostic and therapeutic approaches in Germany.

Methods

We analyzed data on patients diagnosed with SJIA from 3 national registries in Germany. Subsequently, via online surveys and teleconferences among pediatric rheumatologists with a special expertise in the treatment of SJIA, we identified current diagnostic and treatment approaches in Germany. Those were harmonized via the formulation of statements and, supported by findings from a literature search. Finally, an in-person consensus conference using nominal group technique was held to further modify and consent the statements.

Results

Up to 50% of patients diagnosed with SJIA in Germany do not fulfill the International League of Associations for Rheumatology (ILAR) classification criteria, mostly due to the absence of chronic arthritis. Our findings suggest that chronic arthritis is not obligatory for the diagnosis and treatment of SJIA, allowing a diagnosis of probable SJIA. Malignant, infectious and hereditary autoinflammatory diseases should be considered before rendering a diagnosis of probable SJIA. There is substantial variability in the initial treatment of SJIA. Based on registry data, most patients initially receive systemic glucocorticoids, however, increasingly substituted or accompanied by biological agents, i.e. interleukin (IL)-1 and IL-6 blockade (up to 27.2% of patients). We identified preferred initial therapies for probable and definitive SJIA, including step-up patterns and treatment targets for the short-term (resolution of fever, decrease in C-reactive protein by 50% within 7 days), the mid-term (improvement in physician global and active joint count by at least 50% or a JADAS-10 score of maximally 5.4 within 4 weeks) and the long-term (glucocorticoid-free clinically inactive disease within 6 to 12 months), and an explicit treat-to-target strategy.

Conclusions

We developed consensus-based strategies regarding the diagnosis and treatment of probable or definitive SJIA in Germany.
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Metadata
Title
Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany
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 Weißbarth-Riedel
Helmut Wittkowski
Gerd Horneff
Dirk Foell
PRO-KIND SJIA project collaborators
Publication date
01-12-2018
Publisher
BioMed Central
Published in
Pediatric Rheumatology / Issue 1/2018
Electronic ISSN: 1546-0096
DOI
https://doi.org/10.1186/s12969-018-0224-2

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