Universität zu Köln

Hinze, Claas H., Holzinger, Dirk ORCID: 0000-0002-8062-8820, Lainka, Elke, Haas, Johannes-Peter, Speth, Fabian, Kallinich, Tilmann, Rieber, Nikolaus, Hufnagel, Markus, Jansson, Annette F., Hedrich, Christian, Winowski, Hanna, Berger, Thomas, Foeldvari, Ivan, Ganser, Gerd, Hospach, Anton, Huppertz, Hans-Iko, Moenkemoeller, Kirsten, Neudorf, Ulrich, Weissbarth-Riedel, Elisabeth, Wittkowski, Helmut, Horneff, Gerd and Foell, Dirk (2018). Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany. Pediatr. Rheumatol., 16. LONDON: BIOMED CENTRAL LTD. ISSN 1546-0096

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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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Hinze, Claas H. UNSPECIFIED UNSPECIFIED UNSPECIFIED Holzinger, Dirk UNSPECIFIED orcid.org/0000-0002-8062-8820 UNSPECIFIED Lainka, Elke UNSPECIFIED UNSPECIFIED UNSPECIFIED Haas, Johannes-Peter UNSPECIFIED UNSPECIFIED UNSPECIFIED Speth, Fabian UNSPECIFIED UNSPECIFIED UNSPECIFIED Kallinich, Tilmann UNSPECIFIED UNSPECIFIED UNSPECIFIED Rieber, Nikolaus UNSPECIFIED UNSPECIFIED UNSPECIFIED Hufnagel, Markus UNSPECIFIED UNSPECIFIED UNSPECIFIED Jansson, Annette F. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hedrich, Christian UNSPECIFIED UNSPECIFIED UNSPECIFIED Winowski, Hanna UNSPECIFIED UNSPECIFIED UNSPECIFIED Berger, Thomas UNSPECIFIED UNSPECIFIED UNSPECIFIED Foeldvari, Ivan UNSPECIFIED UNSPECIFIED UNSPECIFIED Ganser, Gerd UNSPECIFIED UNSPECIFIED UNSPECIFIED Hospach, Anton UNSPECIFIED UNSPECIFIED UNSPECIFIED Huppertz, Hans-Iko UNSPECIFIED UNSPECIFIED UNSPECIFIED Moenkemoeller, Kirsten UNSPECIFIED UNSPECIFIED UNSPECIFIED Neudorf, Ulrich UNSPECIFIED UNSPECIFIED UNSPECIFIED Weissbarth-Riedel, Elisabeth UNSPECIFIED UNSPECIFIED UNSPECIFIED Wittkowski, Helmut UNSPECIFIED UNSPECIFIED UNSPECIFIED Horneff, Gerd UNSPECIFIED UNSPECIFIED UNSPECIFIED Foell, Dirk UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-198617
DOI:	10.1186/s12969-018-0224-2
Journal or Publication Title:	Pediatr. Rheumatol.
Volume:	16
Date:	2018
Publisher:	BIOMED CENTRAL LTD
Place of Publication:	LONDON
ISSN:	1546-0096
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language INTERLEUKIN-1 RECEPTOR ANTAGONIST; OF-RHEUMATOLOGY RECOMMENDATIONS; ACUTE LYMPHOBLASTIC-LEUKEMIA; ONSET STILLS-DISEASE; PEDIATRIC RHEUMATOLOGY; PRELIMINARY CRITERIA; RANDOMIZED-TRIAL; CHILDREN; CLASSIFICATION; ADULT Multiple languages Pediatrics; Rheumatology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/19861