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Pediatr Rheumatol Online J. 2018 Jan 22;16(1):7. doi: 10.1186/s12969-018-0224-2.

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

Author information

1
Department of Pediatric Rheumatology and Immunology, University Hospital Münster, Münster, Albert-Schweitzer-Campus 1, Building W30, 48149, Münster, Germany. claas.hinze@ukmuenster.de.
2
Department of Pediatric Rheumatology and Immunology, University Hospital Münster, Münster, Albert-Schweitzer-Campus 1, Building W30, 48149, Münster, Germany.
3
Department of Pediatric Hematology-Oncology, University of Duisburg-Essen, Essen, Germany.
4
Department of Pediatrics, University Hospital Essen, Essen, Germany.
5
German Center for Pediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany.
6
Department of Pediatric Pulmonology and Immunology, Charité, Berlin, Germany.
7
Department of Pediatrics, StKM GmbH and Technical University Muenchen, Munich, Germany.
8
The Department of Pediatrics I, University of Tuebingen, Tuebingen, Germany.
9
Department of Pediatrics, University Hospital Freiburg, Freiburg, Germany.
10
Division of Pediatric Rheumatology & Immunology, Dr. von Hauner Children's Hospital, University Hospital Munich, Munich, Germany.
11
Department of Pediatrics, University Hospital Dresden, Dresden, Germany.
12
Department of Women's & Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, UK.
13
Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK.
14
Department of Pediatric Rheumatology, St. Josef Hospital, Sendenhorst, Germany.
15
Vestian Children's Hospital, Datteln, Germany.
16
Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany.
17
Department of Pediatrics, Olga Hospital, Stuttgart, Germany.
18
Department of Pediatrics, Prof. Hess Children's Hospital, Bremen, Germany.
19
Department of Pediatrics, Cologne Municipal Hospital, Cologne, Germany.
20
Department of Pediatrics, University Hospital Hamburg, Hamburg, Germany.
21
Department of Pediatrics, Asklepios Hospital, St. Augustin, Germany.
22
University of Cologne, Cologne, Germany.

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.

KEYWORDS:

Biologics; Diagnosis; Glucocorticoids; Interleukin-1 blockade; Interleukin-6 blockade; Systemic juvenile idiopathic arthritis; Treat-to-target

PMID:
29357887
PMCID:
PMC5778670
DOI:
10.1186/s12969-018-0224-2
[Indexed for MEDLINE]
Free PMC Article

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