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

Autor: Hinze, Claas H., Holzinger, Dirk, 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, Mönkemöller, Kirsten, Neudorf, Ulrich, Weißbarth-Riedel, Elisabeth, Wittkowski, Helmut, Horneff, Gerd, Foell, Dirk, Weller, Frank, Thon, Angelika, Lilienthal, Eggert, Lutz, Thomas, Oommen, Prasad T., Berendes, Rainer, Berrang, Jens, Tenbrock, Klaus, Rietschel, Christoph, Heubner, Georg, Küster, Rolf-Michael
Jazyk: angličtina
Rok vydání: 2018
Předmět:
medicine.medical_specialty
Consensus
lcsh:Diseases of the musculoskeletal system
Databases
Factual

Medizin
Arthritis
Biologics
Biological Factors
03 medical and health sciences
0302 clinical medicine
Rheumatology
Systemic juvenile idiopathic arthritis
Germany
Internal medicine
Diagnosis
medicine
Humans
Immunology and Allergy
Initial treatment
Registries
030212 general & internal medicine
Practice Patterns
Physicians'

Child
Intensive care medicine
Glucocorticoids
030203 arthritis & rheumatology
business.industry
Interleukin-1 blockade
lcsh:RJ1-570
Consensus conference
lcsh:Pediatrics
Chronic arthritis
medicine.disease
Arthritis
Juvenile

Antirheumatic Agents
Child
Preschool

Pediatrics
Perinatology and Child Health

Interleukin-6 blockade
Registry data
lcsh:RC925-935
business
Inactive disease
Treat-to-target
Research Article
Zdroj: PEDIATRIC RHEUMATOLOGY
Pediatric Rheumatology Online Journal, Vol 16, Iss 1, Pp 1-14 (2018)
Pediatric Rheumatology Online Journal
Popis: 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. Electronic supplementary material The online version of this article (10.1186/s12969-018-0224-2) contains supplementary material, which is available to authorized users.
Databáze: OpenAIRE