Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in 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...
| Main Authors: | , , , , , , , , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2018-01-01
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| Series: | Pediatric Rheumatology Online Journal |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s12969-018-0224-2 |
| _version_ | 1819101679645097984 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Claas H. Hinze |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-22T01:22:30Z |
| format | Article |
| id | doaj.art-64103f490e774a9b8a7cd7b21dfddc8c |
| institution | Directory Open Access Journal |
| issn | 1546-0096 |
| language | English |
| last_indexed | 2024-12-22T01:22:30Z |
| publishDate | 2018-01-01 |
| publisher | BMC |
| record_format | Article |
| series | Pediatric Rheumatology Online Journal |
| spelling | doaj.art-64103f490e774a9b8a7cd7b21dfddc8c2022-12-21T18:43:42ZengBMCPediatric Rheumatology Online Journal1546-00962018-01-0116111410.1186/s12969-018-0224-2Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in GermanyClaas H. Hinze0Dirk Holzinger1Elke Lainka2Johannes-Peter Haas3Fabian Speth4Tilmann Kallinich5Nikolaus Rieber6Markus Hufnagel7Annette F. Jansson8Christian Hedrich9Hanna Winowski10Thomas Berger11Ivan Foeldvari12Gerd Ganser13Anton Hospach14Hans-Iko Huppertz15Kirsten Mönkemöller16Ulrich Neudorf17Elisabeth Weißbarth-Riedel18Helmut Wittkowski19Gerd Horneff20Dirk Foell21PRO-KIND SJIA project collaboratorsDepartment of Pediatric Rheumatology and Immunology, University Hospital MünsterDepartment of Pediatric Rheumatology and Immunology, University Hospital MünsterDepartment of Pediatrics, University Hospital EssenGerman Center for Pediatric and Adolescent RheumatologyGerman Center for Pediatric and Adolescent RheumatologyDepartment of Pediatric Pulmonology and Immunology, CharitéDepartment of Pediatrics, StKM GmbH and Technical University MuenchenDepartment of Pediatrics, University Hospital FreiburgDivision of Pediatric Rheumatology & Immunology, Dr. von Hauner Children’s Hospital, University Hospital MunichDepartment of Pediatrics, University Hospital DresdenDepartment of Pediatric Rheumatology, St. Josef HospitalVestian Children’s HospitalHamburg Center for Pediatric and Adolescent RheumatologyDepartment of Pediatric Rheumatology, St. Josef HospitalDepartment of Pediatrics, Olga HospitalDepartment of Pediatrics, Prof. Hess Children’s HospitalDepartment of Pediatrics, Cologne Municipal HospitalDepartment of Pediatrics, University Hospital EssenDepartment of Pediatrics, University Hospital HamburgDepartment of Pediatric Rheumatology and Immunology, University Hospital MünsterDepartment of Pediatrics, Asklepios HospitalDepartment of Pediatric Rheumatology and Immunology, University Hospital MünsterAbstract 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.http://link.springer.com/article/10.1186/s12969-018-0224-2Systemic juvenile idiopathic arthritisDiagnosisTreat-to-targetGlucocorticoidsBiologicsInterleukin-1 blockade |
| spellingShingle | 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 Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany Pediatric Rheumatology Online Journal Systemic juvenile idiopathic arthritis Diagnosis Treat-to-target Glucocorticoids Biologics Interleukin-1 blockade |
| title | Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany |
| title_full | Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany |
| title_fullStr | Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany |
| title_full_unstemmed | Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany |
| title_short | Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany |
| title_sort | practice and consensus based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in germany |
| topic | Systemic juvenile idiopathic arthritis Diagnosis Treat-to-target Glucocorticoids Biologics Interleukin-1 blockade |
| url | http://link.springer.com/article/10.1186/s12969-018-0224-2 |
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