Abstract
BackgroundSystemic 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.MethodsWe 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.ResultsUp 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.ConclusionsWe developed consensus-based strategies regarding the diagnosis and treatment of probable or definitive SJIA in Germany.
Highlights
Systemic juvenile idiopathic arthritis (SJIA) is an autoinflammatory disease associated with chronic arthritis
They collected and analyzed the literature, planned the process and drafted different statements. This included the following key steps: 1) Planning and consensus on aims of the project group, 2) retrieval of real-life patient data from 2 registries in Germany, 3) retrieval of evidence from literature on the following topics: Diagnostic/classification criteria or case definitions, diagnostics or differential diagnosis, therapeutic targets, monitoring, and medications, 4) a survey of diagnostic approaches based on clinical case scenario, 5) survey analysis by the project group, and formulation of statements based on the aforementioned steps, and 6) discussion and consensus of all statements in a revised form during a face-to-face consensus conference organized in June 2016
Characteristics of patients diagnosed with SJIA in Germany Only 59.9 and 57.1% patients diagnosed with SJIA in the Autoinflammatory disease (AID) registry and the ICON-Juvenile idiopathic arthritis (JIA) cohort, respectively, had arthritis at any time during the follow-up
Summary
Systemic juvenile idiopathic arthritis (SJIA) is an autoinflammatory disease associated with chronic arthritis. Diagnosis and effective therapy of SJIA is desirable, so that complications are avoided. Systemic juvenile idiopathic arthritis (SJIA) is a rare and serious autoinflammatory disorder characterized by systemic inflammation (hectic quotidian fevers, typical rash, serositis, hepatosplenomegaly, lymphadenopathy, acute-phase reaction) and variably accompanied or followed by chronic arthritis [1,2,3]. The hypothesis exists that early effective treatment of SJIA during a “window of opportunity” may fundamentally affect its long-term outcome and, reduce the risk of a chronic articular course [4,5,6]. Early diagnosis and treatment of SJIA may be essential in order to avoid long-term complications. The existing International League of Associations for Rheumatology (ILAR) classification criteria for SJIA have been criticized [7]. The various classification criteria are summarized in Additional file 1: Table S1
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