Update of evidence- and consensus-based guidelines for the treatment of juvenile idiopathic arthritis (JIA) by the German Society of Pediatric and Juvenile Rheumatic Diseases (GKJR): New perspectives on interdisciplinary care

Oommen, Prasad T.; Strauß, Timmy; Baltruschat, Karen; Foeldvari, Ivan; Deuter, Christoph; Ganser, Gerd; Haas, Johannes‐Peter; Hinze, Claas; Holzinger, Dirk; Hospach, Anton; Huppertz, Hans-Iko; Illhardt, Arnold; Jung, Michael E.; Kallinich, Tilmann; Klein, Ariane; Minden, Kirsten; Mönkemöller, Kirsten; Mrusek, S.; Neudorf, Ulrich; Dückers, Gregor; Niehues, Tim; Schneider, Matthias; Schoof, Philipp; Thon, A.; Wachowsky, Michael; Wagner, Norbert; Bloedt, Susanne; Hofer, Michaël; Tenbrock, Klaus; Schuetz, Catharina

doi:10.1016/j.clim.2022.109143

Cited by 13 publications

(7 citation statements)

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“…Inhibition of IL-1 or IL-6 has clearly demonstrated to be effective in controlling clinical and laboratory inflammatory manifestations of Still’s disease, with a strong glucocorticoid sparing effect and a tolerable safety profile ( 21 , 24 , 25 ). Despite the lack of internationally shared treatment guidelines, the current approach supports the use of IL-1 inhibitors in patients with Still’s disease ( 14 , 16 ), while IL-6 antagonists may represent an effective treatment choice in refractory cases with persistent inflammatory joint involvement ( 15 , 17 ). Different cytokine imbalances identified based on the different patterns of the disease (systemic versus chronic-articular) could suggest different molecular targets when establishing a personalized treatment approach in Still’s disease ( 26 , 27 ).…”

Section: Discussionmentioning

confidence: 99%

“…Treatment with biotechnological anti-interleukin (IL)-1 agents is recommended in patients with active disease especially in cases refractory to glucocorticoids and conventional disease modifying anti-rheumatic drugs (cDMARDs), avoiding a long-term glucocorticoids exposure ( 14 – 18 ). Agents blocking IL-6 are also recommended and may represent a valuable option in patients refractory to other treatment choices, as for joint involvement in Still’s disease ( 14 – 16 ). Randomized control trials and real-world studies describe the efficacy of anti-IL-1 inhibition on the articular inflammatory involvement, with a significant decrease in the number of tender joints, swollen joints, DAS28 and JADAS27 ( 19 – 21 ).…”

Section: Introductionmentioning

confidence: 99%

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Canakinumab as first-line biological therapy in Still’s disease and differences between the systemic and the chronic-articular courses: Real-life experience from the international AIDA registry

et al. 2022

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ObjectiveInterleukin (IL)-1 inhibitors are largely employed in patients with Still’s disease; in cases with refractory arthritis, IL-6 inhibitors have shown to be effective on articular inflammatory involvement. The aim of the present study is to assess any difference in the effectiveness of the IL-1β antagonist canakinumab prescribed as first-line biologic agent between the systemic and the chronic-articular Still’s disease.MethodsData were drawn from the retrospective phase of the AutoInflammatory Disease Alliance (AIDA) international registry dedicated to Still’s disease. Patients with Still’s disease classified according to internationally accepted criteria (Yamaguchi criteria and/or Fautrel criteria) and treated with canakinumab as first-line biologic agent were enrolled.ResultsA total of 26 patients (17 females, 9 males; 18 patients developing Still’s disease after the age of 16 years) were enrolled; 16 (61.5%) patients suffered from the systemic pattern of the disease; 10 (38.5%) patients suffered from the chronic-articular type. No differences were observed between the systemic and the chronic-articular Still’s disease in the frequency of complete response, of flares after the start of canakinumab (p = 0.701) and in the persistence in therapy (p = 0.62). No statistical differences were observed between the two groups after 3 months, 12 months and at the last assessment in the decrease of: the systemic activity score (p = 0.06, p = 0.17, p = 0.17, respectively); the disease activity score on 28 joints (p = 0.54, p = 0.77, p = 0.98, respectively); the glucocorticoid dosage (p = 0.15, p = 0.50, and p = 0.50, respectively); the use of concomitant disease modifying anti-rheumatic drugs (p = 0.10, p = 1.00, and p = 1.00, respectively). No statistically significant differences were observed in the decrease of erythrocyte sedimentation rate (p = 0.34), C reactive protein (p = 0.48), and serum ferritin levels (p = 0.34) after the start of canakinumab.ConclusionCanakinumab used for Still’s disease has been effective in controlling both clinical and laboratory manifestations disregarding the type of disease course when used as first-line biotechnological agent. These excellent results might have been further enhanced by the early start of IL-1 inhibition.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Canakinumab as first-line biological therapy in Still’s disease and differences between the systemic and the chronic-articular courses: Real-life experience from the international AIDA registry

et al. 2022

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“…Bei zunächst mildem Beginn ohne drohende Dauerschäden kann die Therapie, angepasst an die Erkrankungsaktivität, schrittweise eskaliert werden (siehe ▶ Abb. 4a) [43][44][45][46][47][48].…”

Section: Tripperarthritisunclassified

Woran denken bei Gelenkschmerzen?

Haas

2023

Kinder- und Jugendmedizin

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ZUSAMMENFASSUNGGelenksbeschwerden und Schmerzen im Bereich des muskulo-skelettalen Systems gehören zu den häufigsten Symptomen in kinder- und jugendmedizinischen Praxen und Ambulanzen. Meistens handelt es sich um temporäre durch Bagatelltraumen und/oder Überlastung verursachte Beschwerden oder eine Begleitreaktion z. B. im Rahmen von Infektionserkrankungen. Wiederholte und/oder länger anhaltende Beschwerden insbesondere in gleicher Lokalisation bedürfen jedoch einer eingehenden Abklärung. Differenzialdiagnostisch ist bei unklaren Gelenksbeschwerden eine Vielzahl von Erkrankungen möglich, die zum Teil sehr selten auftreten. Dies erschwert die zielorientierte Abklärung für den Praktiker. Eine rechtzeitige Diagnostik kann einerseits das Auftreten bleibender Schäden und andererseits bedrohliche Verkäufe, die bei einigen Erkrankungen die das muskulo-skelettale System betreffen möglich sind, vermeiden helfen. Die folgende Zusammenstellung soll hier einen praxisorientierten Leitfaden bieten.

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“…The treatment of Still’s disease has advanced remarkably in the last few years, with interleukin 1(IL-1) inhibition representing an effective and safe treatment option in patients with persistent inflammation after adequate glucocorticoid (GC) treatment ( 1 ). The association of conventional disease-modifying antirheumatic drugs (cDMARDs), especially methotrexate, may be useful when some inflammatory manifestations persist despite systemic improvement, such as in patients with polyarticular involvement and those with persistent arthritis or arthralgia ( 2–4 ). While the IL-6 antagonist tocilizumab has been proven to be effective in treating severe and persistent Still’s disease ( 5 , 6 ), tumor necrosis factor inhibitors have been proven to have some role but seem to be replaced by anti-IL-1 and anti-IL-6 agents in terms of systemic efficacy ( 6 ).…”

Section: Introductionmentioning

confidence: 99%

Efficacy of canakinumab in patients with Still’s disease across different lines of biologic therapy: real-life data from the International AIDA Network Registry for Still’s Disease

Vitale,

Caggiano,

Sfikakis

et al. 2023

Front. Med.

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IntroductionThe effectiveness of canakinumab may change according to the different times it is used after Still’s disease onset. This study aimed to investigate whether canakinumab (CAN) shows differences in short- and long-term therapeutic outcomes, according to its use as different lines of biologic treatment.MethodsPatients included in this study were retrospectively enrolled from the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to Still’s disease. Seventy-seven (51 females and 26 males) patients with Still’s disease were included in the present study. In total, 39 (50.6%) patients underwent CAN as a first-line biologic agent, and the remaining 38 (49.4%) patients were treated with CAN as a second-line biologic agent or subsequent biologic agent.ResultsNo statistically significant differences were found between patients treated with CAN as a first-line biologic agent and those previously treated with other biologic agents in terms of the frequency of complete response (p =0.62), partial response (p =0.61), treatment failure (p >0.99), and frequency of patients discontinuing CAN due to lack or loss of efficacy (p =0.2). Of all the patients, 18 (23.4%) patients experienced disease relapse during canakinumab treatment, 9 patients were treated with canakinumab as a first-line biologic agent, and nine patients were treated with a second-line or subsequent biologic agent. No differences were found in the frequency of glucocorticoid use (p =0.34), daily glucocorticoid dosage (p =0.47), or concomitant methotrexate dosage (p =0.43) at the last assessment during CAN treatment.ConclusionCanakinumab has proved to be effective in patients with Still’s disease, regardless of its line of biologic treatment.

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Update of evidence- and consensus-based guidelines for the treatment of juvenile idiopathic arthritis (JIA) by the German Society of Pediatric and Juvenile Rheumatic Diseases (GKJR): New perspectives on interdisciplinary care

Cited by 13 publications

References 73 publications

Canakinumab as first-line biological therapy in Still’s disease and differences between the systemic and the chronic-articular courses: Real-life experience from the international AIDA registry

Canakinumab as first-line biological therapy in Still’s disease and differences between the systemic and the chronic-articular courses: Real-life experience from the international AIDA registry

Woran denken bei Gelenkschmerzen?

Efficacy of canakinumab in patients with Still’s disease across different lines of biologic therapy: real-life data from the International AIDA Network Registry for Still’s Disease

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